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Hidradenitis Suppurativa: A Case Study
DEPARTMENT
Case Study—Primary Care
Hidradenitis Suppurativa: A Case Study Molly L. Schulman, RN, CPNP, & Hana M. Pantuso, RN, CPNP
KEY WORDS Hidradenitis suppurativa, acne inversa, Verneuil’s disease, Sartorius score, Hurley stages, Dermatology Life Quality Index
Hidradenitis suppurativa (HS) is a chronic, recurrent inflammatory disorder of the hair follicles that affects apocrine gland–bearing sites such as the axillary, inguinal, and perianal areas. HS, also known as acne inversa, was initially described by French surgeon Alfred
Section Editors Jo Ann Serota, DNP, RN, CPNP, FAANP Corresponding Editor Ambler Pediatrics Ambler, Pennsylvania Beverly Giordano, MS, RN, CPNP, PMHS University of Florida, Gainesville Gainesville, Florida Donna Hallas, PhD, PNP-BC, CPNP, PMHS, FAANP New York University New York, New York Molly L. Schulman, Pediatric Nurse Practitioner, Emergency Department, Children’s Hospital of the King’s Daughters, Norfolk, VA. Hana M. Pantuso, Pediatric Nurse Practitioner, St. John’s Riverside Hospital, Yonkers, NY. Conflicts of interest: None to report. Correspondence: Molly L. Schulman, RN, CPNP, 1152 Llewelleyn Ave, Norfolk, VA, 23507; e-mail: [email protected]. J Pediatr Health Care. (2015) 29, 364-370. 0891-5245/$36.00 Copyright Q 2015 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. Published online January 6, 2015. http://dx.doi.org/10.1016/j.pedhc.2014.11.011
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Verneuil in 1854 and is thus also referred to as Verneuil’s disease (Revuz, 2010). HS, which is diagnosed clinically, initially presents with tender subcutaneous nodules and is later manifested as recurrent abscesses with subsequent formation of fistulas, dermal contractures, and sinus tracts (Alikhan, Lynch, & Eisen, 2009; Ingram et al., 2012; Jemec, 2012). Smoking and obesity are documented risk factors for HS. HS affects females more than males, with onset of symptoms generally occurring after puberty. HS is referred to in the literature as an ‘‘orphan disease’’ given the lack of knowledge about HS in the mediThere is often a cal community and lengthy delay in the among lay persons, diagnosis and despite a global prevalence of 1% in the geninitiation of eral population (Naldi, treatment for HS 2006). There is often a because early lengthy delay in the diagnosis and initiation lesions are of treatment for HS commonly because early lesions misdiagnosed as are commonly misdiagnosed as simple simple nodules or nodules or abscesses abscesses by by providers who are providers who are unfamiliar with the disease. HS significantly unfamiliar with the affects patients’ quality disease. of life (QOL) because of the chronic and recurrent nature of symptoms, as well as the lack of satisfying treatment options (Jemec, 2012; Lee, Yoon, & Kist, 2007; Revuz, 2010; Revuz et al., 2008). CASE PRESENTATION A previously healthy African American 16-year-old female adolescent presented to the pediatric emergency department (ED) because of axillary and inguinal abscesses. The patient and her mother reported that the lesions had been fluctuating in severity for the preceding Journal of Pediatric Health Care
3 years. The patient initially had been seen by her primary care provider and was referred directly to the ED for incision and drainage. HISTORY OF PRESENT ILLNESS The patient reported having multiple painful boils on her axillary areas and genital area. The latter were reported to be painful and warm to the touch, with active drainage of malodorous pus. The patient had been afebrile. The patient and her mother reported a history of intermittent abscesses in the aforementioned areas for about 3 years in total. The patient had previously undergone incision and drainage of various lesions on multiple occasions. The patient last received a course of an unknown oral antibiotic for treatment of an abscess about 2 months earlier. The patient had no known recent sick contacts and reported no recent travel. MEDICAL HISTORY The patient reported a history of constipation; she had hard stools about twice weekly, intermittently treated with Fleet enemas (C.B. Fleet Company, Lynchburg, VA). The patient and her mother reported a history of an allergy to penicillin resulting in urticaria, as well as a peanut allergy with resulting anaphylaxis. The patient had an EpiPen (Mylan Inc., Canonsburg, PA) for emergency use; otherwise she took no prescription or overthe-counter medications regularly. The patient had no other medical or surgical history. She was up to date on vaccinations for her age. FAMILY HISTORY No significant family medical history was reported. Both parents were alive and well. There was no known family history of HS or diabetes mellitus. SOCIAL/PERSONAL HISTORY The patient was an only child and lived with both parents. She was in the 11th grade and reported receiving good grades. The patient denied tobacco, alcohol, or drug use. The patient had had three lifetime male sexual partners and reported using condoms inconsistently. She had never been pregnant and related no known history of sexually transmitted infections. The patient had a negative human immunodeficiency virus (HIV) test result a few months prior to presentation and declined testing for HIV and other sexually transmitted infections in the ED. A patient health questionnaire (PHQ)-2 used to screen for signs of depression was negative (Maurer, 2012). REVIEW OF SYSTEMS The review of systems was negative for general complaints, including fever and chills. The review of systems revealed a history of constipation with no complaints of abdominal pain, nausea, vomiting, or diarrhea. www.jpedhc.org
PHYSICAL EXAMINATION The patient appeared to be generally well and in no acute distress. She was afebrile with vital signs that were within normal limits for her age. She was noted to be obese with a body mass index of 39.0, which placed her at greater than the 99th percentile (Barlow, 2007). Pertinent positive findings on physical examination included a 4 2 cm tender, indurated abscess in the left axillary area with active purulent drainage. The right axillary area demonstrated a 2 2 cm ulceration over the mid-axillary line. Both axillae showed multiple hypertrophic irregular scars throughout the area. The patient also had a 4 2 cm indurated, tender abscess with central fluctuance to the mons pubis. Malodorous, yellow-green discharge from multiple open lesions in the gluteal clefts was noted. The physical examination was otherwise unremarkable. DIAGNOSTIC TESTING The patient did not require imaging studies emergently. Laboratory studies yielded the following pertinent results: white blood cell count, 7.5 103/ml (normal range, 4.0-10.0 103/ml); hemoglobin, 9.9 g/dl (normal range, 13.0-16.6 g/dl); hematocrit, 32.5% (normal range, 37.0%–49.0%); and mean corpuscular volume, 76.7 fl (normal range, 78-102 fl). The complete blood cell count with differential was otherwise within normal limits. An aerobic culture of purulent drainage yielded few gram-positive cocci and bacilli. DIFFERENTIAL DIAGNOSES Differential diagnoses that warranted consideration included common abscesses, carbuncles, and furuncles, as well as epidermoid cysts (Poli, Jemec, & Revuz, 2006). However, the patient’s history of chronicity and presence of lesions in the typical zones, with recurrence after antibiotic treatment, indicated a diagnosis of HS (Alikhan et al., 2009; Lee et al., 2007). The patient’s physical examination findings, with diffuse involvement of entire areas, indicated HS in Hurley stage III (Jemec, 2012; Poli et al., 2006). The patient also had a diagnosis of obesity as previously noted. In addition, the patient had a diagnosis of anemia identified on a complete blood cell count. The hemoglobin level and mean corpuscular volume below the normal range for her age and sex indicated microcytic anemia, of which iron deficiency anemia is the most common cause. In this patient, nutritional deficiencies and menstrual blood loss are likely sources. Differential diagnoses for the patient’s anemia include thalassemia, anemia of chronic disease, and sideroblastic anemia (Barlow, 2007; Short & Domagalski, 2013; Van Vranken, 2010). TREATMENT PLAN The patient and her mother were briefly counseled in the ED regarding the diagnosis of HS, July/August 2015
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including pathogenesis and treatment. The patient was seen in the ED by the general surgery team, who subsequently performed an incision and drainage of the lesion on the mons pubis, which was well tolerated without complications. The patient was also treated with doses of intra-
venous morphine and intravenous clindamycin in the ED. The surgery team recommended follow-up with dermatology and plastic surgery. The patient was admitted to the general pediatric floor for pain management and intravenous antibiotics.
