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Clinical Pearls in Dermatology
CONCISE REVIEW CLINICIANS CLINICALFOR PEARLS IN DERMATOLOGY
Clinical Pearls in Dermatology Mark Denis P. Davis, MD; John B. Bundrick, MD; and Scott C. Litin, MD
A
t the 2001 annual conference of the American College of Physicians, a new teaching format to aid physician learning, Clinical Pearls, was introduced. Clinical Pearls is designed with the 3 qualities of physicianlearners in mind. First, we physicians enjoy learning from cases. Second, we like concise, practical points that we can use in our practice. Finally, we take pleasure in problem solving. In the Clinical Pearls format, speakers present a number of short cases in their specialty to a general internal medicine audience. Each case is followed by a multiple-choice question answered live by attendees using an audience response system. The answer distribution is shown to attendees. The correct answer is then displayed and the speaker discusses teaching points, clarifying why one answer is most appropriate. Each case presentation ends with a $MJOJDBM1FBSM, defined as a practical teaching point that is supported by the literature but generally not well known to most internists. Clinical Pearls is currently one of the most popular sessions at the American College of Physicians meeting. As a service to its readers, .BZP$MJOJD1SPDFFEJOHT has invited a selected number of these Clinical Pearl presentations to be published in our Concise Reviews for Clinicians section. “Clinical Pearls in Dermatology” is one of them. CASE 1 A patient presents with a 1-year history of intermittently painful feet and legs. The pain is associated with marked flushing of the legs: they are bright red and hot to the touch during episodes of pain. QUESTION Which POF of the following would be the NPTUBQQSPQSJBUF next test? a. Complete blood cell count b. Hemoglobin A1c assay c. Calcium and phosphate assay d. Parathyroid hormone assay e. Electromyography DISCUSSION The patient has erythromelalgia (for photographs of conditions discussed, see Supporting Online Material; eFigure 1). Erythromelalgia is a clinical syndrome
characterized by intermittently hot and red extremities. The lower extremities are involved more frequently than are the upper extremities, and involvement is often symmetrical. The pain can be severe and debilitating.1 Patients may go to great lengths to cool the affected areas during symptoms. Primary and secondary forms of erythromelalgia have been described. Polycythemia vera and other myeloproliferative disorders may underlie the secondary form. 2 Accumulating evidence suggests that erythromelalgia may be a form of smallfiber neuropathy.3 Treatment is difficult; aspirin, nonsteroidal anti-inflammatory agents, anticonvulsant agents such as gabapentin, tricyclic antidepressants, and vasoactive drugs have all been tried. Response varies. Over time, the symptoms can worsen, stay the same, improve, or resolve in approximately equal proportions.1 The most appropriate test for this patient would be a complete blood cell count. In the setting of erythromelalgia, the possibility of an underlying myeloproliferative disease needs to be considered. In this setting, the condition is also more likely to respond to aspirin.2 Diabetes is not associated with erythromelalgia, and so a hemoglobin A1c assay would not be helpful. Calcium, phosphate, and parathyroid hormone levels are not relevant to erythromelalgia. Because muscles are not involved in erythromelalgia, electromyography would not be helpful. CLINICAL PEARL Myeloproliferative disease can underlie erythromelalgia. The importance of making this diagnosis is that this form of erythromelalgia may respond to aspirin. CASE 2 A patient receiving dialysis for chronic renal failure suddenly develops extraordinarily tender patches of skin involving areas on her legs, thighs, and abdominal pannus. These areas are discolored and rock-hard to the touch, and the skin appears ischemic. Later, painful ulcerations appear. From the Department of Dermatology (M.D.P.D.) and Division of General Internal Medicine (J.B.B., S.C.L), Mayo Clinic, Rochester, MN. Individual reprints of this article are not available. Address correspondence to Mark Denis P. Davis, MD, Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 ([email protected]). © 2010 Mayo Foundation for Medical Education and Research
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
855
CLINICAL PEARLS IN DERMATOLOGY
