Grover's disease associated with Waldenström's macroglobulinemia and neutrophilic dermatosis.

A 52-year-old man who had had WaldenstroÈm's disease for 5 years presented to our department with clinical manifestations of hyperviscosity syndrome including headaches and paresthesias of the extremities. All treatments for the haemopathy had been withdrawn 6 months earlier because of drug resistance. Besides splenomegaly, physical examination showed 2 types of cutaneous lesions: nonfollicular and papular lesions, non-pruriginous, some slightly crusted, localized at the upper part of the trunk that developed during previous weeks (Fig. 1) and purplish isolated papules on the lower limbs surrounded by an erythematous halo (Fig. 2), which had appeared recently together with fever. Several skin biopsy specimens were taken. Histological examination of the skin biopsy specimens from the lesions on the back showed focal acantholysis associated with ``corps ronds'' compatible with a diagnosis of transient acantholytic dermatosis (TAD). Direct immuno ̄uorescence was negative. Biopsy specimens of skin lesions of the leg showed a dense dermal and epidermal neutrophilic in®ltrate without acantholysis or vasculitis suggestive of a neutrophilic dermatosis. Bacterial cultures performed on both lesions were sterile. Blood investigations showed pancytopenia (WBC: 1500/mm, haemoglobin level: 8.1 g/dl, platelet count: 29000/mm), elevated sedimentation rate (150/h), high level of total serum proteins (107 g/l) and of gamma globulins (60 g/l). IgA and IgG levels were normal. Circulating anti-intercellular substance antibodies were positive (titre: 1/640) as anti-smooth muscle antibodies (titre: 1/1280, identi®ed as anti-actine antibodies). A bone marrow biopsy showed a high-grade polymorphous lymphoplasmocytic lymphoma. The patient died a few weeks later despite a treatment including chemotherapy (cyclophosphamide, adriamycin, prednisone) and plasmapheresis. During that period, the cutaneous lesions remained unchanged.