Reactive perforating collagenosis in a setting of double disaster: Acquired immunodeficiency syndrome and end-stage renal disease

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8. 9. 10. 11. 12. 13. 14.

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Herpes simplex mimicking leukemia cutis

ous signs of internal malignancies. Geriatrics 1980; 35(12):65-72. Stawiski MA. Skin manifestations of leukemias and lymphomas. Cuffs 1978;21:814-8. Buffer DF, Berger TG, Rodman OG. Leukemia curls mimicking stasis dermatitis. Cuffs 1985;35:47-8. Su WPD, Buechner SA, Li C-Y. Clinicop~tthologie correlations in leukemia cutis. J AM ACAD DERMATOL 1984;11:121-8. Bluefarb SM. Leukemia eutis. Springfield, IlL: Charles C Thomas, 1960:55-78. Vonderheid EC, Milstein HJ, Thompson KID, Wu BC. Chronic herpes simplex infection in cutaneous T-cell lymphomas. Arch Dermatol 1980;116:1018-22. Goldgeier MH, Cohen SR, Braverman IM, Stenn KS. An unusual and fatal case of disseminated cutaneous herpes simplex. J AM Acho DERMATOL1981;4:176-80. Lam MT, Pazin GJ, Armstrong JA, Ho M. Herpes simplex infection in acute myelogenous leukemia and other hematologic malignancies. Cancer 1981;48:216871. Bean SF, Fusaro RM. Atypical cutaneous herpes simplex infection associated with acute myelogenous leukemia. Acta Derm Venereol (Stockh) 1969;49:94-6.

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16. Leming PD, Martin SE, Zwelling LA. Atypical herpes simplex (HSV) infection in a patient with Hodgkin's disease. Cancer 1984;54:3043-7. 17. Buechner SA, Li C-Y, Su WPD. Leukemia cutis: a histopathologie study of 42 cases. Am J Dermatopathol 1985;7:109-19. 18. Braverman IM. Skin signs of systemic disease. 2nd ed. Philadelphia: WB Saunders, 1981:180-I. 19. Cleland JB. Leukaemic infiltrations. Br Med J 1935; 2:1191-4. 20. Anhalt AW, Forsey RR. Herpes zoster, leukaemia curls and leukaemic infiltration of the lesions of herpes zoster. Can Med Assoc J 1956;75:750-1. 21. Barton RL, O'Leary PA. Herpes zoster generalisatus associated with chronic lymphatic leukemia. Arch Dermatol Syph 1945;51:263-5. 22. Caffin CH. Leukaemic infiltration of the site of herpes zoster. Br Med J 1946;1:801. 23. Audouin J, Tourneau AL, Aubert JP, Diebold J. Herpes simplex virus lymphadenitis mimicking tumoral relapse in a patient with Hodgkin's disease in remission, Virchows Arch 1985;408:313-21.

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Reactive perforating collagenosis in a setting of double disaster: Acquired immunodeficiency syndrome and end-stage renal disease David E. Bank, MD, Philip R. Cohen, MD, and Steven R. Kohn, MD New York, New York We report reactive perforatingcollagenosisin an intravenousheroin abuser with acquired immunodeficiency syndrome and end-stage renal disease. The literature on perforating disorders in patients with acquired immunodeficiency syndrome and the pathogenesis of reactive perforating coUagenosis in this setting is reviewed. (J AM ACAO DERMATOL 1989;21:371-4.)

The recent plethora of publications on cutaneous manifestations of acquired immunodeficiency syndrome (AIDS) has included not only viral, fungal, bacterial, protozoan, and arthropod pathogens,TM but also hypersensitivity syndromes, benign hyperproliferative conditions, benign vascular conditions, malignant neoplasms, and miscellaneous disor-

ders: ,s We report a case of reactive perforating collagenosis developing in a patient with AIDS and end-stage renal disease. To our knowledge, reactive perforating collagenosis has never been described in association with AIDS or in association with end-stage renal disease as a result of either heroin nephropathy7 or human immunodeficiency virus (HIV) nephropathy:

From the Department of Dermatology, College of Physicians and Surgeons of Columbia University. Reprint requests: David E. Bank, MD, Departmentof Dermatology, College of Physicians and Surgeons of Columbia University, 630 W. 168th St., New York, NY 10032.