CASE STUDY QUESTIONS 1. 2. 3. 4. 5.
How is HS diagnosed and classified? What does the evidence indicate regarding the epidemiology and pathophysiology of HS? What are the effects of HS on a patient’s QOL? What is the role of the primary care nurse practitioner (NP) in the treatment of patients with HS? What are the best available treatment options for HS?
CASE STUDY ANSWERS Diagnosis and Classification 1. How is HS diagnosed and classified? The diagnosis of HS is made clinically, with no specific confirmatory test available (Mortimer & Lunniss, 2000). Differential diagnoses that ought to be considered include common carbuncles and furuncles, as well as Crohn’s disease in the case of ano-perineal lesions. HS can be differentiated from other diseases through characteristic findings such as resistance to antibiotics and the recovery of multiple species of bacteria upon wound culture, with the latter finding having been noted with this patient. HS is more difficult to diagnose in early or mild cases. Family history and presence of premenstrual flares may aid in the diagnosis at this stage. Diagnostic criteria require the presence of the three main features, which were identified on this patient’s history and physical examination. These features are typical lesions, typical localization, and a history of chronicity with relapses or recurrence of symptoms (Alikhan et al., 2009; Poli et al., 2006). The typical topography of HS lesions involves areas of apocrine gland–bearing skin, including the axillary, inguinal, and anogenital regions. The priThe typical mary lesions of HS topography of HS initially present as tenlesions involves der subcutaneous nodules. Lesions go on to areas of apocrine rupture and coalesce, gland–bearing skin, forming painful dermal including the abscesses. The abscesses of HS are usually axillary, inguinal, described as deep and and ano-genital rounded without cenregions. tral necrosis (Alikhan et al., 2009; Slade, Powell, & Mortimer, 2003). Recurrent lesions in the same vicinity lead to sinus tract formation and the
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development of characteristic hypertrophic fibrous scarring (Mortimer & Lunniss, 2000; Revuz, 2010). Because of the large spectrum of clinical severity in persons with HS, a number of classification systems have been proposed to evaluate severity and guide treatment. The first classification system suggested is Hurley’s clinical staging, as shown in Table 1, which remains useful for overall patient classification. An alternate grading scheme, the Sartorius scoring system, has been developed, featuring uniform outcome variables. This scoring system, as shown in Table 2, has been proposed to allow for more consistent reporting of outcomes in clinical research and for monitoring of individual patients. Both severity indexes lack subjective elements, although authors suggest gathering subjective information from patients through the use of validated questionnaires such as the Dermatology Life Quality Index (DLQI) or the Skindex (Poli et al., 2006; Sartorius, Lapins, Emtestam, & Jemec, 2003). Epidemiology and Pathophysiology 2. What does the evidence indicate regarding the epidemiology and pathophysiology of HS? As noted, HS is often considered to be a rare disease, yet estimates of prevalence have ranged from 0.33 to 4 patients per 1000. Authors of two French case control studies estimated the prevalence to be as high as 1 in 100, with additional studies based on population samples supporting the finding of a 1-year prevalence of 1% (Canoui-Poitrine et al., 2009; Naldi, 2006; Revuz et al., 2008; Vazquez, Alikhan, Weaver, Wetter & Davis, 2012). Women are affected more frequently than men, with a female to male ratio of 3:1. HS usually develops in the second or third decade of life, with peak severity for 10 to 15 years after the onset of symptoms (Jemec, 2012; Mortimer & Lunniss, 2000; Poli et al., 2006; Slade et al., 2003). In female patients, HS onset occurs almost exclusively after menarche; it rarely occurs
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TABLE 1. Hurley’s clinical classifications for hidradenitis suppurativa Hurley stage I II III
Extent of disease Single or multiple abscess formation without sinus tracts and cicatrization Recurrent abscesses with sinus tract formation; lesions remain widely separated Multiple recurrent, interconnected tracts and abscesses with diffuse or near diffuse involvement of an entire area
Note. From Alikhan et al., 2009, Jemec, 2012, & Poli et al., 2006.
before puberty, and premenstrual flares are common. A racial predilection in African Americans, particularly patients of African Caribbean origin, has been proposed by authors but has not been substantiated by demographic evidence (Alikhan et al., 2009; Lee et al., 2007; Slade et al., 2003). The cause of HS remains unclear, with understanding of the pathogenesis having evolved over time. Numerous hypotheses concerning the role of infection, genetics, and immunity, as well as risk factors such as tobacco use and obesity, have been considered (Ingram et al., 2012). Previously thought to be a disorder of the apocrine glands themselves, HS is now considered to be primarily an inflammatory disease originating from occlusion of the hair follicles. That is, HS is best understood as a disorder of the follicular epithelium within apocrine gland–bearing skin (Layton, 2006). HS is notably not due to poor hygiene, nor has any relationship been demonstrated between the development of HS and the use of deodorant or depilatory products (Alikhan et al., 2009; Revuz, 2010; Slade et al., 2003). Both obesity and cigarette smoking are documented risk factors for HS, with both elevated body mass index and tobacco usage correlated with disease severity (Canoui-Poitrine et al., 2009; Jemec, 2012; Sartorius, Emtestam, Jemec, & Lapins, 2009; Vazquez et al., 2012). Obesity is thought to act as a
TABLE 2. Sartorius scoring system Outcome variable Anatomic region involved
Number of lesions
Longest distance between two lesions OR size of single lesion Lesions clearly separated by normal skin
Points per finding 3 points/axilla 3 points/gluteal 3 points/groin 3 points/left inframammary 3 points/right inframammary 3 points/other area 1 point per scar 1 point per other finding 2 points per abscess 2 points per nodule 4 points per fistula 2 points if < 5 cm 4 points if < 10 cm 8 points if > 10 cm 0 if yes 6 if no
Note. From Alikhan et al., 2009, Poli et al., 2006, & Sartorius et al., 2003.
