Zeldzame neutrofiele dermatose met belangrijke hematologische implicaties

Abstract

Rare neutrophilic dermatosis with significant hematologic implications A patient was seen in the dermatology department because of a solitary lesion on the right forearm. This lesion had an annular aspect with a raised, bright erythematous, inflammatory border of confluent papules with an oedematous aspect. Previously, similar lesions had developed, regressing spontaneously after a few weeks. Histological examination showed a marked oedema and a dens dermal predominant neutrophilic infiltrate with leucocytoclasia without vasculitis. Based on the clinical picture, the chronic recurrent course and the histology, the diagnosis of chronic recurrent annular neutrophilic dermatosis (CRAND) was made. Since CRAND belongs to the neutrophilic skin disorders, a comprehensive blood test was performed, showing a monoclonal B cell population. In consultation with a fellow haematologist, additional CT scans of the thorax and abdomen were performed. These showed neither adenopathies nor hepatosplenomegaly, upon which the diagnosis of monoclonal B cell lymphocytosis was made. This condition increases the risk of developing chronic lymphatic leukaemia. This case report thus shows a rare neutrophilic dermatosis that differs from Sweet syndrome given the absence of fever, systemic symptoms or neutrophilia in the blood. This case is specifically relevant because of the additional finding of a monoclonal B cell lymphocytosis, increasing the risk of developing chronic lymphatic leukaemia. This demonstrates the importance of further additional investigation to exclude possible (pre)malignancies when the diagnosis of CRAND is made.