Cutaneous manifestations of neutrophilic disease. A study of seven cases

Abstract

Seven patients with a complex form of neutrophilic dermatosis are reported. Clinically, they had variable associations of four types of lesions: blisters/pustules, plaques, nodules and ulcerations. Histologically, a neutrophilic infiltrate was observed at variable levels in the epidermis, dermis and subcutis. Systemic manifestations were present in all cases (general symptoms, joint, renal, ocular and lung involvements). Three patients had an associated disease (myelodysplasia, metastatic carcinoma, IgG gammopathy). Steroids were the most efficient treatment. These observations, as well as a review of the literature, support the opinion that the neutrophilic dermatosis represents a continuous spectrum encompassing four well-defined entities: subcorneal pustular dermatosis, Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We propose that the different patterns of the neutrophilic dermatosis are the most obvious manifestations of a potentially multisystemic neutrophilic disease and allow its recognition.