- Email: [email protected]
Infectious eccrine hidradenitis caused by nocardia
Case reports 315
J AM ACAD DERMATOL VOLUME 50, NUMBER 2
11.
12.
13.
14.
15.
brous hyperplasia of the gingiva: a side effect of cyclosporin A therapy. Oral Surg Oral Med Oral Pathol 1983;55:274-8. Ashrafi SH, Slaski K, Thu K, Neiman ES, Cunningham MP. Scanning electron microscopy of cyclosporine-induced gingival overgrowth. Scanning Microsc 1996;10:219-25. Bencini PL, Montagnino G, Sala F, De Vecchi A, Crosti C, Tarantino A. Cutaneous lesions in 67 cyclosporin-treated renal transplant recipients. Dermatologica 1986;172:24-30. Reznik VM, Durham BL, Jones KL, Mendoza SA. Changes in facial appearance during cyclosporin treatment. Lancet 1987;1:1405-7. Busque S, Demers P, St-Louis G, Boily J-G, Tousignant J, Lemieux F, et al. Conversion from Neoral (cyclosporine) to tacrolimus of kidney transplant recipients for gingival hyperplasia or hypertrichosis. Transplant Proc 1998;30:1247-8. Hancock RH, Swan RH. Nifedipine-induced gingival overgrowth. J Clin Periodontol 1992;19:12-4.
16. Frauman AG. An overview of the adverse reactions to adrenal corticosteroids. Adverse Drug React Toxicol Rev 1996;15:203-6. 17. Jiang H, Yamamoto S, Kato R. Induction of anagen in telogen mouse skin by topical application of FK506, a potent immunosuppressant. J Invest Dermatol 1995;104:523-5. 18. Yamamoto S, Jiang H, Kato R. Stimulation of hair growth by topical application of FK506, a potent immunosuppressive agent. J Invest Dermatol 1994;102:160-4. 19. Takahashi T, Kamimura A. Cyclosporin A promotes hair epithelial cell proliferation and modulates protein kinase C expression and translocation in hair epithelial cells. J Invest Dermatol 2001; 117:605-11. 20. Fisher GJ, Duell EA, Nickoloff BJ, Annesley TM, Kowalke JK, Ellis CN, et al. Levels of cyclosporin in epidermis of treated psoriasis patients differentially inhibit growth of keratinocytes cultured in serum free versus serum containing media. J Invest Dermatol 1988;91:142-6.
Infectious eccrine hidradenitis caused by Nocardia Diana D. Antonovich, MD, Adrienne Berke, MD, Jane M. Grant-Kels, MD, and Maxwell Fung, MD Farmington, Connecticut Neutrophilic eccrine hidradenitis is a nonspecific clinical reaction pattern classified as a neutrophilic dermatosis that typically occurs in the setting of chemotherapy for hematologic malignant disease. Neutrophilic eccrine hidradenitis more rarely has been reported in association with infectious agents, including Serratia, Enterobacter, Staphylococcus, and HIV. We describe the first case of infectious eccrine hidradenitis occurring in a patient with cutaneous Nocardia infection. (J Am Acad Dermatol 2004;50:315-8.)
N
eutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis that most commonly occurs after administration of chemotherapy. The disorder was first reported, by Harris et al,1 with use of cytarabine to treat a patient with acute myelogenous leukemia. NEH has since been associated with diverse clinical conditions, occurring during use of both chemotherapeutic and nonchemotherapeutic agents,2-4 in immunocompromised and healthy persons,5-8 and infrequently in infection. Infectious NEH is rare, only 4 published reports documenting the presence of an infectious pathoFrom the Department of Dermatology, University of Connecticut Health Center. Funding sources: None. Conflict of interest: None identified. Reprint requests: Adrienne Berke, MD, University of Connecticut Health Center, 263 Farmington Ave, Farmington, CT 06030. Email: [email protected]. 0190-9622/$30.00 Copyright © 2004 by the American Academy of Dermatology, Inc. doi:10.1016/S0190-9622(03)02163-7
gen at the cutaneous eruption site.5,9-11 Five reports of NEH describing the ailment in HIV-infected patients have been published in the English literature.3,12-14 We describe the clinical and histopathologic findings of the first reported case of infectious eccrine hidradenitis (IEH) associated with Nocardia infection.