QUESTION Which POF of the following statements is NPTUBDDVSBUF about this condition? a. Corticosteroids are the treatment of choice b. Surgical excision of the affected areas should be immediately performed c. The prognosis for patients with this condition is dismal, and no treatment consistently works d. Treatment with sodium thiosulfate leads to a predictable improvement in this condition e. Parathyroidectomy will usually be curative DISCUSSION This patient has calciphylaxis (eFigure 2). Calciphylaxis is a rare and ill-understood condition involving the skin, characterized by arteriolar calcification, thrombosis, and consequent skin infarction. Patients present with extraordinarily painful subcutaneous patches of skin on their extremities and pannus: the overlying skin appears ischemic and then infarcts with resultant exquisitely tender ulcerations. The etiology is not understood. Although most commonly seen in the context of chronic renal failure in patients receiving dialysis, it may be seen in other settings such as liver disease. The prognosis is dismal: in a retrospective study of 64 patients with calciphylaxis, the estimated 1-year survival rate was 45.8%, and the 2-year survival rate was 20.0%.4 Currently described treatment approaches focus on correction of calcium and phosphate levels, but no treatment has consistently been demonstrated to be effective for this condition. Corticosteroids are not generally helpful. Patients who have had the ulcerated area surgically debrided may have a better outcome than those who do not; excision of the affected area has not been reported to help. No treatment consistently works. Sodium thiosulfate has recently been reported in case reports to be helpful but does not lead to a predictable improvement in the condition. Whether parathyroidectomy is an appropriate treatment of calciphylaxis remains a matter of debate; in some reports, it is associated with prolonged survival, whereas in others it is not. In a recent report from Mayo Clinic, survival rates were similar for 16 patients who underwent parathyroidectomy and 47 who did not.4 CLINICAL PEARL Calciphylaxis is a diagnosis with a dismal prognosis and no clearly effective treatments. CASE 3 A 39-year-old man with advanced human immunodeficiency virus infection/AIDS presents to the emergency 856
department with severe itching of 3 weeks' duration. He reports that the itching keeps him awake at night, and he rates the itching as 10 of 10 in severity. He has not been adherent to his antiretroviral therapy. Because of ongoing skin itching, he is drinking excessively. He has also been taking diphenhydramine every 4 hours. This practice has apparently increased during the past few days. Although he denies any active suicidal ideation, he appears to be depressed and often has thoughts of “ending it all.” His support structure is poor. On examination, he is constantly scratching his skin. There is blood on his undershorts. His skin is extremely dry. Scattered brown patches are noted on his extremities. The scrotum appears lichenified. QUESTION Which POF of the following is the NPTUJNQPSUBOU next test in the investigation of this patient's pruritus? a. Measurement of viral load b. CD4 count c. Skin biopsy d. Skin scraping for scabies mite e. Liver function tests (aspartate aminotransferase, alanine aminotransferase, and direct bilirubin) DISCUSSION In cases of extreme pruritus, scabies should always be considered in the differential diagnosis; treatment is very effective for this condition. In this patient, scabies preparation was positive, and scabies was diagnosed. Treatment with ivermectin and with topical permethrin was initiated. Many other causes of pruritus exist. Severe itching should also prompt a search for any evidence of dermatitis herpetiformis; underlying liver, kidney or thyroid disease; and underlying malignancy. Dry skin is a very common cause of itching. In the setting of human immunodeficiency virus infection, conditions such as eosinophilic folliculitis should also be considered. All the listed tests could give clues to the etiology of pruritus. However, when faced with a patient with very severe itching, scabies should always be considered as a possibility.5 Measurement of viral load and CD4 count are certainly important in this patient but will not alone give a clue as to the cause of itching. A skin biopsy may be unrevealing in the setting of scabies, unless the scabies mite happens to be included in the biopsy specimen. Liver function tests would be important but did not lead to the diagnosis in this patient. CLINICAL PEARL Always suspect scabies in any patient with severe pruritus.
