Volume 23 Number 5, Part 1 November 1990
Discussion. Our case appears to be part ofthe spectrum of tryptophan-induced disease but is different from previous descriptions of eosinophilia-myalgia syndrome.] Many of the initially described patients had myalgia, weakness, fever, arthralgia, shortness of breath, rash, edema of the extremities, and clinical pneumonia in association with eosinophilia greater than 2000 cells/pI without other known causes of eosinophilia. Our patient had eosinophilia without any other known cause but had a variation of the previously described presentation. Her initial symptoms of fatigue, pain, and edema limited to the legs began about 5 months after starting L-tryptophan. She had never had any other acute symptoms of fever, pneumonia, myalgia, or arthralgia. During the next 6 months induration and thickening of the skin ofthe legs and arms developed, with a clinical and histologic presentation consistent with eosinophilic fasciitis. This progression is not described in the literature. We propose that a more chronic phase of tryptophan-induced disease also exists. A similar illness followed by a scleroderma-like disorder occurred in an epidemic in Spain in 1981. This disease began acutely with cough, dyspnea, pleuritic chest pain, headache, fever, and bilateral pulmonary infiltrates. 2, 3 About one third of the cases progressed to a chronic form ofthe disease associated with peripheral neuropathy, joint contractures, scleroderma-like changes, Raynaud's phenomenon, pulmonary hypertension, sicca syndrome, and liver disease. 3•4 This epidemic was linked to contamination of cooking oil, possibly from aniline or fatty acid anilides found in many samples of oiP, 5 A number of similarities exist between the acute phases of eosinophilia-myalgia syndrome and toxic oil syndrome. The role that L-tryptophan plays in inducing this constellation of symptoms is poorly understood. Possibly this new syndrome is due to a contaminant. A second possible mechanism for the action of L-tryptophan is through its metabolites, particularly serotonin and kynurenine. Scleroderma-like illnesses have been reported after L-5-hydroxytryptophan therapy and in patients with elevated serotonin levels, as in carcinoid. 6, 7 In addition, some patients with scleroderma have elevated urine kynurenine levels, which may result from intrinsic enzyme defects in kynurenine metabolism or from acquired defects such as pyridoxine deficiency. 2, 8, 9 After this article was submitted, a series ofcases including our patient was prepared, This subsequent series compares the use of tryptophan in scleroderma versus eosinophilic fasciitis (Freundlich B, Werth YP, Rook AH, et al. Ann Intern Med [in press]). REFERENCES
P, Ting M, et al. Eosinophilia-myalgia syndrome-New Mexico. MMWR 1989;38:765-6. 2. Kilbourne EM, Rigau-Perez JO, Heath CW, et al. Clinical epidemiology of toxic-oil syndrome. N Engl J Med 1983; 309:1408-14. 1. Blevins WL, Hertzman
Briefcommunications 941 3. Phelps RO, Fleischmajer R. Clinical, pathologic, and im4.
5. 6.
7. 8. 9.
munopathologic manifestations of the toxic oil syndrome. J AM ACAD DERMATOL 1988;18:313-24. Alonso-Ruiz A, Zea-Mendoza AC, Salazar-Vallinas JM, et al. Toxic oil syndrome: a syndromewith features overlapping those of various forms of scleroderma. Semin Arthritis Rheum 1986;15:200-12. Tabuenca JM. Toxic-allergic syndrome caused by ingestion of rapeseed oil denatured with aniline. Lancet 1981;2:567-8. Sternberg EM, Van Woert MH, Young SN, et a1. Development of a scleroderma-like illness during therapy with 1-5hydroxytryptophan and carbidopa. N Engl J Med 1980; 303:782-7. Fries JF, Lindgren JA, Bull JM. Scleroderma-like lesions and the carcinoid syndrome. Arch Intern Med 1973;131: 550-3. Price JM, Yess N, Brown RR, et a1. Tryptophan metabolism: a hitherto unreported abnormality occurring in a family. Arch DermatoI1967;95:462-72. Wolf H. Diseases and clinical states with abnormal tryptophan metabolism. Scand J Clin Lab Invest 1974;136:97Ill.