CASE REPORT An 83-year-old woman had a 2-day history of diffuse pain, tenderness, edema, and erythema of the right leg. The clinical impression was cellulitis. The patient was admitted to the hospital, and treatment with cefazolin was initiated. The medical history was significant for chronic obstructive pulmonary disease, mitral valve prolapse, and breast and endometrial cancer. She had undergone right lumpectomy with axillary node dissection 5 years earlier and total abdominal hysterectomy 15 years earlier. The patient’s current medications included tamoxifen, prednisone 12.5 mg orally once a day, and pulmonary inhalers as needed. With the excep-
316 Case reports
Fig 1. Neutrophils around and infiltrating the ductal epithelium. Luminal abscesses and swollen epithelial cells are evident. (Hematoxylin-eosin stain.)
tion of a serum white blood cell count of 22.9/L (normal, 4.5-11/L), results of hematological studies were within normal range. A chest radiograph was without infiltrates or evidence of acute disease. Although some improvement of the cellulitis was noted, on hospital day 5, multiple, tender, erythematous plaques and nodules involving the right thigh were observed. These lesions were speculated to be secondary to septic or cholesterol emboli from a cardiac source. An echocardiogram revealed mitral valve endocarditis with large vegetation. Under aseptic conditions, 2 biopsy specimens of the cutaneous lesions were obtained. One tissue specimen was cultured and the other was submitted for routine histologic examination. Findings at microscopic examination were consistent with NEH: a sparse superficial and deep perivascular and interstitial, polymorphous infiltrate of neutrophils, lymphocytes, and histiocytes. Neutrophils were present around and infiltrating the dermal eccrine ducts with extension in the ductal lumina (Fig 1). Acid-fast, Gram, and Gomori methenamine silver staining showed filamentous organisms within eccrine epithelial cells (Fig 2). Subsequent serial blood cultures were positive for Nocardia asteroides. Tissue culture confirmed the cutaneous presence of N asteroides. Treatment with trimethoprim-sulfamethoxazole resulted in complete resolution of the skin lesions within 3 weeks. The patient experienced no known recurrence.
DISCUSSION IEH was first described in 1985 by Moreno et al9 in a patient with chronic renal failure for which he received hemodialysis. That patient experienced recurrent eruptions of erythematous papules over a period of 3 years, until cutaneous biopsy showed features typical of NEH, and a tissue culture revealed the presence of Serratia organisms.
J AM ACAD DERMATOL FEBRUARY 2004
Fig 2. Left panel, Gomori methenamine stain shows positive staining of filamentous organisms in ductal epithelium (arrows). Right panel, Higher magnification of the organisms (arrows).
The authors speculated that this patient was immunocompromised owing to chronic renal failure, a condition that predisposed him to IEH from a nosocomial pathogen. IEH has subsequently been reported in a healthy, immunocompetent patient with Enterobacter cloacae as the offending microbe.5 The organism in that instance was identified by culture of an intact pustule. Skin biopsy confirmed IEH. The patient initially presented with a 2-month history of cutaneous lesions, which promptly resolved after 1 week of appropriate antibiotic therapy. A third report of IEH, associated with Staphylococcus aureusinfection, occurred in a man receiving immunosuppressive therapy after heart transplantation.10 Tissue cultures revealed S aureus infection, and the histologic findings were consistent with NEH. Again, after a prolonged history of recurrent eruptions, the lesions resolved within 5 days of initiation of antibiotic therapy. A second report of Serratiarelated IEH has been documented.11 Confirmation was made by tissue culture, histologic examination, and electron microscopic identification of bacteria within the lesions. This case of IEH occurred in a young man with spinal ependymoma, for which he received no chemotherapy. Although not clearly within the category of IEH, HIV infection has been associated with development of NEH. In 1990 Smith et al3 described 2 cases of NEH occurring in HIV-positive men who were taking zidovudine. The authors suggested the eruptions might have been the result of therapy with this agent, which has pharmacologic similarities to cytarabine. This line of thinking also was advanced regarding a case of NEH in an HIV-infected patient who had been receiving stavudine, a new reverse transcriptase inhibitor.12 However, in 2 additional cases of NEH, the HIVinfected patients were not receiving antiviral ther-
J AM ACAD DERMATOL VOLUME 50, NUMBER 2