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
CLINICAL PEARLS IN DERMATOLOGY
CASE 4 You are asked to see a 58-year-old woman who has a 5-month history of multiple ulcerations and abscesses involving both breasts. These started after breast reduction surgery that was performed because of pendulous breasts. The ulcerations began at the surgical incision sites 3 weeks postoperatively. Her history is remarkable for treated hypertension and quiescent ulcerative colitis. The patient has had multiple admissions to plastic surgery for incision and drainage of breast abscesses and has had several short admissions to the infectious diseases service for treatment with intravenous antibiotics. Each of these led to temporary improvement in the ulcerations. However, despite these hospital admissions for intravenous antibiotics and surgical debridement, the ulcerations progressed. The patient had no history of ulcerations before the breast reduction surgery. QUESTION Which POF of the following treatments would NPTU MJLFMZ result in resolution of these abscesses? a. Nasal swab for methicillin-resistant 4UBQIZMPDPDDVT BVSFVT and eradication of carrier state with linezolid b. Bilateral mastectomy c. Culture of swab obtained from the affected area; longterm suppressive oral antibiotic treatment depending on antimicrobial susceptibilities d. Oral prednisone e. Skin grafting of ulcerations DISCUSSION The patient was diagnosed as having pyoderma gangrenosum involving the breasts (eFigure 3). The clues to the diagnosis were the occurrence of pathergy (ulcerations occurred at the incision sites), the characteristic clinical appearance, and a history of a characteristic underlying disease (ulcerative colitis). Pyoderma gangrenosum is characterized by ulcerations, frequently with a characteristic clinical appearance, that occur spontaneously or are associated with pathergy.6 Diagnosis of the condition is important because it responds well to treatment with systemic corticosteroids and other immunosuppressive regimens. In this case of pyoderma gangrenosum involving the breasts, aggressive debridement, intravenous antibiotics, and repeated hospitalizations had only resulted in more extensive ulcerations. For this patient, the treatment par excellence would be systemic corticosteroids. Detection and treatment of methicillinresistant 4BVSFVT or other infection had already been done and had only led to temporary improvement in the condition. Bilateral mastectomy would be the worst option in the setting of pyoderma gangrenosum: pyoderma gangrenosum would occur at the surgical scars and progress over the chest because of pathergy (ulcerations occurring at the incision sites).
CLINICAL PEARL Pyoderma gangrenosum should be suspected in patients developing recurrent ulcerations with raised, overhanging, purple edges and may be precipitated by surgery and surgical debridement. Systemic corticosteroids and other immunosuppressive regimens are needed. CASE 5 A 54-year-old woman presents with a 6-month history of bilateral, painful reddened lower legs. She reports that her legs feel tight. She has been hospitalized on 3 occasions for recurrent cellulitis of the legs. She notices that her legs improve when she is in the hospital but soon become red again after discharge. On examination, she is obese. Both legs are affected from the knees downward; circumferentially, the skin is red, feels slightly warm to the touch, and is indurated. QUESTION Which POF of the following would be the NPTUBQQSPQSJBUF approach to management of this patient's condition? a. Hospitalization for intravenous antibiotics b. Initiation of cephalexin for her intractable cellulitis c. Unna boot d. Knee-high support stockings, 30 to 40 mm Hg e. Oral corticosteroids DISCUSSION This patient has lipodermatosclerosis (eFigure 4). Lipodermatosclerosis describes bound-down, sclerotic skin involving the lower extremities. Other commonly used terms to describe this entity include QPTUQIMFCJUJD MJNC TZOESPNF, IZQPEFSNJUJT TDMFSPEFSNBGPSNJT, and TDMFSPTJOH QBOOJDV MJUJs. In the past, it has also been called JOEVSBUFEDFMMVMJUJT or DISPOJD DFMMVMJUJT because it was mistakenly thought to represent the result of recurrent episodes of cellulitis. Lipodermatosclerosis is associated with female sex, middle age, high body mass index, and venous abnormalities.7 Compression therapy is the most commonly accepted management. These patients are often misdiagnosed as having cellulitis and are hospitalized; cellulitis that is bilateral would be extremely unusual. Intravenous or oral antibiotics will not help lipodermatosclerosis. Neither will systemic corticosteroids. An Unna boot could be helpful if it provided sufficient compression. The only treatment that has consistently been helpful in this condition is compression therapy: support stockings are the most common treatment used. CLINICAL PEARL Lipodermatosclerosis may be misdiagnosed as cellulitis.