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secondary factor in HS, aggravating lesions through mechanical trauma and shearing at areas of skin folds (Lee et al., 2007; Slade et al., 2003). Bacterial infection is not an initial causative factor in the pathogenesis of HS, as evidenced by the sterility of pus from early lesions and the absence of serious infectious complications in persons with HS. Furthermore, routine bacteriologic studies are frequently negative. Of the pathogens found in lesions, a large variety of microorganisms may be isolated. However, there is a high risk of contamination of HS wound cultures with resident skin flora when samples are collected from the surface of lesions. In cultures obtained from deeper HS lesions, Staphylococcus aureus, coagulase-negative staphylococci, and various anaerobic bacterial species are commonly isolated (Alikhan et al., 2009; Oprica & Nord, 2006; Revuz, 2010). Despite the relationship between the onset of HS and puberty, hyperandrogenism is not thought to play a role in the pathogenesis of HS. Signs of virilization, such as hirsutism, are unusual in female patients with HS, and most have normal androgen profiles (Alikhan et al., 2009; Faure & Drapier-Faure, 2006; Mortimer & Lunniss, 2000; Revuz, 2010). Investigators have also postulated that an underlying immunologic aberration may be involved in the pathogenesis of HS, given its chronic relapsing nature. HS appears to be a genetically heterogeneous disease; an autosomaldominant pattern has been reported in the literature, with 30% to 40% of patients reporting a family history of HS (Alikhan et al., 2009; Revuz, 2010). Effects on QOL 3. What are the effects of HS on a patient’s QOL? It is not surprising that HS may have a negative impact on a patient’s QOL, given the clinical characteristics and chronicity. Characteristics of HS that impact QOL include the chronicity of the disease, the lack of satisfactory treatment options, and the intimate locations of lesions (Lee et al., 2007; Revuz et al., 2008; Wolkenstein, Loundou, Barrau, Auquier, & Revuz, 2007). On average, patients experience symptoms of HS for about 18 to 20 years and often receive initial misdiagnoses, leading to poor or inappropriate treatment that may add to the disease burden (Poli et al., 2006). HS is recurrent in nature, often necessitating repeated absences from work or school because of symptoms such as pain, malodorous discharge, and July/August 2015
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the unsightly appearance of lesions (Onderdijk et al., 2013). Because HS occurs primarily in the axilla, groin, and mammary areas, patients commonly report feeling embarrassed and self-conscious, with the ano-genital regions producing the most impairment on QOL and interpersonal relationships (Matusiak, Bieniek, & Szepietowski, 2010; Wolkenstein, 2006). Multiple studies have confirmed that many patients with HS commonly experience depression as a result of their condition (Dufour, Emtestam, & Jemec, 2014; Esmann & Jemec, 2011; Matusiak et al., 2010; Wolkenstein et al., 2007). For example, Esmann and Jemec (2011) interviewed patients using a qualitative methodology, which revealed that HS has a significant psychosocial impact. Patients reported feeling ‘‘unworthy’’ and ‘‘unlovable’’ and described their lesions as ‘‘ugly, smelly, and embarrassing.’’ Studies using the DLQI as a quantitative measure have shown that HS has a greater negative impact on QOL than other chronic dermatologic conditions such as alopecia and psoriasis. QOL impact, as measured with the DLQI, is correlated with disease severity, as well other specific factors such as the frequency of flares, the amount of sick days taken, and the extent of pain and drainage from lesions (Dufour et al., 2014; Matusiak et al., 2010; Onderdijk et al., 2013; Wolkenstein, 2006). Role of the NP in Treating Patients With HS 4. What is the role of the primary care NP in the treatment of patients with HS? HS represents a management challenge, not only for specialists in dermatology and surgery, but also for primary care providers and clinicians who see patients in urgent and emergency care settings (Alikhan et al., 2009; Vazquez et al., 2012). A multidisciplinary approach involving primary care, dermatology, plastic surgery, and other specialties is often indicated (Lee et al., 2007). Because relapses and flares are common in persons with HS and no single definitive therapy is available, an array of treatment modalities may be indicated depending on the disease severity and patient presentation (Alikhan et al., 2009). Given the chronic nature of the condition, primary care NPs play a key role by providing a patientcentered medical home, thus ensuring continuous and comprehensive care to maximize outcomes. For adolescents and young adults with HS, comprehensive care will entail providing supportive care and addressing QOL issues, as discussed later. Other aspects of the NP’s role may include coordination with specialists, as well as screening for and management of comorbidities. In terms of supportive care, a number of recommendations may prove to be helpful for patients with HS regardless of disease severity. Cessation of smoking for patients who smoke cigarettes should be encouraged, using multidisciplinary interventions as indi368
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cated. Patients should be counseled regarding weight loss and the maintenance of a healthy weight. Other general measures include advising patients to wear soft or friction-free undergarments and clothing, including the avoidance of tight jeans (Alikhan et al., 2009; Slade et al., 2003). Given the documented effect of HS on QOL, assessment and management of QOL issues is an important aspect of the primary care provider’s role in the care of patients with HS. Providers are advised to assess pain using a visual analog scale and to further assess QOL with use of the DLQI (Wolkenstein, 2006). Screening for depression can be performed with use of a number of commonly available and validated screening tools, such as the PHQ-2 and PHQ-9 (Maurer, 2012). Patients should be counseled on stress management and referred to a support group if one is available (Alikhan et al., 2009; Mortimer & Lunniss, 2000; Slade et al., 2003). The effect of HS on a patient’s psychosocial well-being may warrant referrals for individual counseling and/or psychiatric care if indicted (Rambhatla, Lim, & Hamzavi, 2012). Treatment Options 5. What are the best available treatment options for HS? Treatment of HS should be undertaken in a stepwise fashion depending upon disease severity (Ingram et al., 2012). Management is tailored according to the severity and distribution of HS lesions, taking into account QOL issues with attention to the risks and benefits of any treatment. Therapies recommended for patients with Hurley stage I disease include topical antibiotics, a short course of systemic antibiotics, intralesional steroids, and adjuvant treatment with zinc gluconate. Secondline therapies recommended for patients with Hurley stage II disease include long-term antibiotic therapy, CO2 laser ablation, immunosuppressive therapies, limited surgical excision, and radiation therapy. For patients presenting with stage III disease, authors recommend largely palliative treatment with immunosuppressive therapy, as well as surgical treatment with wide surgical excision of the affected regions with or without radiation therapy (Alikhan et al., 2009; Jemec & Revuz, 2006; Rambhatla et al., 2012; Revuz, 2010). Topical antibiotic treatment with clindamycin has been used with success and is the best-studied topical medication. When specific pathogens, such as anaerobes, are isolated, antibiotic therapy should be targeted based on the resulting sensitivities. However, the indicated duration of treatment is not standardized (Oprica & Nord, 2006). Oral antibiotic treatment regimens that have been studied retrospectively include combination therapy with clindamycin and rifampin, a combination that is effective in treating other follicular occlusion disorders. This regimen has been shown effective in significantly reducing patients’ Sartorius scores, although adverse gastrointestinal effects caused Journal of Pediatric Health Care