apy at the time of eruption.13,14 In 4 of the 5 total cases reported in the English literature, special microbial stains were negative for organisms.3,12,13 It is not clear whether tissue cultures were performed in any of the cases. In each previously reported case of bacterial IEH, the offending organism was first appreciated with lesional culture. Our case was unique in that it represented the first case of IEH in which the infectious agent was detected histologically within the eccrine ductal epithelium and subsequently identified with tissue culture. It also represents the fifth reported case of IEH and the first report of IEH occurring with N asteroides infection. This organism is an aerobic, gram-positive, partially acid-fast, branching actinomycete and is a common inhabitant of soil.15 The organism is primarily an opportunistic pathogen, most frequently infecting immunocompromised persons.15 Inoculation of N asteroides occurs by inhalation of bacterial mycelia or by transcutaneous inoculation.16 The most common sites of infection include lung, brain, and skin, in decreasing order of frequency.16 First-line therapy for nocardiosis is treatment with trimethoprim-sulfamethoxazole for 3 months to lifelong use, depending on the overall health status of the patient.16,17 In our patient a chest radiograph showed no evidence of pulmonary infection. Furthermore, there was no known history of occupational exposure or traumatic transcutaneous inoculation. The source of cutaneous infection is unclear. It can be presumed that development of mitral valve vegetation preceded IEH, perhaps serving as the nidus of infection, although it is conceivable that the reverse is true. Why the infection occurred in association with NEH is unknown. Daily prednisone use is likely to have resulted in some degree of immunocompromise. The patient’s breast and endometrial carcinomas were both in remission, and she had not received chemotherapy. The skin eruptions resolved with appropriate antimicrobial therapy for nocardiosis. This outcome supports the hypothesis that an infectious agent had been the source of NEH. Although possible, it is less likely that an opportunistic infection developed and was superimposed on precedent, noninfectious NEH. Despite the increase in the number of cases since the condition was first described by Harris et al, the pathophysiologic mechanism of NEH remains obscure. Some authors have speculated that the cutaneous eruption is caused by a pharmacologic agent exerting a toxic effect on the eccrine gland or a reaction to an underlying malignan-
Case reports 317
cy.3,18,19 However, in light of the diversity of the clinical manifestations, these explanations are oversimplifications, not accounting for the occurrence of NEH in healthy persons or its association with infectious agents. Our case supports the contention that NEH is a characteristic cutaneous response to nonspecific stimuli and should be included in the differential diagnosis of a spectrum of clinical presentations. As has been emphasized by previous authors,12 when a case of NEH is being considered, the possibility of an infectious association must be appreciated and investigated by cutaneous tissue culture, because infectious and noninfectious NEH are generally indistinguishable histologically.10 Although noninfectious NEH is typically self-limited, necessitating no treatment, IEH must be treated with antibiotic therapy, a consideration that emphasizes the importance of differentiating the two conditions during diagnosis.
REFERENCES 1. Harrist TJ, Fine JD, Berman RS, Murphy GF, Mihm MC Jr. Neutrophilic eccrine hidradenitis: a distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy. Arch Dermatol 1982;118:263-6. 2. Kuttner BJ, Kurban RS. Neutrophilic eccrine hidradenitis in the absence of an underlying malignancy. Cutis 1988;41:403-5. 3. Smith KJ, Skelton HG 3rd, James WD, Holland TT, Lupton GP, Angritt P. Neutrophilic eccrine hidradenitis in HIV-infected patients. J Am Acad Dermatol 1990;23:945-7. 4. Bachmeyer C, Chaibi P, Aractingi S. NEH induced by granulocyte colony- stimulating factor. Br J Dermatol 1998;139:354-5. 5. Allegue F, Rocamora A, Martin-Gonzalez M, Alonso ML, Ledo A. Infectious eccrine hidradenitis. J Am Acad Dermatol 1990;22: 1119-20. 6. Manganoni AM, Facchetti F, Gavazzoni R, Marocolo D, De Panfilis G. Neutrophil eccrine hidradenitis in a healthy woman. Dermatology 1994;189:211-2. 7. Simon M, Cremer H, von den Driesch P. Idiopathic recurrent palmoplantar hidradenitis in children. Arch Dermatol 1998;134:76-9. 8. Buezo GF, Requena L, Fraga Fernandez J, Garcia Diez A, Fernandez Herrera JM. Idiopathic palmoplantar hidradenitis. Am J Dermatopathol 1996;18:413-6. 9. Moreno A, Barnadas MA, Ravella A, Moragas JM. Infectious eccrine hidradenitis in a patient undergoing hemodialysis. Arch Dermatol 1985;121:1106-7. 10. Taira JW, Gerber HB. Eccrine hidradenitis. Int J Dermatol 1992; 31:433-4. 11. Combemale P, Faisant M, Azoulay-Petit C, Dupin M, Kanitakis J. Neutrophilic eccrine hidradenitis secondary to infection with Serratia marcescens. Br J Dermatol 2000;142:784-88. 12. Krischer J, Rutschmann O, Roten SV, Harms M, Saurat JH, Pechere M. Neutrophilic eccrine hidradenitis in a patient with AIDS. J Dermatol 1998;25:199-200. 13. Sevila A, Morell A, Banuls J, Silvestre JF, Betlloch I. Neutrophilic eccrine hidradenitis in an HIV-infected patient. Int J Dermatol 1996;35:651-2.