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
857
CLINICAL PEARLS IN DERMATOLOGY
CASE 6 A patient comes to see you for help with recurrent herpes simplex labialis (eFigure 5). She experiences quite severe episodes twice a year, with a prodrome of tingling of her lip before the appearance of lesions. These episodes cause her discomfort and usually last 7 to 10 days. She thinks that they affect her job performance and worries that people often stare at her cold sores. She has no other medical problems. She would like easy-to-follow management suggestions. QUESTION Which POF of the following treatment recommendations would be NPTUBQQSPQSJBUF in this patient? a. When acute episodes occur, treat with 2 g of valacyclovir twice daily for 1 day b. Begin long-term suppressive therapy with acyclovir (200 mg orally 3 to 5 times a day) or valacyclovir (500 mg once daily) c. Use over-the-counter pain medications when episodes occur d. Apply penciclovir cream to lesions 4 times daily for 2 days e. Treat acute episodes with 400 mg of acyclovir orally once daily for 5 days DISCUSSION Recurrent labial herpes affects roughly one-third of the US population, and these patients typically experience 1 to 6 episodes per year. These infections appear at the vermilion border of the lip in about 90% of cases, the palate in 5% of cases, and elsewhere above the chin or on the oral mucosa more rarely. Papules on an erythematous base become vesicles within hours and subsequently progress through ulcerated, crusted, and healing stages within 72 to 96 hours. Before skin lesions appear, 60% of patients experience prodromal tingling, itching, and burning.8 Acyclovir, valacyclovir, and famciclovir are the 3 antiviral drugs routinely used to treat symptomatic herpes simplex virus (HSV) infections. Diagnosing HSV infections is usually straightforward in immunocompetent patients, and all the available drugs have an excellent margin of safety because they are converted by viral thymidine kinase to the active drug only inside virally infected cells. Unfortunately, confusion often arises because various dosing regimens are recommended. Treatments should be started within 48 hours of an outbreak. A supply of treatment medication or a prescription with instructions to initiate treatment should be given to the patient. For recurrent labial herpes simplex, intermittent episodic therapy with antiviral agents is recommended; systemic treatments are more effective than topical ones. Regimens approved by the US Food and Drug Administration (FDA) include 2 g of valacyclovir orally twice daily for 1 day or 1500 mg of famciclovir (three 500-mg tablets) in 1 dose.8 858
Both regimens are very easy to follow and convenient for patients. However, both agents are expensive. If insurance does not cover the valacyclovir, then consideration can be given to use 400 mg of oral acyclovir 5 times daily for 5 days. Topical regimens that are FDA-approved but less effective than the already discussed regimens for systemic drugs are topical application of penciclovir cream (1%) every 2 hours while waking and application of acyclovir cream (5%) 5 times daily for 4 days. Should this patient take suppressive therapy? The episodes occur only twice yearly, but they may be affecting her job performance. Suppressive therapy has been shown to reduce the frequency of recurrences by 70% to 80% in patients who have 6 or more recurrences per year. Treatment is also effective in patients with less frequent recurrences. Long-term suppressive therapy is generally useful in immunocompetent patients who experience recurrences more than every 2 to 4 months or in whom recurrences are associated with systemic complications or affect job performance. On balance, given that this patient's episodes are infrequent, suppressive therapy would not be recommended. Pain medications can be helpful but will not eliminate or shorten episodes. Painful oral and labial lesions associated with primary HSV-1 infection can be treated with viscous lidocaine. Oral opiates or intravenous hydration are generally reserved for severe cases. CLINICAL PEARL A dosing regimen of 2 g of valacyclovir orally twice daily for 1 day is an effective treatment for recurrent herpes simplex labialis and is easy and convenient for patients. REFERENCES 1. Davis MD, O’Fallon WM, Rogers RS III, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. "SDI %FSNBUPM. 2000;136(3):330-336. 2. Tefferi A. Polycythemia vera: a comprehensive review and clinical recommendations. Mayo Clin Proc. 2003;78(2):174-194. 3. Davis MD, Sandroni P, Rooke TW, Low PA. Erythromelalgia: vasculopathy, neuropathy, or both? a prospective study of vascular and neurophysiologic studies in erythromelalgia. "SDI%FSNBUPM. 2003;139(10):1337-1343. 4. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. +"N"DBE%FSNBUPM. 2007;56(4):569-579.. 5. Bouvresse S, Chosidow O. Scabies in healthcare settings. $VSS0QJO*OGFDU %JT. 2010;23(2):111-118. 6. Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. *OU+%FSNB UPM. 2004;43(11):790-800. 7. Bruce AJ, Bennett DD, Lohse CM, Rooke TW, Davis MD. Lipodermatosclerosis: review of cases evaluated at Mayo Clinic. + "N "DBE %FSNBUPM. 2002;46(2):187-192. 8. Cernik C, Gallina K, Brodell RT. The treatment of herpes simplex infections: an evidence-based review. "SDI*OUFSO.FE. 2008;168(11):1137-1144.