patients to discontinue therapy in all studies (Gener et al., 2009; Jemec & Revuz, 2006; Mendonca & Griffiths, 2006; van der Zee, Boer, Prens, & Jemec, 2009). Other nonsurgical treatment options include retinoids, which have been considered for use in HS treatment because of their efficacy in the treatment of acne. However, studies to date show a lack of efficacy for patients with HS who take oral isotretinoin. Hormonal therapy, specifically antiandrogen therapy, has been subject to several trials with mixed results. Immunosuppression with systemic steroids has also been used because of the nonspecific anti-inflammatory effects that may prove useful during the inflammatory stages of HS. Although case reports have shown improvement with the use of drugs such as cyclosporine, relapse tends to occur after discontinuation of these medications (Alikhan et al., 2009; Rambhatla et al., 2012). Surgical management, specifically wide and radical excision, is considered the gold standard for severe or intractable HS. Surgical interventions should be initiated in a multidisciplinary setting, Surgical allowing optimal management, preoperative and postspecifically wide operative treatment. Interventions range and radical from local excision to excision, is wide or radical exciconsidered the sion with healing by secondary intention or gold standard for grafting, with the severe or choice of surgical techintractable HS. nique depending upon the region and disease severity. In addition to more extensive surgeries, simple incision and drainage of select lesions may be indicated at intervals for temporary relief, as was performed with this patient in the ED (Alikhan et al., 2009; Lapins & Emtestam, 2006; Revuz, 2010). FOLLOW-UP/OUTCOMES The patient was discharged the following day, with an appointment having been scheduled with dermatology. Further patient education regarding supportive care and treatment of HS was provided by the pediatric hospitalist. Prescriptions were issued for oral ibuprofen and trimethoprim/sulfamethoxazole tablets. The patient was also scheduled to follow up with her primary care provider for outpatient management of HS. Primary care provider follow-up was further advised for evaluation of microcytic anemia, because the diagnosis of iron deficiency anemia requires laboratory evidence of anemia and of low iron stores, such as through decreased serum ferritin levels (Short & Domagalski, 2013; Van Vranken, 2010). The patient also requires additional laboratory testing for obesitywww.jpedhc.org
related medical conditions, such as hyperlipidemia, type 2 diabetes mellitus, and nonalcoholic fatty liver disease. In addition to the recommended laboratory evaluation, the patient requires assessment of behavioral risks related to obesity such as current eating habits and sedentary behaviors before the initiation of multidisciplinary interventions. Although the patient’s screening for depression with use of the PHQ-2 was negative, depression is an important comorbidity of obesity and of HS. Therefore, depression screening may be repeated using instruments developed for primary care such as the Patient Health Questionnaire for Adolescents or the Beck Depression Inventory–Primary Care Version, both of which have been used successfully in adolescents (Maurer, 2012; U.S. Preventive Services Task Force, 2009). CONCLUSION In summary, HS is a chronic, recurrent skin disease affecting areas of apocrine gland–bearing skin that affects mainly females after the onset of puberty, with large variations in clinical severity and presentation. Although HS is not uncommon, it remains an orphan disease that is not well known or understood by medical providers, leading to delays in diagnosis and initiation of treatment, as occurred with this patient. The diagnosis is made clinically with no confirmatory test. HS significantly affects patients’ QOL because of its chronic nature, along with the lack of satisfactory treatments. As this case study highlights, management ought to be multidisciplinary, taking into account QOL, along with disease severity and patient preference, in conjunction with specialties including plastic surgery and dermatology. We thank Dr. David Listman for his assistance in seeking Institutional Review Board approval for this case study. We also extend our gratitude to Dr. Rita Marie John for our educations as pediatric nurse practitioners. REFERENCES Alikhan, A., Lynch, P. J., & Eisen, D. B. (2009). Hidradenitis suppurativa: A comprehensive review. Journal of the American Academy of Dermatology, 60(4), 539-561. Barlow, S. E. (2007). Expert Committee recommendations regarding the prevention, assessment, and treatment of child and adolescent overweight and obesity: Summary report. Pediatrics, 120(Suppl. 4), S164-S192. Canoui-Poitrine, F., Revuz, J. E., Wolkenstein, P., Viallette, C., Gabison, G., Pouget, F., . Bastuji-Garin, S. (2009). Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity. Journal of the American Academy of Dermatology, 61, 51-57. Dufour, D. N., Emtestam, L., & Jemec, G. B. (2014). Hidradenitis suppurativa: A common and burdensome, yet under-recognised,
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inflammatory skin disease. Postgraduate Medical Journal, 90(1062), 216-221. Esmann, S., & Jemec, G. B. (2011). Psychosocial impact of hidradenitis suppurativa: A qualitative study. Acta Dermato-Venereologica, 91(3), 328-332. Faure, M., & Drapier-Faure, E. (2006). Endocrinology. In G. B. . Jemec, J. E. . Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 95-99). Heidelberg, Germany: Springer. Gener, G., Canoui-Poitrine, F., Revuz, J. E., Faye, O., Poli, F., Gabison, G., . Bastuji-Garin, S. (2009). Combination therapy with clindamycin and rifampicin for hidradenitis suppurativa: A series of 116 consecutive patients. Dermatology, 219(2), 148-154. Ingram, J. R., Desai, N., Kai, A. C., Chua, A. L., Woo, P. N., Kerdel, F., . Piguet, V. (2012). Interventions for hidradenitis suppurativa (protocol). Cochrane Database of Systematic Reviews(9), CD010081. Jemec, G. B., & Revuz, J. E. (2006). Treatment. In G. B. Jemec, J. E. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 183-186). Heidelberg, Germany: Springer. Jemec, G. B. E. (2012). Hidradenitis suppurativa. The New England Journal of Medicine, 366(2), 158-164. Lapins, J., & Emtestam, L. (2006). Surgery. In G. B. Jemec, J. E. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 161-173). Heidelberg, Germany: Springer. Layton, A. (2006). Pathology of hidradenitis suppurativa. In G. B. Jemec, J. E. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 25-33). Heidelberg, Germany: Springer. Lee, R. A., Yoon, A., & Kist, J. (2007). Hidradenitis suppurativa: An update. Advances in Dermatology, 23, 289-306. Matusiak, L., Bieniek, A., & Szepietowski, J. C. (2010). Psychophysical aspects of hidradenitis suppurativa. Acta Dermato-Venereologica, 90(3), 264-268. Maurer, D. M. (2012). Screening for depression. American Family Physician, 85(2), 139-144. Mendonca, C. O., & Griffiths, C. E. M. (2006). Clindamycin and rifampicin combination therapy for hidradenitis suppurativa. British Journal of Dermatology, 154(5), 977-978. Mortimer, P. S., & Lunniss, P. J. (2000). Hidradenitis suppurativa. Journal of the Royal Society of Medicine, 93(8), 420-422. Naldi, L. (2006). Epidemiology. In G. B. E. Jemec, J. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 58-64). Heidelberg, Germany: Springer. Onderdijk, A. J., van der Zee, H. H., Esmann, S., Lophaven, S., Dufour, D. N., Jemec, G. B., & Boer, J. (2013). Depression in patients with hidradenitis suppurativa. Journal of the European Academy of Dermatology and Venereology, 27(4), 473-478. Oprica, C., & Nord, C. E. (2006). Bacteriology of hidradenitis suppurativa. In G. B. Jemec, J. E. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 86-94). Heidelberg, Germany: Springer.