318 Case reports
14. Bachmeyer C, Reygagne P, Aractingi S. Recurrent neutrophilic eccrine hidradenitis in an HIV-1-infected patient. Dermatology 2000;200:328-30. 15. Chazen G. Nocardia. Infect Control 1987;8:260-3. 16. Filice GA. Nocardiosis. In: Braunwald EW, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison’s principles of internal medicine. 15th ed. New York: McGraw-Hill; 2001. p. 1006-8.
J AM ACAD DERMATOL FEBRUARY 2004
17. Gilbert DN, Moellering RC, Sande MA. The Sanford guide to antimicrobial therapy. 30th ed. Hyde Park (VT): Antimicrobial Therapy, 2000. p. 76. 18. Hurt MA, Halvorson RD, Petr FC Jr, et al. Eccrine squamous syringometaplasia. Arch Dermatol 1990;126:73-7. 19. Vion B, Alvero H. Neutrophilic eccrine hidradenitis. Dermatologica 1991;183:70-2.
Viral-associated trichodysplasia in patients who are immunocompromised COL Leonard C. Sperling, MC, USA,a Maria-Magdalena Tomaszewski, MD,b and Douglas A. Thomas, MDc Bethesda, Maryland; Washington, District of Columbia; and Las Vegas, Nevada Viral-associated trichodysplasia of immunosuppression is a newly described clinicopathologic entity found in patients who are undergoing drug-induced immunosuppression to prevent organ transplant rejection. Patients have numerous erythematous papules concentrated in the central portion of the face and variable degrees of hair loss, most severely affecting facial hair. Histologic findings of facial papules are highly distinctive and unique, and suggest that the entire machinery of the follicular bulb is devoted to the manufacture of inner root sheath-type keratin. Electron microscopy reveals intranuclear viral particles, but precise viral identification has not yet been achieved. (J Am Acad Dermatol 2004;50:318-22.)
I
n 1999, Haycox et al1 described an individual who was immunocompromised with alopecia, facial papules, and unique histologic findings. This distinctive condition was called trichodysplasia spinulosa. Electron microscopy performed on a biopsy specimen revealed viral particles (38-nm diameter) suggestive of a papovavirus infection. However, an attempt to detect human papillomavirus subtypes using polymerase chain reaction was unsuccessful, even though 18 different viral subtypes were searched for using dot blot hybridization and consensus primer polymerase chain reaction. A test for BK virus, a polyoma virus, was also negative. BK virus was a likely suspect because of its size (ap-
From the Department of Dermatology, Uniformed Services University, Bethesdaa; the Department of Dermatopathology, Armed Forces Institute of Pathology, Washington, DCb; and Las Vegas Skin and Cancer Clinics, Ltd.c Supported by the Department of Dermatopathology, Armed Forces Institute of Pathology; and Department of Dermatology, Uniformed Services University. Conflicts of interest: None identified. Presented orally at the Las Vegas Dermatology Seminar, Las Vegas, Nev, November 16, 2002. Reprints not available from authors. doi:10.1016/S0190-9622(03)01490-7
proximately 45 nm) and its prevalence in patients who are immunocompromised and undergo organ transplant.2,3 We wish to confirm that trichodysplasia spinulosa is a distinctive and highly diagnosable condition by presenting an additional case. Our patient was a 13-year-old girl who had undergone kidney transplantation 9 months before skin disease developed. When her dermatosis began, she was taking prednisone, tacrolimus, and mycophenolate mofetil. During the course of several months a progressively worsening skin eruption developed consisting of erythematous, 1- to 3-mm papules concentrated in the midfacial area, especially the nose, malar region, glabella, and chin (Fig 1). Papules were so numerous and confluent that they distorted the contours of the nose. Some papules had a central, whitish, keratotic, spiny excrescence. There was almost total hair loss affecting the eyebrows and eyelashes, but the scalp was not affected. The lesions were mildly pruritic. Topical corticosteroids were tried without benefit, and a biopsy of a facial lesion was performed. The histologic findings were dramatic and unique, and effected most anagen hairs in the specimen. The affected follicles were very large, al-
J AM ACAD DERMATOL VOLUME 50, NUMBER 2
11.