Correct answers: 1. a, 2. c, 3. d, 4. d, 5. d, 6. a Supporting Online Material www.mayoclinicproceedings.com/content/85/9/855/suppl/DC1 eFigures 1-5
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
Clinical Pearls in Dermatology Mark Denis P. Davis, MD; John B. Bundrick, MD; and Scott C. Litin, MD
A
t the 2001 annual conference of the American College of Physicians, a new teaching format to aid physician learning, Clinical Pearls, was introduced. Clinical Pearls is designed with the 3 qualities of physicianlearners in mind. First, we physicians enjoy learning from cases. Second, we like concise, practical points that we can use in our practice. Finally, we take pleasure in problem solving. In the Clinical Pearls format, speakers present a number of short cases in their specialty to a general internal medicine audience. Each case is followed by a multiple-choice question answered live by attendees using an audience response system. The answer distribution is shown to attendees. The correct answer is then displayed and the speaker discusses teaching points, clarifying why one answer is most appropriate. Each case presentation ends with a $MJOJDBM1FBSM, defined as a practical teaching point that is supported by the literature but generally not well known to most internists. Clinical Pearls is currently one of the most popular sessions at the American College of Physicians meeting. As a service to its readers, .BZP$MJOJD1SPDFFEJOHT has invited a selected number of these Clinical Pearl presentations to be published in our Concise Reviews for Clinicians section. “Clinical Pearls in Dermatology” is one of them. CASE 1 A patient presents with a 1-year history of intermittently painful feet and legs. The pain is associated with marked flushing of the legs: they are bright red and hot to the touch during episodes of pain. QUESTION Which POF of the following would be the NPTUBQQSPQSJBUF next test? a. Complete blood cell count b. Hemoglobin A1c assay c. Calcium and phosphate assay d. Parathyroid hormone assay e. Electromyography DISCUSSION The patient has erythromelalgia (for photographs of conditions discussed, see Supporting Online Material; eFigure 1). Erythromelalgia is a clinical syndrome
characterized by intermittently hot and red extremities. The lower extremities are involved more frequently than are the upper extremities, and involvement is often symmetrical. The pain can be severe and debilitating.1 Patients may go to great lengths to cool the affected areas during symptoms. Primary and secondary forms of erythromelalgia have been described. Polycythemia vera and other myeloproliferative disorders may underlie the secondary form. 2 Accumulating evidence suggests that erythromelalgia may be a form of smallfiber neuropathy.3 Treatment is difficult; aspirin, nonsteroidal anti-inflammatory agents, anticonvulsant agents such as gabapentin, tricyclic antidepressants, and vasoactive drugs have all been tried. Response varies. Over time, the symptoms can worsen, stay the same, improve, or resolve in approximately equal proportions.1 The most appropriate test for this patient would be a complete blood cell count. In the setting of erythromelalgia, the possibility of an underlying myeloproliferative disease needs to be considered. In this setting, the condition is also more likely to respond to aspirin.2 Diabetes is not associated with erythromelalgia, and so a hemoglobin A1c assay would not be helpful. Calcium, phosphate, and parathyroid hormone levels are not relevant to erythromelalgia. Because muscles are not involved in erythromelalgia, electromyography would not be helpful. CLINICAL PEARL Myeloproliferative disease can underlie erythromelalgia. The importance of making this diagnosis is that this form of erythromelalgia may respond to aspirin. CASE 2 A patient receiving dialysis for chronic renal failure suddenly develops extraordinarily tender patches of skin involving areas on her legs, thighs, and abdominal pannus. These areas are discolored and rock-hard to the touch, and the skin appears ischemic. Later, painful ulcerations appear. From the Department of Dermatology (M.D.P.D.) and Division of General Internal Medicine (J.B.B., S.C.L), Mayo Clinic, Rochester, MN. Individual reprints of this article are not available. Address correspondence to Mark Denis P. Davis, MD, Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 ([email protected]). © 2010 Mayo Foundation for Medical Education and Research
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
855
CLINICAL PEARLS IN DERMATOLOGY