Pyoderma gangrenosum associated with disease: Treatment with thalidomide
Beh~et's
Malcolm H. A. Rustin, MD,a Jeremy J. H. Gilkes, MD,b and Trevor W. E. Robinson, FRCpb
London, England Pyoderma gangrenosum (PG) is a painful, destructive, ulcerating skin disease that has characteristic sharply defined, violaceous, undermined edges surrounded by. an erythematous zone. Four of the five patients originally described by Brunsting et al. l had an associated ulcerative colitis. Since then, PG has been associated with many other diseases, such as seropositive and seronegative arthritis, Crohn's disease, myeloma, leukemias, lymphomas, chronic active hepatitis, among others. 2 • 3 We describe an association of PG with Beh~t's disease and report rapid resolution of the ulceration during the administration of thalidomide. Case report. A 40-year-old white man had had recurrent, painful oral and pharyngeal ulcerations from 1980 to 1985. Repeated bacterial and viral cultures were sterile, and a biopsy specimen of one showed nonspecific changes. In June 1985 scrotal ulceration and arthralgias developed, and in August 1985 vesicopustular lesions appeared on the arms, neck, and shoulders. A skin biopsy specimen showed a leukocytoclastic vasculitis, and cutaneous pathergy was elicited. Behc;et's disease From the Department of Dermatology, The Royal Free Hospital," and University College and Middlesex School of Medicine, The Middlesex Hospital. b Reprint requests: Malcolm H. A. Rustin, MD, Department of Dennatology, The Royal Free Hospital, Pond Street, London NW3 2QG, England.
16/4/20705
942
Brief communications
Journal of the American Academy of Dermatology
Fig. 1. Well-demarcated necrotic ulcer on right shin with slate-blue margins, undermined edges, and zone of surrounding erythema.
Fig. 2. Photomicrograph of skin biopsy specimen taken through the edge of ulcer. Epidermal ulceration and hemorrhage are present. Chronic inflammatory cell infiltrate in dermis extends into subcutis. (Hematoxylin-eosin stain; Xl 00.) was diagnosed, and, because of worsening of the painful oral ulcerations, treatment with azathioprine, 100 mg/day, and prednisolone, 12.5 mg/day, was begun. In October 1987 theazathioprine was withdrawn because of abnormal results of liver function tests but the 12.5 mg/day prednisolone was continued. In October 1988 a bluish discoloration spontaneously appeared on the right shin and rapidly evolved into a painful ulcer. Within 1 week further pustular lesions appeared on the abdomen, thighs, and legs and also evolved into painful ulcers. On examination, four well-circumscribed, necrotic ulcers were on the legs. The largest, on the anterior surface of the right skin, measured 5 >< 7.5 cmand had slate-blue undermined edges
and a zone of surrounding erythema (Fig. 1). On the arms, abdomen, and shins were scars at sites of previous pustular and ulcerated lesions. A clinical diagnosis of PO was made. Investigations revealed a white blood cell count of 15.4 X 103 with 92% neutrophils. Platelet count; hemoglobin, urea, and electrolyte levels; liver function; and protein electrophoresis were normal. Bence Jones protein was not detected in the urine. Antinuclear and organ-specific antibodies were negative. Repeated cultures of ulcerated lesions were sterile, and a skin biopsy specimen of an ulcer cultured and stained for bacteria, acid-fast bacilli, and fungi was negative. A biopsy specimen taken through the edge ofan ulcer showed
Volume 23 Number 5, Part I November 1990
Brief communications
943
Fig. 3. Higher-power photomicrograph showing thrombosis and fibrinoid necrosis of blood vessels at base of ulcer but normal blood vessels toward edge of ulcer. (Hematoxylin-eosin . stain; X400.) epidermal ulceration and hemorrhage, together with a chronic inflammatory cell infiltrate in the dermis that extended into the subcutis (Fig. 2). No vasculitis or fibrinoid necrosis of the blood vessels was seen adjacent to the ulcerated area (Fig. 3). An increased dosage of prednisolone (60 mg/day) plus minocycline (200 mg/day) resulted in a rapid healing of the ulcers and diminution of the pain. However, because subsequent reduction in the dosage ofthe prednisolone to 50 mg/day caused a recurrence of the ulceration and because azathioprine had previously caused abnormal liver function, treatment with thalidomide, 400 mg/day, was begun. The PG eventually healed after 2 months, and during a 6-month follow-up the dosage of prednisolone was reduced to 3 mg/day and the thalidomide to 50 mg/day without further ulceration. The patient noticed that while receiving this treatment he had had no further oral ulcerations, although after I month he required anticoagulation for a right femoral vein thrombosis and pulmonary emboli.