12.
13.
14.
15.
brous hyperplasia of the gingiva: a side effect of cyclosporin A therapy. Oral Surg Oral Med Oral Pathol 1983;55:274-8. Ashrafi SH, Slaski K, Thu K, Neiman ES, Cunningham MP. Scanning electron microscopy of cyclosporine-induced gingival overgrowth. Scanning Microsc 1996;10:219-25. Bencini PL, Montagnino G, Sala F, De Vecchi A, Crosti C, Tarantino A. Cutaneous lesions in 67 cyclosporin-treated renal transplant recipients. Dermatologica 1986;172:24-30. Reznik VM, Durham BL, Jones KL, Mendoza SA. Changes in facial appearance during cyclosporin treatment. Lancet 1987;1:1405-7. Busque S, Demers P, St-Louis G, Boily J-G, Tousignant J, Lemieux F, et al. Conversion from Neoral (cyclosporine) to tacrolimus of kidney transplant recipients for gingival hyperplasia or hypertrichosis. Transplant Proc 1998;30:1247-8. Hancock RH, Swan RH. Nifedipine-induced gingival overgrowth. J Clin Periodontol 1992;19:12-4.
16. Frauman AG. An overview of the adverse reactions to adrenal corticosteroids. Adverse Drug React Toxicol Rev 1996;15:203-6. 17. Jiang H, Yamamoto S, Kato R. Induction of anagen in telogen mouse skin by topical application of FK506, a potent immunosuppressant. J Invest Dermatol 1995;104:523-5. 18. Yamamoto S, Jiang H, Kato R. Stimulation of hair growth by topical application of FK506, a potent immunosuppressive agent. J Invest Dermatol 1994;102:160-4. 19. Takahashi T, Kamimura A. Cyclosporin A promotes hair epithelial cell proliferation and modulates protein kinase C expression and translocation in hair epithelial cells. J Invest Dermatol 2001; 117:605-11. 20. Fisher GJ, Duell EA, Nickoloff BJ, Annesley TM, Kowalke JK, Ellis CN, et al. Levels of cyclosporin in epidermis of treated psoriasis patients differentially inhibit growth of keratinocytes cultured in serum free versus serum containing media. J Invest Dermatol 1988;91:142-6.
Infectious eccrine hidradenitis caused by Nocardia Diana D. Antonovich, MD, Adrienne Berke, MD, Jane M. Grant-Kels, MD, and Maxwell Fung, MD Farmington, Connecticut Neutrophilic eccrine hidradenitis is a nonspecific clinical reaction pattern classified as a neutrophilic dermatosis that typically occurs in the setting of chemotherapy for hematologic malignant disease. Neutrophilic eccrine hidradenitis more rarely has been reported in association with infectious agents, including Serratia, Enterobacter, Staphylococcus, and HIV. We describe the first case of infectious eccrine hidradenitis occurring in a patient with cutaneous Nocardia infection. (J Am Acad Dermatol 2004;50:315-8.)
N
eutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis that most commonly occurs after administration of chemotherapy. The disorder was first reported, by Harris et al,1 with use of cytarabine to treat a patient with acute myelogenous leukemia. NEH has since been associated with diverse clinical conditions, occurring during use of both chemotherapeutic and nonchemotherapeutic agents,2-4 in immunocompromised and healthy persons,5-8 and infrequently in infection. Infectious NEH is rare, only 4 published reports documenting the presence of an infectious pathoFrom the Department of Dermatology, University of Connecticut Health Center. Funding sources: None. Conflict of interest: None identified. Reprint requests: Adrienne Berke, MD, University of Connecticut Health Center, 263 Farmington Ave, Farmington, CT 06030. Email: [email protected]. 0190-9622/$30.00 Copyright © 2004 by the American Academy of Dermatology, Inc. doi:10.1016/S0190-9622(03)02163-7
gen at the cutaneous eruption site.5,9-11 Five reports of NEH describing the ailment in HIV-infected patients have been published in the English literature.3,12-14 We describe the clinical and histopathologic findings of the first reported case of infectious eccrine hidradenitis (IEH) associated with Nocardia infection.