QUESTION Which POF of the following statements is NPTUBDDVSBUF about this condition? a. Corticosteroids are the treatment of choice b. Surgical excision of the affected areas should be immediately performed c. The prognosis for patients with this condition is dismal, and no treatment consistently works d. Treatment with sodium thiosulfate leads to a predictable improvement in this condition e. Parathyroidectomy will usually be curative DISCUSSION This patient has calciphylaxis (eFigure 2). Calciphylaxis is a rare and ill-understood condition involving the skin, characterized by arteriolar calcification, thrombosis, and consequent skin infarction. Patients present with extraordinarily painful subcutaneous patches of skin on their extremities and pannus: the overlying skin appears ischemic and then infarcts with resultant exquisitely tender ulcerations. The etiology is not understood. Although most commonly seen in the context of chronic renal failure in patients receiving dialysis, it may be seen in other settings such as liver disease. The prognosis is dismal: in a retrospective study of 64 patients with calciphylaxis, the estimated 1-year survival rate was 45.8%, and the 2-year survival rate was 20.0%.4 Currently described treatment approaches focus on correction of calcium and phosphate levels, but no treatment has consistently been demonstrated to be effective for this condition. Corticosteroids are not generally helpful. Patients who have had the ulcerated area surgically debrided may have a better outcome than those who do not; excision of the affected area has not been reported to help. No treatment consistently works. Sodium thiosulfate has recently been reported in case reports to be helpful but does not lead to a predictable improvement in the condition. Whether parathyroidectomy is an appropriate treatment of calciphylaxis remains a matter of debate; in some reports, it is associated with prolonged survival, whereas in others it is not. In a recent report from Mayo Clinic, survival rates were similar for 16 patients who underwent parathyroidectomy and 47 who did not.4 CLINICAL PEARL Calciphylaxis is a diagnosis with a dismal prognosis and no clearly effective treatments. CASE 3 A 39-year-old man with advanced human immunodeficiency virus infection/AIDS presents to the emergency 856
department with severe itching of 3 weeks' duration. He reports that the itching keeps him awake at night, and he rates the itching as 10 of 10 in severity. He has not been adherent to his antiretroviral therapy. Because of ongoing skin itching, he is drinking excessively. He has also been taking diphenhydramine every 4 hours. This practice has apparently increased during the past few days. Although he denies any active suicidal ideation, he appears to be depressed and often has thoughts of “ending it all.” His support structure is poor. On examination, he is constantly scratching his skin. There is blood on his undershorts. His skin is extremely dry. Scattered brown patches are noted on his extremities. The scrotum appears lichenified. QUESTION Which POF of the following is the NPTUJNQPSUBOU next test in the investigation of this patient's pruritus? a. Measurement of viral load b. CD4 count c. Skin biopsy d. Skin scraping for scabies mite e. Liver function tests (aspartate aminotransferase, alanine aminotransferase, and direct bilirubin) DISCUSSION In cases of extreme pruritus, scabies should always be considered in the differential diagnosis; treatment is very effective for this condition. In this patient, scabies preparation was positive, and scabies was diagnosed. Treatment with ivermectin and with topical permethrin was initiated. Many other causes of pruritus exist. Severe itching should also prompt a search for any evidence of dermatitis herpetiformis; underlying liver, kidney or thyroid disease; and underlying malignancy. Dry skin is a very common cause of itching. In the setting of human immunodeficiency virus infection, conditions such as eosinophilic folliculitis should also be considered. All the listed tests could give clues to the etiology of pruritus. However, when faced with a patient with very severe itching, scabies should always be considered as a possibility.5 Measurement of viral load and CD4 count are certainly important in this patient but will not alone give a clue as to the cause of itching. A skin biopsy may be unrevealing in the setting of scabies, unless the scabies mite happens to be included in the biopsy specimen. Liver function tests would be important but did not lead to the diagnosis in this patient. CLINICAL PEARL Always suspect scabies in any patient with severe pruritus.