diseases, and neither disease has a characteristic histologic appearance. In a recent review of 86 cases of PG, none of the patients had Beh~et's disease. lO Munro and Cox ll first described a patient with Beh~et's disease in whom PG developed. 1I We believe that our patient is the second to be described, although cases were reported from Korea and Portugal in a recent meeting on Beh~et's disease at the Mayo Clinic. Because of successful treatment of the mucocutaneous and articular manifestations of Behc;et's disease with thalidomide,12, 13 we decided to treat our patient's resistant ulceration. It is interesting, however, that when his cutaneous and mucosal lesions were in remission a venous thrombosis developed. It may be that thalidomide is not an effective treatment of this manifestation of the disease.
Discussion. Our patient's Beh~et's disease was diagnosed by the presence of cutaneous pathergy, together with two major criteria (recurrent oral and genital ulceration) and one minor criterion (peripheral arthritis). According to the 1985 symposium on Beh~et'sdisease, a firm diagnosis can be reached if a patient has only pathergy and one major or minor criterion. 4 The other major criteria are anterior or posterior eye involvement, erythema nodosum, and superficial or deep thrombophlebitis. The minor criteria are peripheral arthritis; neuropsychiatric, pleuropulmonary, or gastrointestinal manifestations; epididymo-orchitis; arterial occlusions; a history of hyperreactivity at needle prick sites; and a family history of Beh~t's disease. Behc;et's disease and PG share several common features. Pathergy is found in 40% to 88% of patients with Beh~et's disease,5 and PG can be precipitated by skin trauma. 6-9 Primary pustular lesions can occur in both
REFERENCES 1. Brunsting LA, Goeckerman WH, O'Leary PA. Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol 1930;22:655-80. 2. J-Iickman JG, Lazarus GS. Pyoderma gangrenosum: a reappraisal of associated systemic diseases. Br J Dermatol 1980;102:235-7. 3. Crow KD, Bowers RE. Bullous haemorrhagic ulceration, a variant of pyoderma gangrenosum. Trans St Johns Hasp Dermatol Soc 1974;60:142-51. 4. Dilsen N, Koni~e M, Aral O. Our diagnostic criteria of Beh~et's disease-an overview. In: Lehner T, Barnes CG, cds. Recent advances in Beh~et's disease: London: Royal Society of Medicine Services: 1986:177-80. 5. Wong RC, Ellis CN, Diaz LA. Beh~et's disease.lnt J Dermatol 1984;23:25-32. 6. Mahood JM, Sneddon IB. Pyoderma gangrenosum complicating non-Hodgkin's lymphoma. Br J Dermatol 1980; 102:223-5. 7. Toolis F, Parker AC, Barnetson R. Pyoderma gangreno-
Journal of the American Academy of Dermatology
944 Brief communications
8. 9. 10. 11. 12. 13.
sum associated with acute myeloproliferative disease (?erythroleukaemia). Postgrad Med J 1978;54:830-3. Rustin MRA, Staughton RCD, Coomes EN. Hairy cell leukemia and pyoderma gangrenosum [Letter]. J AM ACAD DERMATOL 1985;13:300-1. Perry RO, Winkelmann RK. Bullous pyoderma gangrenesum and leukemia. Arch Dermatol 1972;106:901-5. Powell FC, Schroeter AL, Su WPD, et al. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985;217:17386. Munro CS, Cox NH. Pyoderma gangrenosum associated with Beh~et's syndrome-response to thalidomide. Clin Exp Dermatol 1988;13:408-10. Jorizzo JL, Schmalsteig FC, Solomon AR, et al. Thalidomide effects in Beh~et's syndrome and pustular vasculitis. Arch Intern Med 1986;146:878-81. Hamza MR. Treatment of Behr;:et's disease with thalidomide. Clin RheumatoI1986;5:365-71.