CASE REPORT An 83-year-old woman had a 2-day history of diffuse pain, tenderness, edema, and erythema of the right leg. The clinical impression was cellulitis. The patient was admitted to the hospital, and treatment with cefazolin was initiated. The medical history was significant for chronic obstructive pulmonary disease, mitral valve prolapse, and breast and endometrial cancer. She had undergone right lumpectomy with axillary node dissection 5 years earlier and total abdominal hysterectomy 15 years earlier. The patient’s current medications included tamoxifen, prednisone 12.5 mg orally once a day, and pulmonary inhalers as needed. With the excep-
316 Case reports
Fig 1. Neutrophils around and infiltrating the ductal epithelium. Luminal abscesses and swollen epithelial cells are evident. (Hematoxylin-eosin stain.)
tion of a serum white blood cell count of 22.9/L (normal, 4.5-11/L), results of hematological studies were within normal range. A chest radiograph was without infiltrates or evidence of acute disease. Although some improvement of the cellulitis was noted, on hospital day 5, multiple, tender, erythematous plaques and nodules involving the right thigh were observed. These lesions were speculated to be secondary to septic or cholesterol emboli from a cardiac source. An echocardiogram revealed mitral valve endocarditis with large vegetation. Under aseptic conditions, 2 biopsy specimens of the cutaneous lesions were obtained. One tissue specimen was cultured and the other was submitted for routine histologic examination. Findings at microscopic examination were consistent with NEH: a sparse superficial and deep perivascular and interstitial, polymorphous infiltrate of neutrophils, lymphocytes, and histiocytes. Neutrophils were present around and infiltrating the dermal eccrine ducts with extension in the ductal lumina (Fig 1). Acid-fast, Gram, and Gomori methenamine silver staining showed filamentous organisms within eccrine epithelial cells (Fig 2). Subsequent serial blood cultures were positive for Nocardia asteroides. Tissue culture confirmed the cutaneous presence of N asteroides. Treatment with trimethoprim-sulfamethoxazole resulted in complete resolution of the skin lesions within 3 weeks. The patient experienced no known recurrence.
DISCUSSION IEH was first described in 1985 by Moreno et al9 in a patient with chronic renal failure for which he received hemodialysis. That patient experienced recurrent eruptions of erythematous papules over a period of 3 years, until cutaneous biopsy showed features typical of NEH, and a tissue culture revealed the presence of Serratia organisms.
J AM ACAD DERMATOL FEBRUARY 2004
Fig 2. Left panel, Gomori methenamine stain shows positive staining of filamentous organisms in ductal epithelium (arrows). Right panel, Higher magnification of the organisms (arrows).
The authors speculated that this patient was immunocompromised owing to chronic renal failure, a condition that predisposed him to IEH from a nosocomial pathogen. IEH has subsequently been reported in a healthy, immunocompetent patient with Enterobacter cloacae as the offending microbe.5 The organism in that instance was identified by culture of an intact pustule. Skin biopsy confirmed IEH. The patient initially presented with a 2-month history of cutaneous lesions, which promptly resolved after 1 week of appropriate antibiotic therapy. A third report of IEH, associated with Staphylococcus aureusinfection, occurred in a man receiving immunosuppressive therapy after heart transplantation.10 Tissue cultures revealed S aureus infection, and the histologic findings were consistent with NEH. Again, after a prolonged history of recurrent eruptions, the lesions resolved within 5 days of initiation of antibiotic therapy. A second report of Serratiarelated IEH has been documented.11 Confirmation was made by tissue culture, histologic examination, and electron microscopic identification of bacteria within the lesions. This case of IEH occurred in a young man with spinal ependymoma, for which he received no chemotherapy. Although not clearly within the category of IEH, HIV infection has been associated with development of NEH. In 1990 Smith et al3 described 2 cases of NEH occurring in HIV-positive men who were taking zidovudine. The authors suggested the eruptions might have been the result of therapy with this agent, which has pharmacologic similarities to cytarabine. This line of thinking also was advanced regarding a case of NEH in an HIV-infected patient who had been receiving stavudine, a new reverse transcriptase inhibitor.12 However, in 2 additional cases of NEH, the HIVinfected patients were not receiving antiviral ther-
J AM ACAD DERMATOL VOLUME 50, NUMBER 2