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Poli, F., Jemec, G. B. E., & Revuz, J. (2006). Clinical presentation. In G. B. E. Jemec, J. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 11-24). Heidelberg, Germany: Springer. Rambhatla, P. V., Lim, H. W., & Hamzavi, I. (2012). A systematic review of treatments for hidradenitis suppurativa. Archives of Dermatology, 148(4), 439-446. Revuz, J. (2010). Hidradenitis suppurativa. La Pressa Medicale, 39(12), 1254-1264. Revuz, J. E., Canoui-Poitrine, F., Wolkenstein, P., Viallette, C., Gabison, G., Pouget, F., . Bastuji-Garin, S. (2008). Prevalence and factors associated with hidradenitis suppurativa: Results from two case-control studies. Journal of the American Academy of Dermatology, 59, 596-601. Sartorius, K., Emtestam, L., Jemec, G. B. E., & Lapins, J. (2009). Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity. British Journal of Dermatology, 161, 831-839. Sartorius, K., Lapins, J., Emtestam, L., & Jemec, G. B. E. (2003). Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. British Journal of Dermatology, 149, 211-213. Short, M. W., & Domagalski, J. E. (2013). Iron deficiency anemia: Evaluation and management. American Family Physician, 87(2), 98-104. Slade, D. E. M., Powell, B. W., & Mortimer, P. S. (2003). Hidradenitis suppurativa: Pathogenesis and management. British Journal of Plastic Surgery, 56(5), 451-461. U.S. Preventive Services Task Force. (2009). Screening and treatment for major depressive disorder in children and adolescents: U.S. Preventive Services Task Force recommendation statement. Pediatrics, 123(4), 1223-1338. van der Zee, H. H., Boer, J., Prens, E. P., & Jemec, G. B. E. (2009). The effect of combined treatment with oral clindamycin and oral rifampicin in patients with hidradenitis suppurativa. Dermatology, 219(2), 143-147. Van Vranken, M. (2010). Evaluation of microcytosis. American Family Physician, 82(9), 1117-1122. Vazquez, B. G., Alikhan, A., Weaver, A. L., Wetter, D. A., & Davis, M. D. (2012). Incidence of hidradenitis suppurativa and associated factors: A population-based study of Olmstead County, Minnesota. Journal of Investigative Dermatology, 133, 97-103. Wolkenstein, P. (2006). Quality of life in hidradenitis suppurativa. In G. B. Jemec, J. E. Revuz & J. J. Leyden (Eds.), Hidradenitis suppurativa (pp. 116-119). Heidelberg, Germany: Springer. Wolkenstein, P., Loundou, A., Barrau, K., Auquier, P., & Revuz, J. (2007). Quality of life impairment in hidradenitis suppurativa: A study of 61 cases. Journal of the American Academy of Dermatology, 56, 621-623.
Journal of Pediatric Health Care
Case Study—Primary Care
Hidradenitis Suppurativa: A Case Study Molly L. Schulman, RN, CPNP, & Hana M. Pantuso, RN, CPNP
KEY WORDS Hidradenitis suppurativa, acne inversa, Verneuil’s disease, Sartorius score, Hurley stages, Dermatology Life Quality Index
Hidradenitis suppurativa (HS) is a chronic, recurrent inflammatory disorder of the hair follicles that affects apocrine gland–bearing sites such as the axillary, inguinal, and perianal areas. HS, also known as acne inversa, was initially described by French surgeon Alfred
Section Editors Jo Ann Serota, DNP, RN, CPNP, FAANP Corresponding Editor Ambler Pediatrics Ambler, Pennsylvania Beverly Giordano, MS, RN, CPNP, PMHS University of Florida, Gainesville Gainesville, Florida Donna Hallas, PhD, PNP-BC, CPNP, PMHS, FAANP New York University New York, New York Molly L. Schulman, Pediatric Nurse Practitioner, Emergency Department, Children’s Hospital of the King’s Daughters, Norfolk, VA. Hana M. Pantuso, Pediatric Nurse Practitioner, St. John’s Riverside Hospital, Yonkers, NY. Conflicts of interest: None to report. Correspondence: Molly L. Schulman, RN, CPNP, 1152 Llewelleyn Ave, Norfolk, VA, 23507; e-mail: [email protected]. J Pediatr Health Care. (2015) 29, 364-370. 0891-5245/$36.00 Copyright Q 2015 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. Published online January 6, 2015. http://dx.doi.org/10.1016/j.pedhc.2014.11.011
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Verneuil in 1854 and is thus also referred to as Verneuil’s disease (Revuz, 2010). HS, which is diagnosed clinically, initially presents with tender subcutaneous nodules and is later manifested as recurrent abscesses with subsequent formation of fistulas, dermal contractures, and sinus tracts (Alikhan, Lynch, & Eisen, 2009; Ingram et al., 2012; Jemec, 2012). Smoking and obesity are documented risk factors for HS. HS affects females more than males, with onset of symptoms generally occurring after puberty. HS is referred to in the literature as an ‘‘orphan disease’’ given the lack of knowledge about HS in the mediThere is often a cal community and lengthy delay in the among lay persons, diagnosis and despite a global prevalence of 1% in the geninitiation of eral population (Naldi, treatment for HS 2006). There is often a because early lengthy delay in the diagnosis and initiation lesions are of treatment for HS commonly because early lesions misdiagnosed as are commonly misdiagnosed as simple simple nodules or nodules or abscesses abscesses by by providers who are providers who are unfamiliar with the disease. HS significantly unfamiliar with the affects patients’ quality disease. of life (QOL) because of the chronic and recurrent nature of symptoms, as well as the lack of satisfying treatment options (Jemec, 2012; Lee, Yoon, & Kist, 2007; Revuz, 2010; Revuz et al., 2008). CASE PRESENTATION A previously healthy African American 16-year-old female adolescent presented to the pediatric emergency department (ED) because of axillary and inguinal abscesses. The patient and her mother reported that the lesions had been fluctuating in severity for the preceding Journal of Pediatric Health Care
3 years. The patient initially had been seen by her primary care provider and was referred directly to the ED for incision and drainage. HISTORY OF PRESENT ILLNESS The patient reported having multiple painful boils on her axillary areas and genital area. The latter were reported to be painful and warm to the touch, with active drainage of malodorous pus. The patient had been afebrile. The patient and her mother reported a history of intermittent abscesses in the aforementioned areas for about 3 years in total. The patient had previously undergone incision and drainage of various lesions on multiple occasions. The patient last received a course of an unknown oral antibiotic for treatment of an abscess about 2 months earlier. The patient had no known recent sick contacts and reported no recent travel. MEDICAL HISTORY The patient reported a history of constipation; she had hard stools about twice weekly, intermittently treated with Fleet enemas (C.B. Fleet Company, Lynchburg, VA). The patient and her mother reported a history of an allergy to penicillin resulting in urticaria, as well as a peanut allergy with resulting anaphylaxis. The patient had an EpiPen (Mylan Inc., Canonsburg, PA) for emergency use; otherwise she took no prescription or overthe-counter medications regularly. The patient had no other medical or surgical history. She was up to date on vaccinations for her age. FAMILY HISTORY No significant family medical history was reported. Both parents were alive and well. There was no known family history of HS or diabetes mellitus. SOCIAL/PERSONAL HISTORY The patient was an only child and lived with both parents. She was in the 11th grade and reported receiving good grades. The patient denied tobacco, alcohol, or drug use. The patient had had three lifetime male sexual partners and reported using condoms inconsistently. She had never been pregnant and related no known history of sexually transmitted infections. The patient had a negative human immunodeficiency virus (HIV) test result a few months prior to presentation and declined testing for HIV and other sexually transmitted infections in the ED. A patient health questionnaire (PHQ)-2 used to screen for signs of depression was negative (Maurer, 2012). REVIEW OF SYSTEMS The review of systems was negative for general complaints, including fever and chills. The review of systems revealed a history of constipation with no complaints of abdominal pain, nausea, vomiting, or diarrhea. www.jpedhc.org
