Pustulose aseptique des plis et érythroblastopénie auto-immune

Abstract

Amicrobial pustulosis of the skin folds represents a new entity within the spectrum of neutrophilic dermatoses. This disease is characterized by acute onset of pustular lesions in the skin folds, association with an autoimmune disorder, and improvement under systemic corticosteroids. A 24-year-old woman had been presenting pustular dermatosis for several months involving the inguinal folds and the scalp. The pustules coalesced to form oozing and crusting plaques. Eczematous lesions were located on the trunk. She also presented macrocytic anemia related to autoimmune erythroblastopenia. Bacteriological culture was negative. Antinuclear antibodies were found with anti-SSA specificity. Histopathological examination of a skin biopsy specimen showed pustules in the epidermis together with an inflammatory dermal infiltrate. Cutaneous direct immunofluorescence testing was negative. The patient responded to systemic corticosteroids. Thirty-six cases of amicrobial pustulosis of the skin folds have been reported in the literature. All but two previously reported patients were females with an autoimmune disorder (chiefly systemic lupus erythematosus). The clinical picture is characterized by aseptic pustular lesions of the major and minor skin folds of the scalp and the anogenital area associated with eczematous lesions. Diagnostic criteria have recently been proposed. This disease responds to systemic corticosteroids. We report a new case of amicrobial pustulosis of the skin folds associated with autoimmune erythroblastopenia, which to the best of our knowledge has been described only once in the literature.