PYODERMA-GANGRENOSUM, SUBCORNEAL PUSTULAR DERMATOSIS AND NEUTROPHILICPULMONARY INVOLVEMENTS IN A PATIENT WITH MONOCLONAL IGA GAMMOPATHY

Introduction. The term << neutrophilic dermatosis >> is normaly used t o cover a number of dermatoses characterized by their response to cort icotherapy or to drugs effective on the neutrophilic polynuclear. Neut rophilic dermatosis was frequently associated with hematologic disease . We study the case of a neutrophilic dermatosis accompanied by a pulm onary involvement and then review the literature on the reported cases of neutrophilic dermatosis with extra-cutaneous localisations. Case s tudy. A woman aged 54 displayed a pyoderma gangrenosum successively fo llowed by a pustular dermatosis (related to subcorneal pustular dermat osis of Sneddon-Wilkinson) and by a pulmonary involvement not caused b y any underlying infection. The laboratory results revealed a benign I gA gammaglobulin. The use of corticotherapy and a relay by dapsone led to the gradual disappearance of the cutaneous and pulmonary lesions. Discussion. Pulmonary involvement have been observed in the Sweet synd rome and in the pyoderma gangrenosum. The neutrophilic lung is diagnos ed once a tumorous or infectious cause has been eliminated and on the basis of the response to the cortisone treatment. Conclusion. The link between pulmonary involvement and neutrophilic dermatosis is sometime s mentionned but is in fact often difficult to confirm. The positive r eaction to corticosteroids could be a diagnostic test.