Pseudopelade of Brocq in a child S. M. Bulengo-Ransby, MD, and J. T. Headington, MD Ann Arbor, Michigan Pseudopelade, first characterized by Brocql in 1905, is a rare and slowly progressive type of alopecia that results in permanenthair loss.1-4 Pseudopelade usually occurs in adults in the third to fifth decades of life and is more freFrom the Department of Dematology, University of Michigan. Reprint requests: Stella M. Bulengo-Ransby, MD, Department of Dermatology, University of Michigan Medical Center, 1910 Taubman Center, Ann Arbor, MI48109·0314. 16/4/22407
quent in women. 5 A review ofthe English-language literature lacked a report of pseudopelade in children. We report a case of pseudopelade of Brocq in a healthy 5-yearoldgirland emphasizethat early diagnosis and therapeutic intervention may prevent progression. Case report. A healthy 5-year-old girl was seen in 1989 with 3-month history of hair loss. There was no history of trauma or infection. Medical and family history were unremarkable. Examination revealed four discrete, oval patches ofscalp hair loss measuring 1 to 2 em (Fig. I). The lesions were smooth, had minimal erythema, and slight scale, were devoid of hair, and lacked follicular orifices. No broken hairs, pustules, or crust were seen. The pull test yielded anagen hairs. The remainder of the physical examination was normal. A scalp biopsy specimen revealed the typical findings of pseudopelade. A transverse section of a scalp lesion showed absence offollicular units from a portion ofthe specimen. The units were characterized by slight lamellar fibroplasia and absence of sebaceous epithelium. Slight angiocentrie lymphocytic inflammation was present. At the junction of dermis and subcutis, follicular stele were increased. The patient was treated with hydroxychloroquine, 200 mg daily, after a normal ophthalmologic examination. She was seen at intervals of 3 months and tolerated the medication well. After 18 months of treatment no further progression of alopecia was present and the medication was discontinued.
Discussion. The lesions of pseudopelade vary greatly in their clinical expression; the usual patient has a well-defined area of hair loss with absence of most follicular orifices. A few terminal hairs may be found in otherwise hairless areas. In patients with straight hair the remaining hair shafts in the pseudopeladic patch are sometimes paradoxically curly. Many patients, after months or years
Fig. 1. Pseudopelade of skin of scalp.
Discussion. Our case appears to be part ofthe spectrum of tryptophan-induced disease but is different from previous descriptions of eosinophilia-myalgia syndrome.] Many of the initially described patients had myalgia, weakness, fever, arthralgia, shortness of breath, rash, edema of the extremities, and clinical pneumonia in association with eosinophilia greater than 2000 cells/pI without other known causes of eosinophilia. Our patient had eosinophilia without any other known cause but had a variation of the previously described presentation. Her initial symptoms of fatigue, pain, and edema limited to the legs began about 5 months after starting L-tryptophan. She had never had any other acute symptoms of fever, pneumonia, myalgia, or arthralgia. During the next 6 months induration and thickening of the skin ofthe legs and arms developed, with a clinical and histologic presentation consistent with eosinophilic fasciitis. This progression is not described in the literature. We propose that a more chronic phase of tryptophan-induced disease also exists. A similar illness followed by a scleroderma-like disorder occurred in an epidemic in Spain in 1981. This disease began acutely with cough, dyspnea, pleuritic chest pain, headache, fever, and bilateral pulmonary infiltrates. 2, 3 About one third of the cases progressed to a chronic form ofthe disease associated with peripheral neuropathy, joint contractures, scleroderma-like changes, Raynaud's phenomenon, pulmonary hypertension, sicca syndrome, and liver disease. 3•4 This epidemic was linked to contamination of cooking oil, possibly from aniline or fatty acid anilides found in many samples of oiP, 5 A number of similarities exist between the acute phases of eosinophilia-myalgia syndrome and toxic oil syndrome. The role that L-tryptophan plays in inducing this constellation of symptoms is poorly understood. Possibly this new syndrome is due to a contaminant. A second possible mechanism for the action of L-tryptophan is through its metabolites, particularly serotonin and kynurenine. Scleroderma-like illnesses have been reported after L-5-hydroxytryptophan therapy and in patients with elevated serotonin levels, as in carcinoid. 6, 7 In addition, some patients with scleroderma have elevated urine kynurenine levels, which may result from intrinsic enzyme defects in kynurenine metabolism or from acquired defects such as pyridoxine deficiency. 2, 8, 9 After this article was submitted, a series ofcases including our patient was prepared, This subsequent series compares the use of tryptophan in scleroderma versus eosinophilic fasciitis (Freundlich B, Werth YP, Rook AH, et al. Ann Intern Med [in press]). REFERENCES
P, Ting M, et al. Eosinophilia-myalgia syndrome-New Mexico. MMWR 1989;38:765-6. 2. Kilbourne EM, Rigau-Perez JO, Heath CW, et al. Clinical epidemiology of toxic-oil syndrome. N Engl J Med 1983; 309:1408-14. 1. Blevins WL, Hertzman
Briefcommunications 941 3. Phelps RO, Fleischmajer R. Clinical, pathologic, and im4.