PHYSICAL EXAMINATION The patient appeared to be generally well and in no acute distress. She was afebrile with vital signs that were within normal limits for her age. She was noted to be obese with a body mass index of 39.0, which placed her at greater than the 99th percentile (Barlow, 2007). Pertinent positive findings on physical examination included a 4 2 cm tender, indurated abscess in the left axillary area with active purulent drainage. The right axillary area demonstrated a 2 2 cm ulceration over the mid-axillary line. Both axillae showed multiple hypertrophic irregular scars throughout the area. The patient also had a 4 2 cm indurated, tender abscess with central fluctuance to the mons pubis. Malodorous, yellow-green discharge from multiple open lesions in the gluteal clefts was noted. The physical examination was otherwise unremarkable. DIAGNOSTIC TESTING The patient did not require imaging studies emergently. Laboratory studies yielded the following pertinent results: white blood cell count, 7.5 103/ml (normal range, 4.0-10.0 103/ml); hemoglobin, 9.9 g/dl (normal range, 13.0-16.6 g/dl); hematocrit, 32.5% (normal range, 37.0%–49.0%); and mean corpuscular volume, 76.7 fl (normal range, 78-102 fl). The complete blood cell count with differential was otherwise within normal limits. An aerobic culture of purulent drainage yielded few gram-positive cocci and bacilli. DIFFERENTIAL DIAGNOSES Differential diagnoses that warranted consideration included common abscesses, carbuncles, and furuncles, as well as epidermoid cysts (Poli, Jemec, & Revuz, 2006). However, the patient’s history of chronicity and presence of lesions in the typical zones, with recurrence after antibiotic treatment, indicated a diagnosis of HS (Alikhan et al., 2009; Lee et al., 2007). The patient’s physical examination findings, with diffuse involvement of entire areas, indicated HS in Hurley stage III (Jemec, 2012; Poli et al., 2006). The patient also had a diagnosis of obesity as previously noted. In addition, the patient had a diagnosis of anemia identified on a complete blood cell count. The hemoglobin level and mean corpuscular volume below the normal range for her age and sex indicated microcytic anemia, of which iron deficiency anemia is the most common cause. In this patient, nutritional deficiencies and menstrual blood loss are likely sources. Differential diagnoses for the patient’s anemia include thalassemia, anemia of chronic disease, and sideroblastic anemia (Barlow, 2007; Short & Domagalski, 2013; Van Vranken, 2010). TREATMENT PLAN The patient and her mother were briefly counseled in the ED regarding the diagnosis of HS, July/August 2015
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including pathogenesis and treatment. The patient was seen in the ED by the general surgery team, who subsequently performed an incision and drainage of the lesion on the mons pubis, which was well tolerated without complications. The patient was also treated with doses of intra-
venous morphine and intravenous clindamycin in the ED. The surgery team recommended follow-up with dermatology and plastic surgery. The patient was admitted to the general pediatric floor for pain management and intravenous antibiotics.
CASE STUDY QUESTIONS 1. 2. 3. 4. 5.
How is HS diagnosed and classified? What does the evidence indicate regarding the epidemiology and pathophysiology of HS? What are the effects of HS on a patient’s QOL? What is the role of the primary care nurse practitioner (NP) in the treatment of patients with HS? What are the best available treatment options for HS?
CASE STUDY ANSWERS Diagnosis and Classification 1. How is HS diagnosed and classified? The diagnosis of HS is made clinically, with no specific confirmatory test available (Mortimer & Lunniss, 2000). Differential diagnoses that ought to be considered include common carbuncles and furuncles, as well as Crohn’s disease in the case of ano-perineal lesions. HS can be differentiated from other diseases through characteristic findings such as resistance to antibiotics and the recovery of multiple species of bacteria upon wound culture, with the latter finding having been noted with this patient. HS is more difficult to diagnose in early or mild cases. Family history and presence of premenstrual flares may aid in the diagnosis at this stage. Diagnostic criteria require the presence of the three main features, which were identified on this patient’s history and physical examination. These features are typical lesions, typical localization, and a history of chronicity with relapses or recurrence of symptoms (Alikhan et al., 2009; Poli et al., 2006). The typical topography of HS lesions involves areas of apocrine gland–bearing skin, including the axillary, inguinal, and anogenital regions. The priThe typical mary lesions of HS topography of HS initially present as tenlesions involves der subcutaneous nodules. Lesions go on to areas of apocrine rupture and coalesce, gland–bearing skin, forming painful dermal including the abscesses. The abscesses of HS are usually axillary, inguinal, described as deep and and ano-genital rounded without cenregions. tral necrosis (Alikhan et al., 2009; Slade, Powell, & Mortimer, 2003). Recurrent lesions in the same vicinity lead to sinus tract formation and the
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development of characteristic hypertrophic fibrous scarring (Mortimer & Lunniss, 2000; Revuz, 2010). Because of the large spectrum of clinical severity in persons with HS, a number of classification systems have been proposed to evaluate severity and guide treatment. The first classification system suggested is Hurley’s clinical staging, as shown in Table 1, which remains useful for overall patient classification. An alternate grading scheme, the Sartorius scoring system, has been developed, featuring uniform outcome variables. This scoring system, as shown in Table 2, has been proposed to allow for more consistent reporting of outcomes in clinical research and for monitoring of individual patients. Both severity indexes lack subjective elements, although authors suggest gathering subjective information from patients through the use of validated questionnaires such as the Dermatology Life Quality Index (DLQI) or the Skindex (Poli et al., 2006; Sartorius, Lapins, Emtestam, & Jemec, 2003). Epidemiology and Pathophysiology 2. What does the evidence indicate regarding the epidemiology and pathophysiology of HS? As noted, HS is often considered to be a rare disease, yet estimates of prevalence have ranged from 0.33 to 4 patients per 1000. Authors of two French case control studies estimated the prevalence to be as high as 1 in 100, with additional studies based on population samples supporting the finding of a 1-year prevalence of 1% (Canoui-Poitrine et al., 2009; Naldi, 2006; Revuz et al., 2008; Vazquez, Alikhan, Weaver, Wetter & Davis, 2012). Women are affected more frequently than men, with a female to male ratio of 3:1. HS usually develops in the second or third decade of life, with peak severity for 10 to 15 years after the onset of symptoms (Jemec, 2012; Mortimer & Lunniss, 2000; Poli et al., 2006; Slade et al., 2003). In female patients, HS onset occurs almost exclusively after menarche; it rarely occurs
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TABLE 1. Hurley’s clinical classifications for hidradenitis suppurativa Hurley stage I II III
Extent of disease Single or multiple abscess formation without sinus tracts and cicatrization Recurrent abscesses with sinus tract formation; lesions remain widely separated Multiple recurrent, interconnected tracts and abscesses with diffuse or near diffuse involvement of an entire area
Note. From Alikhan et al., 2009, Jemec, 2012, & Poli et al., 2006.