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
CLINICAL PEARLS IN DERMATOLOGY
CASE 4 You are asked to see a 58-year-old woman who has a 5-month history of multiple ulcerations and abscesses involving both breasts. These started after breast reduction surgery that was performed because of pendulous breasts. The ulcerations began at the surgical incision sites 3 weeks postoperatively. Her history is remarkable for treated hypertension and quiescent ulcerative colitis. The patient has had multiple admissions to plastic surgery for incision and drainage of breast abscesses and has had several short admissions to the infectious diseases service for treatment with intravenous antibiotics. Each of these led to temporary improvement in the ulcerations. However, despite these hospital admissions for intravenous antibiotics and surgical debridement, the ulcerations progressed. The patient had no history of ulcerations before the breast reduction surgery. QUESTION Which POF of the following treatments would NPTU MJLFMZ result in resolution of these abscesses? a. Nasal swab for methicillin-resistant 4UBQIZMPDPDDVT BVSFVT and eradication of carrier state with linezolid b. Bilateral mastectomy c. Culture of swab obtained from the affected area; longterm suppressive oral antibiotic treatment depending on antimicrobial susceptibilities d. Oral prednisone e. Skin grafting of ulcerations DISCUSSION The patient was diagnosed as having pyoderma gangrenosum involving the breasts (eFigure 3). The clues to the diagnosis were the occurrence of pathergy (ulcerations occurred at the incision sites), the characteristic clinical appearance, and a history of a characteristic underlying disease (ulcerative colitis). Pyoderma gangrenosum is characterized by ulcerations, frequently with a characteristic clinical appearance, that occur spontaneously or are associated with pathergy.6 Diagnosis of the condition is important because it responds well to treatment with systemic corticosteroids and other immunosuppressive regimens. In this case of pyoderma gangrenosum involving the breasts, aggressive debridement, intravenous antibiotics, and repeated hospitalizations had only resulted in more extensive ulcerations. For this patient, the treatment par excellence would be systemic corticosteroids. Detection and treatment of methicillinresistant 4BVSFVT or other infection had already been done and had only led to temporary improvement in the condition. Bilateral mastectomy would be the worst option in the setting of pyoderma gangrenosum: pyoderma gangrenosum would occur at the surgical scars and progress over the chest because of pathergy (ulcerations occurring at the incision sites).
CLINICAL PEARL Pyoderma gangrenosum should be suspected in patients developing recurrent ulcerations with raised, overhanging, purple edges and may be precipitated by surgery and surgical debridement. Systemic corticosteroids and other immunosuppressive regimens are needed. CASE 5 A 54-year-old woman presents with a 6-month history of bilateral, painful reddened lower legs. She reports that her legs feel tight. She has been hospitalized on 3 occasions for recurrent cellulitis of the legs. She notices that her legs improve when she is in the hospital but soon become red again after discharge. On examination, she is obese. Both legs are affected from the knees downward; circumferentially, the skin is red, feels slightly warm to the touch, and is indurated. QUESTION Which POF of the following would be the NPTUBQQSPQSJBUF approach to management of this patient's condition? a. Hospitalization for intravenous antibiotics b. Initiation of cephalexin for her intractable cellulitis c. Unna boot d. Knee-high support stockings, 30 to 40 mm Hg e. Oral corticosteroids DISCUSSION This patient has lipodermatosclerosis (eFigure 4). Lipodermatosclerosis describes bound-down, sclerotic skin involving the lower extremities. Other commonly used terms to describe this entity include QPTUQIMFCJUJD MJNC TZOESPNF, IZQPEFSNJUJT TDMFSPEFSNBGPSNJT, and TDMFSPTJOH QBOOJDV MJUJs. In the past, it has also been called JOEVSBUFEDFMMVMJUJT or DISPOJD DFMMVMJUJT because it was mistakenly thought to represent the result of recurrent episodes of cellulitis. Lipodermatosclerosis is associated with female sex, middle age, high body mass index, and venous abnormalities.7 Compression therapy is the most commonly accepted management. These patients are often misdiagnosed as having cellulitis and are hospitalized; cellulitis that is bilateral would be extremely unusual. Intravenous or oral antibiotics will not help lipodermatosclerosis. Neither will systemic corticosteroids. An Unna boot could be helpful if it provided sufficient compression. The only treatment that has consistently been helpful in this condition is compression therapy: support stockings are the most common treatment used. CLINICAL PEARL Lipodermatosclerosis may be misdiagnosed as cellulitis.
.BZP$MJO1SPDr4FQUFNCFS rEPJNDQrXXXNBZPDMJOJDQSPDFFEJOHTDPN
For personal use. Mass reproduce only with permission from Mayo a Clinic Proceedings.