CASE R E P O R T A 38-year-old black man with a history of intravenous drug abuse and a diagnosis of AIDS came to Columbia-Presbyterian Medical Center in April 1986

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Fig. 1. Hyperkeratotic lesions, with central umbilication, of reactive perforating collagenosis on posterior aspect of chest. Fig. 2. Central plug of basophilic debris and collagen is noted. Epidermis is absent at base of lesion. (Hematoxylin-eosin stain; • Fig. 3. Dermal collagen perforating through epidermis. (Hematoxylin-eosin stain;

x325.) with complaints of dyspnea, fatigue, anorexia, and abdominal pain. Laboratory tests revealed a blood urea nitrogen concentration of 100 mg/dl and a serum creatinine level of 8 mg/dl. An ultrasound study showed symmetric, echogenic kidneys, 9 cm in length, without obstruction. He had proteinuria, 4 to 5 gm/day, and a urinalysis showed no erythrocytes, leukocytes, or casts. A test for antinuclear antibodies was negative. The nephrology service considered the patient's renal disorder to be compatible with either heroin nephropathy or HIV nephropathy; a distinction between these diagnoses could not be determined on the basis of the patient's history and laboratory test findings. A renal biopsy was not performed. Graft thrombosis and infection complicated hemodialysis, and the patient began peritoneal dialysis in April 1987. Five months later, in addition to generalized pruritus, the patient noted a pruritic erup-

tion. Examination revealed hyperkeratotic, umbilicated papules, 1 to 3 mm in diameter, scattered on the posterior aspect of the chest, arms, legs, and dorsum of each foot (Fig. 1). Examination of a biopsy specimen showed perpendicular collagen bundles perforating through the epidermis (Figs. 2 and 3). The clinical features suggested, and histologic findings confirmed, a diagnosis of reactive perforating collagenosis. DISCUSSION Recognition of the rapidly expanding panorama of dermatologic presentations of H I V infection is essential to the diagnosis and treatment of patients with AIDS.I6 We report the first case of reactive perforating collagenosis in a patient with AIDS. Reactive perforating collagenosis is a disease of

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unknown cause in which dermal collagen is extruded through the epidermisY Reactive perforating collagenosis occurs in two forms: inherited and acquired. I~In the inherited form, first described by Mehregan et al. in 1967, 9patients have lesions that appear early in life on exposed surfaces, often after superficial trauma. ~~After the pinhead-sized papules enlarge and develop central umbilication, they spontaneously resolve and leave a temporarily hypopigmented or hyperpigrnented patch. Reports of familial reactive perforating collagenosis support a genetic predisposition to development. T M In contrast, the acquired form of reactive perforating collagenosis manifests in adult life, is not familial, usually is accompanied by general pruritus, and has been associated with lymphoma, diabetes reeltitus, and end-stage renal disease.~5-~7 The pathogenesis of reactive perforating collagenosis is unknown. Mehregan et al.9 and Nyters and Mehregan ~~originally postulated that minor superficial trauma in genetically susceptible persons led to'~necrobiosis of the collagen in the dermal papillae, which was subsequently removed from the body by means of "transepithelial elimination." Additional clinical investigations by Cochran et al. ~6further supported this theory. In contrast, on the basis of clinical and histologic observations in two patients with the inherited form of reactive perforating collagenosis, Millard et al. ts stated that trauma was an insufficient etiologic factor for the lesions and showed that the extruded collagen was normal. Thus the role of trauma in the pathogenesis of reactive perforating collagenosis remains unclear. Recently Mayoral and Penneys3 described a male intravenous drug user with AIDS and disseminated histoplasmosis who showed transepidermal elimination of fungal cells with a granulomatous dermatitis. His lesions resolved with amphotericin B therapy. Interestingly, as demonstrated by our patient and the patient with AIDS and disseminated histoplasmosis, 3 a normal immune system is not necessary for transepidermal elimination to occur. Thus the alterations in the immune system caused by HIV infection do not impair the skin's ability to respond in this fashion. Our patient initially had the typical clinical and histologic findings of the acquired form of reactive perforating eollagenosis. Whether his reactive perforating collagenosis was causally related to either his HIV infection and/or his renal disease remains

Reactive perforating collagenosis 373 unclear. Wherever the defect in reactive perforating collagenosis lies, there appears to be a common pathway that causes skin to react in this fashion. Pruritus alone is insufficient to induce reactive perforating collagenosis; therefore there must be an additional--currently undefined---etiologic factor. In summary, we describe a patient with AIDS and end-stage renal disease who subsequently developed reactive perforating collagenosis. Thus, in addition to adding reactive perforating collagenosis to the growing list of cutaneous diseases associated with AIDS, we also add AIDS to the list of diseases showing perforation.