5. 6.
7. 8. 9.
munopathologic manifestations of the toxic oil syndrome. J AM ACAD DERMATOL 1988;18:313-24. Alonso-Ruiz A, Zea-Mendoza AC, Salazar-Vallinas JM, et al. Toxic oil syndrome: a syndromewith features overlapping those of various forms of scleroderma. Semin Arthritis Rheum 1986;15:200-12. Tabuenca JM. Toxic-allergic syndrome caused by ingestion of rapeseed oil denatured with aniline. Lancet 1981;2:567-8. Sternberg EM, Van Woert MH, Young SN, et a1. Development of a scleroderma-like illness during therapy with 1-5hydroxytryptophan and carbidopa. N Engl J Med 1980; 303:782-7. Fries JF, Lindgren JA, Bull JM. Scleroderma-like lesions and the carcinoid syndrome. Arch Intern Med 1973;131: 550-3. Price JM, Yess N, Brown RR, et a1. Tryptophan metabolism: a hitherto unreported abnormality occurring in a family. Arch DermatoI1967;95:462-72. Wolf H. Diseases and clinical states with abnormal tryptophan metabolism. Scand J Clin Lab Invest 1974;136:97Ill.
Pyoderma gangrenosum associated with disease: Treatment with thalidomide
Beh~et's
Malcolm H. A. Rustin, MD,a Jeremy J. H. Gilkes, MD,b and Trevor W. E. Robinson, FRCpb
London, England Pyoderma gangrenosum (PG) is a painful, destructive, ulcerating skin disease that has characteristic sharply defined, violaceous, undermined edges surrounded by. an erythematous zone. Four of the five patients originally described by Brunsting et al. l had an associated ulcerative colitis. Since then, PG has been associated with many other diseases, such as seropositive and seronegative arthritis, Crohn's disease, myeloma, leukemias, lymphomas, chronic active hepatitis, among others. 2 • 3 We describe an association of PG with Beh~t's disease and report rapid resolution of the ulceration during the administration of thalidomide. Case report. A 40-year-old white man had had recurrent, painful oral and pharyngeal ulcerations from 1980 to 1985. Repeated bacterial and viral cultures were sterile, and a biopsy specimen of one showed nonspecific changes. In June 1985 scrotal ulceration and arthralgias developed, and in August 1985 vesicopustular lesions appeared on the arms, neck, and shoulders. A skin biopsy specimen showed a leukocytoclastic vasculitis, and cutaneous pathergy was elicited. Behc;et's disease From the Department of Dermatology, The Royal Free Hospital," and University College and Middlesex School of Medicine, The Middlesex Hospital. b Reprint requests: Malcolm H. A. Rustin, MD, Department of Dennatology, The Royal Free Hospital, Pond Street, London NW3 2QG, England.
16/4/20705
942
Brief communications
Journal of the American Academy of Dermatology
Fig. 1. Well-demarcated necrotic ulcer on right shin with slate-blue margins, undermined edges, and zone of surrounding erythema.
