Clinicopathologic features of ulcerative-atrophic sarcoidosis.
BACKGROUND
Sarcoidosis is a chronic granulomatous disease of unknown etiology. Cutaneous disease is common and includes two clinicopathologic categories: granulomatous infiltration or a reactive phenomenon. In the granulomatous infiltrative group, clinical manifestations can be variable. Ulcers in sarcoidosis are uncommonly recognized and have been categorized previously under the rubric of atrophic, necrobiosis-like, or ulcerative sarcoidosis.


METHODS
We evaluated retrospectively sarcoidosis patients presenting to the Johns Hopkins Department of Dermatology between June 1989 and May 2002. Multiple skin biopsies were performed for histopathologic evaluation. Investigation for extracutaneous manifestations, including routine serologic assays, chest radiography, pulmonary function tests, electrocardiogram, and angiotensin-converting enzyme level, and referral for ophthalmologic examination were performed in all patients.


RESULTS
Of 147 consecutive patients presenting with cutaneous sarcoidosis, seven demonstrated ulcerative-atrophic sarcoidosis lesions. All patients were African-American (five females and two males). All patients had ulcers surrounded by atrophic necrobiosis lipoidica-like plaques on the pretibial areas. All patients had other mucocutaneous manifestations of sarcoidosis, with the majority having evidence of internal disease. Combined immunosuppressive and immunomodulatory therapy was effective in controlling the cutaneous manifestations of all patients with ulcerative sarcoidosis.


CONCLUSIONS
The ulcerative variant is a poorly defined subset of cutaneous sarcoidosis. Trauma, superimposed on atrophic plaques, appears to be the principal mechanism of this rare variant of cutaneous sarcoidosis.
Yoo
Simon S
SS
Department of Dermatology, Division of Immunodermatology, Johns Hopkins University, Baltimore, Maryland, USA.
Mimouni
Daniel
D
Nikolskaia
Olga V
OV
Kouba
David J
DJ
Sauder
Daniel N
DN
Nousari
Carlos H
CH
D000328:Adult; D001284:Atrophy / Q000473:pathology; D001707:Biopsy, Needle; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D035002:Lower Extremity; D008297:Male; D008875:Middle Aged; D017441:Necrobiotic Disorders / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012507:Sarcoidosis / Q000188:drug therapy / Q000473:pathology*; D012883:Skin Ulcer / Q000188:drug therapy / Q000473:pathology*; D016896:Treatment Outcome
D007166:Immunosuppressive Agents