REFERENCES 1. Kaplan MH, Sadick N, McNutt NS, Meltzer M, Sarngadharan MG, Pahwa S. Dermatologic findings and manifestations of acquired immunodeficiency syndrome (AIDS). J AM AcAr> D~RMA'roL 1987;16:485-506. 2. Fisher BK, Warner LC. Cutaneous manifestations of the acquired immunodeficiency syndrome: ulxiate 1987. Int J Dermatol 1987;26:615-30. 3. Mayoral F, Penneys NS. Disseminated histoplasmosis presenting as a transepidermal elimination disorder in an AIDS victim. J AM ACAD DEKMATOL1985;15:842-4. 4. Cohen PR, Beltrani VP, Grossman ME. Disseminated herpes zoster in patients with human immunodeficiency virus infection. Am J Med 1988;84:1076-80. 5. Sindrup JH, Lisby G, Weismann K, Wantzin GL. Skin manifestations in AIDS, HIV infections, and AIDSrelated complex. Int J Dermatol 1987;26:267-72. 6. Cohen PR, Kurzrock R. Tongue lesions in the acquired immunodeficiency syndrome. Cutis 1987;40:406-9. 7. Cunningham EE, Zielezny MA, Venuto RC. Heroinassociated nephropathy: a nationwide problem. JAMA 1983;250:2935-6. 8. Rao TKS, Friedman EA, Nicastri AD. The types of renal disease in the acquired immunodeficiency syndrome. N Engl J Med 1987;316:1062-8. 9. Mehregan AH, Schwartz DD, Livingood CS, Reactive perforating collagenosis. Arch Dermatol 1967;96:27782. 10. Nyters A, Mehregan AH. Reactive perforating collagenosis (Mehregan): report of two new eases and a review of the literature. Aeta Derm Venereol (Stockh) 1973; 53:375-80. 11. Nair BK, Sarojini PA, Basheer AM, Nair CH. Reactive perforating collagenosis. Br J Dermatol 1974;91:399403. 12. Kanan MW. Familial reactive perforating collagenosis and intolerance to cold. Br J Dermatol 1974;91:405-14. 13. Berlin C, Goldberg LH. Reactive perforating collagenosis and phototraumatism. Dermatotogica 1985;171:255-8. 14. Rotta D. Reactive perforating eollagenosis: report of 3 eases. Dermatologica 1983;166:308-10. 15. Poliak SC, Lebwohl MG, Pallis A, Prioleau PG. Reactive perforating collagenosis associated with diabetes mellitus. N Engl J Med 1982;306:81-4. 16. Cochran R J, Tucker SB, Wilkin JK. Reactive perforating

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Bank et al. collagenos[s of diabetes mellitus and renal failure. Cutis 1983;31:55-8. 17. Henry JC, Jorizzo JL, Apisarnthanarax P. Reactive perforating collagenosis in the setting of prurigo nodularis. Int J Dermatol 1983;22:381-7.

18. Millard PR, Young E, Harrison DE, Wajnarowska F. Reactive perforating collagenosis: light, ultrastructural and immunohistological studies. Histopathology 1986; 10:1047-56.

Rowell's syndrome Report o f a case A. Parodi, MD, E. F. Drago, MD, G. Varaldo, MD, and A. Rebora, M D

Genoa, Italy We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti--Sj-T antibodies found in the original Rowell's syndrome cases has been recently claimed. A review of the literature suggests that most of the cases of Rowell's syndrome described thus far in fact may be cases of coincidental association of lupus erythematosus and erythema multiforme. (J AM ACAD DEm~ATOL 1989;21: 374-7.) In 1963 Rowell et al. ~ described a syndrome characterized b y lupus erythematosus with erythema multiforme-like lesions, a positive test for rheumatoid factor, speckled antinuclear antibodies, and a serum antibody directed to an extract of human tissues. Since its original description, 27 cases of the syndrome have been reported, including that described by Scholtz 2 in 1922 and those by others) 6 A review of these reports, however, reveals that none of the cases reported after that described by Rowell et al. conforms to their diagnostic criteria of the syndrome. W e describe a patient who initially had annular lesions and whose laboratory test results fit Roweli's description and discuss the reasons that may support the genuinity of this rare clinical entity. CASE REPORT

A 62-year-old man had a history of figurate lesions of the face and back that 10 years previously had been diagnosed histologically as subacute cutaneous lupus erythematosus and had been treated successfully with

From the Department of Dermatology,Universityof Genoa. Reprint requests: A. Parodi, MD, Department of Dermatology, Universityof Genoa, V[ale BenedettoXV, Genoa, Italy. 374

Fig. 1. Transient annular lesions of thigh. chloroquine. Two months before he came to our department of dermatology, he had noticed figurate lesions on his legs, particularly on the left thigh, that rapidly and completely disappeared (Fig. 1). The appearance of a new annular lesion on his right thigh prompted him to consult us. The patient denied receiving any drug for many years. Physical examination revealed five apparently differ-