Fig. 2. Photomicrograph of skin biopsy specimen taken through the edge of ulcer. Epidermal ulceration and hemorrhage are present. Chronic inflammatory cell infiltrate in dermis extends into subcutis. (Hematoxylin-eosin stain; Xl 00.) was diagnosed, and, because of worsening of the painful oral ulcerations, treatment with azathioprine, 100 mg/day, and prednisolone, 12.5 mg/day, was begun. In October 1987 theazathioprine was withdrawn because of abnormal results of liver function tests but the 12.5 mg/day prednisolone was continued. In October 1988 a bluish discoloration spontaneously appeared on the right shin and rapidly evolved into a painful ulcer. Within 1 week further pustular lesions appeared on the abdomen, thighs, and legs and also evolved into painful ulcers. On examination, four well-circumscribed, necrotic ulcers were on the legs. The largest, on the anterior surface of the right skin, measured 5 >< 7.5 cmand had slate-blue undermined edges
and a zone of surrounding erythema (Fig. 1). On the arms, abdomen, and shins were scars at sites of previous pustular and ulcerated lesions. A clinical diagnosis of PO was made. Investigations revealed a white blood cell count of 15.4 X 103 with 92% neutrophils. Platelet count; hemoglobin, urea, and electrolyte levels; liver function; and protein electrophoresis were normal. Bence Jones protein was not detected in the urine. Antinuclear and organ-specific antibodies were negative. Repeated cultures of ulcerated lesions were sterile, and a skin biopsy specimen of an ulcer cultured and stained for bacteria, acid-fast bacilli, and fungi was negative. A biopsy specimen taken through the edge ofan ulcer showed
Volume 23 Number 5, Part I November 1990
Brief communications
943
Fig. 3. Higher-power photomicrograph showing thrombosis and fibrinoid necrosis of blood vessels at base of ulcer but normal blood vessels toward edge of ulcer. (Hematoxylin-eosin . stain; X400.) epidermal ulceration and hemorrhage, together with a chronic inflammatory cell infiltrate in the dermis that extended into the subcutis (Fig. 2). No vasculitis or fibrinoid necrosis of the blood vessels was seen adjacent to the ulcerated area (Fig. 3). An increased dosage of prednisolone (60 mg/day) plus minocycline (200 mg/day) resulted in a rapid healing of the ulcers and diminution of the pain. However, because subsequent reduction in the dosage ofthe prednisolone to 50 mg/day caused a recurrence of the ulceration and because azathioprine had previously caused abnormal liver function, treatment with thalidomide, 400 mg/day, was begun. The PG eventually healed after 2 months, and during a 6-month follow-up the dosage of prednisolone was reduced to 3 mg/day and the thalidomide to 50 mg/day without further ulceration. The patient noticed that while receiving this treatment he had had no further oral ulcerations, although after I month he required anticoagulation for a right femoral vein thrombosis and pulmonary emboli.
diseases, and neither disease has a characteristic histologic appearance. In a recent review of 86 cases of PG, none of the patients had Beh~et's disease. lO Munro and Cox ll first described a patient with Beh~et's disease in whom PG developed. 1I We believe that our patient is the second to be described, although cases were reported from Korea and Portugal in a recent meeting on Beh~et's disease at the Mayo Clinic. Because of successful treatment of the mucocutaneous and articular manifestations of Behc;et's disease with thalidomide,12, 13 we decided to treat our patient's resistant ulceration. It is interesting, however, that when his cutaneous and mucosal lesions were in remission a venous thrombosis developed. It may be that thalidomide is not an effective treatment of this manifestation of the disease.
Discussion. Our patient's Beh~et's disease was diagnosed by the presence of cutaneous pathergy, together with two major criteria (recurrent oral and genital ulceration) and one minor criterion (peripheral arthritis). According to the 1985 symposium on Beh~et'sdisease, a firm diagnosis can be reached if a patient has only pathergy and one major or minor criterion. 4 The other major criteria are anterior or posterior eye involvement, erythema nodosum, and superficial or deep thrombophlebitis. The minor criteria are peripheral arthritis; neuropsychiatric, pleuropulmonary, or gastrointestinal manifestations; epididymo-orchitis; arterial occlusions; a history of hyperreactivity at needle prick sites; and a family history of Beh~t's disease. Behc;et's disease and PG share several common features. Pathergy is found in 40% to 88% of patients with Beh~et's disease,5 and PG can be precipitated by skin trauma. 6-9 Primary pustular lesions can occur in both
REFERENCES 1. Brunsting LA, Goeckerman WH, O'Leary PA. Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol 1930;22:655-80. 2. J-Iickman JG, Lazarus GS. Pyoderma gangrenosum: a reappraisal of associated systemic diseases. Br J Dermatol 1980;102:235-7. 3. Crow KD, Bowers RE. Bullous haemorrhagic ulceration, a variant of pyoderma gangrenosum. Trans St Johns Hasp Dermatol Soc 1974;60:142-51. 4. Dilsen N, Koni~e M, Aral O. Our diagnostic criteria of Beh~et's disease-an overview. In: Lehner T, Barnes CG, cds. Recent advances in Beh~et's disease: London: Royal Society of Medicine Services: 1986:177-80. 5. Wong RC, Ellis CN, Diaz LA. Beh~et's disease.lnt J Dermatol 1984;23:25-32. 6. Mahood JM, Sneddon IB. Pyoderma gangrenosum complicating non-Hodgkin's lymphoma. Br J Dermatol 1980; 102:223-5. 7. Toolis F, Parker AC, Barnetson R. Pyoderma gangreno-
Journal of the American Academy of Dermatology
944 Brief communications
8. 9. 10. 11. 12. 13.