before puberty, and premenstrual flares are common. A racial predilection in African Americans, particularly patients of African Caribbean origin, has been proposed by authors but has not been substantiated by demographic evidence (Alikhan et al., 2009; Lee et al., 2007; Slade et al., 2003). The cause of HS remains unclear, with understanding of the pathogenesis having evolved over time. Numerous hypotheses concerning the role of infection, genetics, and immunity, as well as risk factors such as tobacco use and obesity, have been considered (Ingram et al., 2012). Previously thought to be a disorder of the apocrine glands themselves, HS is now considered to be primarily an inflammatory disease originating from occlusion of the hair follicles. That is, HS is best understood as a disorder of the follicular epithelium within apocrine gland–bearing skin (Layton, 2006). HS is notably not due to poor hygiene, nor has any relationship been demonstrated between the development of HS and the use of deodorant or depilatory products (Alikhan et al., 2009; Revuz, 2010; Slade et al., 2003). Both obesity and cigarette smoking are documented risk factors for HS, with both elevated body mass index and tobacco usage correlated with disease severity (Canoui-Poitrine et al., 2009; Jemec, 2012; Sartorius, Emtestam, Jemec, & Lapins, 2009; Vazquez et al., 2012). Obesity is thought to act as a
TABLE 2. Sartorius scoring system Outcome variable Anatomic region involved
Number of lesions
Longest distance between two lesions OR size of single lesion Lesions clearly separated by normal skin
Points per finding 3 points/axilla 3 points/gluteal 3 points/groin 3 points/left inframammary 3 points/right inframammary 3 points/other area 1 point per scar 1 point per other finding 2 points per abscess 2 points per nodule 4 points per fistula 2 points if < 5 cm 4 points if < 10 cm 8 points if > 10 cm 0 if yes 6 if no
Note. From Alikhan et al., 2009, Poli et al., 2006, & Sartorius et al., 2003.
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secondary factor in HS, aggravating lesions through mechanical trauma and shearing at areas of skin folds (Lee et al., 2007; Slade et al., 2003). Bacterial infection is not an initial causative factor in the pathogenesis of HS, as evidenced by the sterility of pus from early lesions and the absence of serious infectious complications in persons with HS. Furthermore, routine bacteriologic studies are frequently negative. Of the pathogens found in lesions, a large variety of microorganisms may be isolated. However, there is a high risk of contamination of HS wound cultures with resident skin flora when samples are collected from the surface of lesions. In cultures obtained from deeper HS lesions, Staphylococcus aureus, coagulase-negative staphylococci, and various anaerobic bacterial species are commonly isolated (Alikhan et al., 2009; Oprica & Nord, 2006; Revuz, 2010). Despite the relationship between the onset of HS and puberty, hyperandrogenism is not thought to play a role in the pathogenesis of HS. Signs of virilization, such as hirsutism, are unusual in female patients with HS, and most have normal androgen profiles (Alikhan et al., 2009; Faure & Drapier-Faure, 2006; Mortimer & Lunniss, 2000; Revuz, 2010). Investigators have also postulated that an underlying immunologic aberration may be involved in the pathogenesis of HS, given its chronic relapsing nature. HS appears to be a genetically heterogeneous disease; an autosomaldominant pattern has been reported in the literature, with 30% to 40% of patients reporting a family history of HS (Alikhan et al., 2009; Revuz, 2010). Effects on QOL 3. What are the effects of HS on a patient’s QOL? It is not surprising that HS may have a negative impact on a patient’s QOL, given the clinical characteristics and chronicity. Characteristics of HS that impact QOL include the chronicity of the disease, the lack of satisfactory treatment options, and the intimate locations of lesions (Lee et al., 2007; Revuz et al., 2008; Wolkenstein, Loundou, Barrau, Auquier, & Revuz, 2007). On average, patients experience symptoms of HS for about 18 to 20 years and often receive initial misdiagnoses, leading to poor or inappropriate treatment that may add to the disease burden (Poli et al., 2006). HS is recurrent in nature, often necessitating repeated absences from work or school because of symptoms such as pain, malodorous discharge, and July/August 2015
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the unsightly appearance of lesions (Onderdijk et al., 2013). Because HS occurs primarily in the axilla, groin, and mammary areas, patients commonly report feeling embarrassed and self-conscious, with the ano-genital regions producing the most impairment on QOL and interpersonal relationships (Matusiak, Bieniek, & Szepietowski, 2010; Wolkenstein, 2006). Multiple studies have confirmed that many patients with HS commonly experience depression as a result of their condition (Dufour, Emtestam, & Jemec, 2014; Esmann & Jemec, 2011; Matusiak et al., 2010; Wolkenstein et al., 2007). For example, Esmann and Jemec (2011) interviewed patients using a qualitative methodology, which revealed that HS has a significant psychosocial impact. Patients reported feeling ‘‘unworthy’’ and ‘‘unlovable’’ and described their lesions as ‘‘ugly, smelly, and embarrassing.’’ Studies using the DLQI as a quantitative measure have shown that HS has a greater negative impact on QOL than other chronic dermatologic conditions such as alopecia and psoriasis. QOL impact, as measured with the DLQI, is correlated with disease severity, as well other specific factors such as the frequency of flares, the amount of sick days taken, and the extent of pain and drainage from lesions (Dufour et al., 2014; Matusiak et al., 2010; Onderdijk et al., 2013; Wolkenstein, 2006). Role of the NP in Treating Patients With HS 4. What is the role of the primary care NP in the treatment of patients with HS? HS represents a management challenge, not only for specialists in dermatology and surgery, but also for primary care providers and clinicians who see patients in urgent and emergency care settings (Alikhan et al., 2009; Vazquez et al., 2012). A multidisciplinary approach involving primary care, dermatology, plastic surgery, and other specialties is often indicated (Lee et al., 2007). Because relapses and flares are common in persons with HS and no single definitive therapy is available, an array of treatment modalities may be indicated depending on the disease severity and patient presentation (Alikhan et al., 2009). Given the chronic nature of the condition, primary care NPs play a key role by providing a patientcentered medical home, thus ensuring continuous and comprehensive care to maximize outcomes. For adolescents and young adults with HS, comprehensive care will entail providing supportive care and addressing QOL issues, as discussed later. Other aspects of the NP’s role may include coordination with specialists, as well as screening for and management of comorbidities. In terms of supportive care, a number of recommendations may prove to be helpful for patients with HS regardless of disease severity. Cessation of smoking for patients who smoke cigarettes should be encouraged, using multidisciplinary interventions as indi368