apy at the time of eruption.13,14 In 4 of the 5 total cases reported in the English literature, special microbial stains were negative for organisms.3,12,13 It is not clear whether tissue cultures were performed in any of the cases. In each previously reported case of bacterial IEH, the offending organism was first appreciated with lesional culture. Our case was unique in that it represented the first case of IEH in which the infectious agent was detected histologically within the eccrine ductal epithelium and subsequently identified with tissue culture. It also represents the fifth reported case of IEH and the first report of IEH occurring with N asteroides infection. This organism is an aerobic, gram-positive, partially acid-fast, branching actinomycete and is a common inhabitant of soil.15 The organism is primarily an opportunistic pathogen, most frequently infecting immunocompromised persons.15 Inoculation of N asteroides occurs by inhalation of bacterial mycelia or by transcutaneous inoculation.16 The most common sites of infection include lung, brain, and skin, in decreasing order of frequency.16 First-line therapy for nocardiosis is treatment with trimethoprim-sulfamethoxazole for 3 months to lifelong use, depending on the overall health status of the patient.16,17 In our patient a chest radiograph showed no evidence of pulmonary infection. Furthermore, there was no known history of occupational exposure or traumatic transcutaneous inoculation. The source of cutaneous infection is unclear. It can be presumed that development of mitral valve vegetation preceded IEH, perhaps serving as the nidus of infection, although it is conceivable that the reverse is true. Why the infection occurred in association with NEH is unknown. Daily prednisone use is likely to have resulted in some degree of immunocompromise. The patient’s breast and endometrial carcinomas were both in remission, and she had not received chemotherapy. The skin eruptions resolved with appropriate antimicrobial therapy for nocardiosis. This outcome supports the hypothesis that an infectious agent had been the source of NEH. Although possible, it is less likely that an opportunistic infection developed and was superimposed on precedent, noninfectious NEH. Despite the increase in the number of cases since the condition was first described by Harris et al, the pathophysiologic mechanism of NEH remains obscure. Some authors have speculated that the cutaneous eruption is caused by a pharmacologic agent exerting a toxic effect on the eccrine gland or a reaction to an underlying malignan-
Case reports 317
cy.3,18,19 However, in light of the diversity of the clinical manifestations, these explanations are oversimplifications, not accounting for the occurrence of NEH in healthy persons or its association with infectious agents. Our case supports the contention that NEH is a characteristic cutaneous response to nonspecific stimuli and should be included in the differential diagnosis of a spectrum of clinical presentations. As has been emphasized by previous authors,12 when a case of NEH is being considered, the possibility of an infectious association must be appreciated and investigated by cutaneous tissue culture, because infectious and noninfectious NEH are generally indistinguishable histologically.10 Although noninfectious NEH is typically self-limited, necessitating no treatment, IEH must be treated with antibiotic therapy, a consideration that emphasizes the importance of differentiating the two conditions during diagnosis.
REFERENCES 1. Harrist TJ, Fine JD, Berman RS, Murphy GF, Mihm MC Jr. Neutrophilic eccrine hidradenitis: a distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy. Arch Dermatol 1982;118:263-6. 2. Kuttner BJ, Kurban RS. Neutrophilic eccrine hidradenitis in the absence of an underlying malignancy. Cutis 1988;41:403-5. 3. Smith KJ, Skelton HG 3rd, James WD, Holland TT, Lupton GP, Angritt P. Neutrophilic eccrine hidradenitis in HIV-infected patients. J Am Acad Dermatol 1990;23:945-7. 4. Bachmeyer C, Chaibi P, Aractingi S. NEH induced by granulocyte colony- stimulating factor. Br J Dermatol 1998;139:354-5. 5. Allegue F, Rocamora A, Martin-Gonzalez M, Alonso ML, Ledo A. Infectious eccrine hidradenitis. J Am Acad Dermatol 1990;22: 1119-20. 6. Manganoni AM, Facchetti F, Gavazzoni R, Marocolo D, De Panfilis G. Neutrophil eccrine hidradenitis in a healthy woman. Dermatology 1994;189:211-2. 7. Simon M, Cremer H, von den Driesch P. Idiopathic recurrent palmoplantar hidradenitis in children. Arch Dermatol 1998;134:76-9. 8. Buezo GF, Requena L, Fraga Fernandez J, Garcia Diez A, Fernandez Herrera JM. Idiopathic palmoplantar hidradenitis. Am J Dermatopathol 1996;18:413-6. 9. Moreno A, Barnadas MA, Ravella A, Moragas JM. Infectious eccrine hidradenitis in a patient undergoing hemodialysis. Arch Dermatol 1985;121:1106-7. 10. Taira JW, Gerber HB. Eccrine hidradenitis. Int J Dermatol 1992; 31:433-4. 11. Combemale P, Faisant M, Azoulay-Petit C, Dupin M, Kanitakis J. Neutrophilic eccrine hidradenitis secondary to infection with Serratia marcescens. Br J Dermatol 2000;142:784-88. 12. Krischer J, Rutschmann O, Roten SV, Harms M, Saurat JH, Pechere M. Neutrophilic eccrine hidradenitis in a patient with AIDS. J Dermatol 1998;25:199-200. 13. Sevila A, Morell A, Banuls J, Silvestre JF, Betlloch I. Neutrophilic eccrine hidradenitis in an HIV-infected patient. Int J Dermatol 1996;35:651-2.