sum associated with acute myeloproliferative disease (?erythroleukaemia). Postgrad Med J 1978;54:830-3. Rustin MRA, Staughton RCD, Coomes EN. Hairy cell leukemia and pyoderma gangrenosum [Letter]. J AM ACAD DERMATOL 1985;13:300-1. Perry RO, Winkelmann RK. Bullous pyoderma gangrenesum and leukemia. Arch Dermatol 1972;106:901-5. Powell FC, Schroeter AL, Su WPD, et al. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985;217:17386. Munro CS, Cox NH. Pyoderma gangrenosum associated with Beh~et's syndrome-response to thalidomide. Clin Exp Dermatol 1988;13:408-10. Jorizzo JL, Schmalsteig FC, Solomon AR, et al. Thalidomide effects in Beh~et's syndrome and pustular vasculitis. Arch Intern Med 1986;146:878-81. Hamza MR. Treatment of Behr;:et's disease with thalidomide. Clin RheumatoI1986;5:365-71.
Pseudopelade of Brocq in a child S. M. Bulengo-Ransby, MD, and J. T. Headington, MD Ann Arbor, Michigan Pseudopelade, first characterized by Brocql in 1905, is a rare and slowly progressive type of alopecia that results in permanenthair loss.1-4 Pseudopelade usually occurs in adults in the third to fifth decades of life and is more freFrom the Department of Dematology, University of Michigan. Reprint requests: Stella M. Bulengo-Ransby, MD, Department of Dermatology, University of Michigan Medical Center, 1910 Taubman Center, Ann Arbor, MI48109·0314. 16/4/22407
quent in women. 5 A review ofthe English-language literature lacked a report of pseudopelade in children. We report a case of pseudopelade of Brocq in a healthy 5-yearoldgirland emphasizethat early diagnosis and therapeutic intervention may prevent progression. Case report. A healthy 5-year-old girl was seen in 1989 with 3-month history of hair loss. There was no history of trauma or infection. Medical and family history were unremarkable. Examination revealed four discrete, oval patches ofscalp hair loss measuring 1 to 2 em (Fig. I). The lesions were smooth, had minimal erythema, and slight scale, were devoid of hair, and lacked follicular orifices. No broken hairs, pustules, or crust were seen. The pull test yielded anagen hairs. The remainder of the physical examination was normal. A scalp biopsy specimen revealed the typical findings of pseudopelade. A transverse section of a scalp lesion showed absence offollicular units from a portion ofthe specimen. The units were characterized by slight lamellar fibroplasia and absence of sebaceous epithelium. Slight angiocentrie lymphocytic inflammation was present. At the junction of dermis and subcutis, follicular stele were increased. The patient was treated with hydroxychloroquine, 200 mg daily, after a normal ophthalmologic examination. She was seen at intervals of 3 months and tolerated the medication well. After 18 months of treatment no further progression of alopecia was present and the medication was discontinued.
Discussion. The lesions of pseudopelade vary greatly in their clinical expression; the usual patient has a well-defined area of hair loss with absence of most follicular orifices. A few terminal hairs may be found in otherwise hairless areas. In patients with straight hair the remaining hair shafts in the pseudopeladic patch are sometimes paradoxically curly. Many patients, after months or years
Fig. 1. Pseudopelade of skin of scalp.