857
CLINICAL PEARLS IN DERMATOLOGY
CASE 6 A patient comes to see you for help with recurrent herpes simplex labialis (eFigure 5). She experiences quite severe episodes twice a year, with a prodrome of tingling of her lip before the appearance of lesions. These episodes cause her discomfort and usually last 7 to 10 days. She thinks that they affect her job performance and worries that people often stare at her cold sores. She has no other medical problems. She would like easy-to-follow management suggestions. QUESTION Which POF of the following treatment recommendations would be NPTUBQQSPQSJBUF in this patient? a. When acute episodes occur, treat with 2 g of valacyclovir twice daily for 1 day b. Begin long-term suppressive therapy with acyclovir (200 mg orally 3 to 5 times a day) or valacyclovir (500 mg once daily) c. Use over-the-counter pain medications when episodes occur d. Apply penciclovir cream to lesions 4 times daily for 2 days e. Treat acute episodes with 400 mg of acyclovir orally once daily for 5 days DISCUSSION Recurrent labial herpes affects roughly one-third of the US population, and these patients typically experience 1 to 6 episodes per year. These infections appear at the vermilion border of the lip in about 90% of cases, the palate in 5% of cases, and elsewhere above the chin or on the oral mucosa more rarely. Papules on an erythematous base become vesicles within hours and subsequently progress through ulcerated, crusted, and healing stages within 72 to 96 hours. Before skin lesions appear, 60% of patients experience prodromal tingling, itching, and burning.8 Acyclovir, valacyclovir, and famciclovir are the 3 antiviral drugs routinely used to treat symptomatic herpes simplex virus (HSV) infections. Diagnosing HSV infections is usually straightforward in immunocompetent patients, and all the available drugs have an excellent margin of safety because they are converted by viral thymidine kinase to the active drug only inside virally infected cells. Unfortunately, confusion often arises because various dosing regimens are recommended. Treatments should be started within 48 hours of an outbreak. A supply of treatment medication or a prescription with instructions to initiate treatment should be given to the patient. For recurrent labial herpes simplex, intermittent episodic therapy with antiviral agents is recommended; systemic treatments are more effective than topical ones. Regimens approved by the US Food and Drug Administration (FDA) include 2 g of valacyclovir orally twice daily for 1 day or 1500 mg of famciclovir (three 500-mg tablets) in 1 dose.8 858
Both regimens are very easy to follow and convenient for patients. However, both agents are expensive. If insurance does not cover the valacyclovir, then consideration can be given to use 400 mg of oral acyclovir 5 times daily for 5 days. Topical regimens that are FDA-approved but less effective than the already discussed regimens for systemic drugs are topical application of penciclovir cream (1%) every 2 hours while waking and application of acyclovir cream (5%) 5 times daily for 4 days. Should this patient take suppressive therapy? The episodes occur only twice yearly, but they may be affecting her job performance. Suppressive therapy has been shown to reduce the frequency of recurrences by 70% to 80% in patients who have 6 or more recurrences per year. Treatment is also effective in patients with less frequent recurrences. Long-term suppressive therapy is generally useful in immunocompetent patients who experience recurrences more than every 2 to 4 months or in whom recurrences are associated with systemic complications or affect job performance. On balance, given that this patient's episodes are infrequent, suppressive therapy would not be recommended. Pain medications can be helpful but will not eliminate or shorten episodes. Painful oral and labial lesions associated with primary HSV-1 infection can be treated with viscous lidocaine. Oral opiates or intravenous hydration are generally reserved for severe cases. CLINICAL PEARL A dosing regimen of 2 g of valacyclovir orally twice daily for 1 day is an effective treatment for recurrent herpes simplex labialis and is easy and convenient for patients. REFERENCES 1. Davis MD, O’Fallon WM, Rogers RS III, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. "SDI %FSNBUPM. 2000;136(3):330-336. 2. Tefferi A. Polycythemia vera: a comprehensive review and clinical recommendations. Mayo Clin Proc. 2003;78(2):174-194. 3. Davis MD, Sandroni P, Rooke TW, Low PA. Erythromelalgia: vasculopathy, neuropathy, or both? a prospective study of vascular and neurophysiologic studies in erythromelalgia. "SDI%FSNBUPM. 2003;139(10):1337-1343. 4. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. +"N"DBE%FSNBUPM. 2007;56(4):569-579.. 5. Bouvresse S, Chosidow O. Scabies in healthcare settings. $VSS0QJO*OGFDU %JT. 2010;23(2):111-118. 6. Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. *OU+%FSNB UPM. 2004;43(11):790-800. 7. Bruce AJ, Bennett DD, Lohse CM, Rooke TW, Davis MD. Lipodermatosclerosis: review of cases evaluated at Mayo Clinic. + "N "DBE %FSNBUPM. 2002;46(2):187-192. 8. Cernik C, Gallina K, Brodell RT. The treatment of herpes simplex infections: an evidence-based review. "SDI*OUFSO.FE. 2008;168(11):1137-1144.
Correct answers: 1. a, 2. c, 3. d, 4. d, 5. d, 6. a Supporting Online Material www.mayoclinicproceedings.com/content/85/9/855/suppl/DC1 eFigures 1-5
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