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cated. Patients should be counseled regarding weight loss and the maintenance of a healthy weight. Other general measures include advising patients to wear soft or friction-free undergarments and clothing, including the avoidance of tight jeans (Alikhan et al., 2009; Slade et al., 2003). Given the documented effect of HS on QOL, assessment and management of QOL issues is an important aspect of the primary care provider’s role in the care of patients with HS. Providers are advised to assess pain using a visual analog scale and to further assess QOL with use of the DLQI (Wolkenstein, 2006). Screening for depression can be performed with use of a number of commonly available and validated screening tools, such as the PHQ-2 and PHQ-9 (Maurer, 2012). Patients should be counseled on stress management and referred to a support group if one is available (Alikhan et al., 2009; Mortimer & Lunniss, 2000; Slade et al., 2003). The effect of HS on a patient’s psychosocial well-being may warrant referrals for individual counseling and/or psychiatric care if indicted (Rambhatla, Lim, & Hamzavi, 2012). Treatment Options 5. What are the best available treatment options for HS? Treatment of HS should be undertaken in a stepwise fashion depending upon disease severity (Ingram et al., 2012). Management is tailored according to the severity and distribution of HS lesions, taking into account QOL issues with attention to the risks and benefits of any treatment. Therapies recommended for patients with Hurley stage I disease include topical antibiotics, a short course of systemic antibiotics, intralesional steroids, and adjuvant treatment with zinc gluconate. Secondline therapies recommended for patients with Hurley stage II disease include long-term antibiotic therapy, CO2 laser ablation, immunosuppressive therapies, limited surgical excision, and radiation therapy. For patients presenting with stage III disease, authors recommend largely palliative treatment with immunosuppressive therapy, as well as surgical treatment with wide surgical excision of the affected regions with or without radiation therapy (Alikhan et al., 2009; Jemec & Revuz, 2006; Rambhatla et al., 2012; Revuz, 2010). Topical antibiotic treatment with clindamycin has been used with success and is the best-studied topical medication. When specific pathogens, such as anaerobes, are isolated, antibiotic therapy should be targeted based on the resulting sensitivities. However, the indicated duration of treatment is not standardized (Oprica & Nord, 2006). Oral antibiotic treatment regimens that have been studied retrospectively include combination therapy with clindamycin and rifampin, a combination that is effective in treating other follicular occlusion disorders. This regimen has been shown effective in significantly reducing patients’ Sartorius scores, although adverse gastrointestinal effects caused Journal of Pediatric Health Care
patients to discontinue therapy in all studies (Gener et al., 2009; Jemec & Revuz, 2006; Mendonca & Griffiths, 2006; van der Zee, Boer, Prens, & Jemec, 2009). Other nonsurgical treatment options include retinoids, which have been considered for use in HS treatment because of their efficacy in the treatment of acne. However, studies to date show a lack of efficacy for patients with HS who take oral isotretinoin. Hormonal therapy, specifically antiandrogen therapy, has been subject to several trials with mixed results. Immunosuppression with systemic steroids has also been used because of the nonspecific anti-inflammatory effects that may prove useful during the inflammatory stages of HS. Although case reports have shown improvement with the use of drugs such as cyclosporine, relapse tends to occur after discontinuation of these medications (Alikhan et al., 2009; Rambhatla et al., 2012). Surgical management, specifically wide and radical excision, is considered the gold standard for severe or intractable HS. Surgical interventions should be initiated in a multidisciplinary setting, Surgical allowing optimal management, preoperative and postspecifically wide operative treatment. Interventions range and radical from local excision to excision, is wide or radical exciconsidered the sion with healing by secondary intention or gold standard for grafting, with the severe or choice of surgical techintractable HS. nique depending upon the region and disease severity. In addition to more extensive surgeries, simple incision and drainage of select lesions may be indicated at intervals for temporary relief, as was performed with this patient in the ED (Alikhan et al., 2009; Lapins & Emtestam, 2006; Revuz, 2010). FOLLOW-UP/OUTCOMES The patient was discharged the following day, with an appointment having been scheduled with dermatology. Further patient education regarding supportive care and treatment of HS was provided by the pediatric hospitalist. Prescriptions were issued for oral ibuprofen and trimethoprim/sulfamethoxazole tablets. The patient was also scheduled to follow up with her primary care provider for outpatient management of HS. Primary care provider follow-up was further advised for evaluation of microcytic anemia, because the diagnosis of iron deficiency anemia requires laboratory evidence of anemia and of low iron stores, such as through decreased serum ferritin levels (Short & Domagalski, 2013; Van Vranken, 2010). The patient also requires additional laboratory testing for obesitywww.jpedhc.org
related medical conditions, such as hyperlipidemia, type 2 diabetes mellitus, and nonalcoholic fatty liver disease. In addition to the recommended laboratory evaluation, the patient requires assessment of behavioral risks related to obesity such as current eating habits and sedentary behaviors before the initiation of multidisciplinary interventions. Although the patient’s screening for depression with use of the PHQ-2 was negative, depression is an important comorbidity of obesity and of HS. Therefore, depression screening may be repeated using instruments developed for primary care such as the Patient Health Questionnaire for Adolescents or the Beck Depression Inventory–Primary Care Version, both of which have been used successfully in adolescents (Maurer, 2012; U.S. Preventive Services Task Force, 2009). CONCLUSION In summary, HS is a chronic, recurrent skin disease affecting areas of apocrine gland–bearing skin that affects mainly females after the onset of puberty, with large variations in clinical severity and presentation. Although HS is not uncommon, it remains an orphan disease that is not well known or understood by medical providers, leading to delays in diagnosis and initiation of treatment, as occurred with this patient. The diagnosis is made clinically with no confirmatory test. HS significantly affects patients’ QOL because of its chronic nature, along with the lack of satisfactory treatments. As this case study highlights, management ought to be multidisciplinary, taking into account QOL, along with disease severity and patient preference, in conjunction with specialties including plastic surgery and dermatology. We thank Dr. David Listman for his assistance in seeking Institutional Review Board approval for this case study. We also extend our gratitude to Dr. Rita Marie John for our educations as pediatric nurse practitioners. REFERENCES Alikhan, A., Lynch, P. J., & Eisen, D. B. (2009). Hidradenitis suppurativa: A comprehensive review. Journal of the American Academy of Dermatology, 60(4), 539-561. Barlow, S. E. (2007). Expert Committee recommendations regarding the prevention, assessment, and treatment of child and adolescent overweight and obesity: Summary report. Pediatrics, 120(Suppl. 4), S164-S192. Canoui-Poitrine, F., Revuz, J. E., Wolkenstein, P., Viallette, C., Gabison, G., Pouget, F., . Bastuji-Garin, S. (2009). Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity. Journal of the American Academy of Dermatology, 61, 51-57. Dufour, D. N., Emtestam, L., & Jemec, G. B. (2014). Hidradenitis suppurativa: A common and burdensome, yet under-recognised,
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Journal of Pediatric Health Care