318 Case reports
14. Bachmeyer C, Reygagne P, Aractingi S. Recurrent neutrophilic eccrine hidradenitis in an HIV-1-infected patient. Dermatology 2000;200:328-30. 15. Chazen G. Nocardia. Infect Control 1987;8:260-3. 16. Filice GA. Nocardiosis. In: Braunwald EW, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison’s principles of internal medicine. 15th ed. New York: McGraw-Hill; 2001. p. 1006-8.
J AM ACAD DERMATOL FEBRUARY 2004
17. Gilbert DN, Moellering RC, Sande MA. The Sanford guide to antimicrobial therapy. 30th ed. Hyde Park (VT): Antimicrobial Therapy, 2000. p. 76. 18. Hurt MA, Halvorson RD, Petr FC Jr, et al. Eccrine squamous syringometaplasia. Arch Dermatol 1990;126:73-7. 19. Vion B, Alvero H. Neutrophilic eccrine hidradenitis. Dermatologica 1991;183:70-2.
Viral-associated trichodysplasia in patients who are immunocompromised COL Leonard C. Sperling, MC, USA,a Maria-Magdalena Tomaszewski, MD,b and Douglas A. Thomas, MDc Bethesda, Maryland; Washington, District of Columbia; and Las Vegas, Nevada Viral-associated trichodysplasia of immunosuppression is a newly described clinicopathologic entity found in patients who are undergoing drug-induced immunosuppression to prevent organ transplant rejection. Patients have numerous erythematous papules concentrated in the central portion of the face and variable degrees of hair loss, most severely affecting facial hair. Histologic findings of facial papules are highly distinctive and unique, and suggest that the entire machinery of the follicular bulb is devoted to the manufacture of inner root sheath-type keratin. Electron microscopy reveals intranuclear viral particles, but precise viral identification has not yet been achieved. (J Am Acad Dermatol 2004;50:318-22.)
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n 1999, Haycox et al1 described an individual who was immunocompromised with alopecia, facial papules, and unique histologic findings. This distinctive condition was called trichodysplasia spinulosa. Electron microscopy performed on a biopsy specimen revealed viral particles (38-nm diameter) suggestive of a papovavirus infection. However, an attempt to detect human papillomavirus subtypes using polymerase chain reaction was unsuccessful, even though 18 different viral subtypes were searched for using dot blot hybridization and consensus primer polymerase chain reaction. A test for BK virus, a polyoma virus, was also negative. BK virus was a likely suspect because of its size (ap-
From the Department of Dermatology, Uniformed Services University, Bethesdaa; the Department of Dermatopathology, Armed Forces Institute of Pathology, Washington, DCb; and Las Vegas Skin and Cancer Clinics, Ltd.c Supported by the Department of Dermatopathology, Armed Forces Institute of Pathology; and Department of Dermatology, Uniformed Services University. Conflicts of interest: None identified. Presented orally at the Las Vegas Dermatology Seminar, Las Vegas, Nev, November 16, 2002. Reprints not available from authors. doi:10.1016/S0190-9622(03)01490-7
proximately 45 nm) and its prevalence in patients who are immunocompromised and undergo organ transplant.2,3 We wish to confirm that trichodysplasia spinulosa is a distinctive and highly diagnosable condition by presenting an additional case. Our patient was a 13-year-old girl who had undergone kidney transplantation 9 months before skin disease developed. When her dermatosis began, she was taking prednisone, tacrolimus, and mycophenolate mofetil. During the course of several months a progressively worsening skin eruption developed consisting of erythematous, 1- to 3-mm papules concentrated in the midfacial area, especially the nose, malar region, glabella, and chin (Fig 1). Papules were so numerous and confluent that they distorted the contours of the nose. Some papules had a central, whitish, keratotic, spiny excrescence. There was almost total hair loss affecting the eyebrows and eyelashes, but the scalp was not affected. The lesions were mildly pruritic. Topical corticosteroids were tried without benefit, and a biopsy of a facial lesion was performed. The histologic findings were dramatic and unique, and effected most anagen hairs in the specimen. The affected follicles were very large, al-