Bullous Wells' Syndrome Associated with Non-Hodgkin's Lymphocytic Lymphoma

Abstract

3/µl; eosinophils 14.3% neutrophils 48%, lymphocytes 31.2%, monocytes 6.5%, basophils 0.2%), total immunoglobulin E (IgE) = 751 IU/ml, C-reactive protein (CRP) 1.25 mg/dl, erythrocyte sedimentation rate (ESR) in the first hour 60 mm; viral markers (Epstein Barr virus, cytomegalovirus, hepatitis A, B and C virus), cryoglobulin, ANCA, LAC, ANA, ENA and anti-DNA antibodies were all negative. Histopathological examination of the lesion on the left leg showed an epidermis characterized by multiple, sometimes confluent vesicles containing serum and eosinophil granulocytes. The underlying papillary dermis was markedly oedematous, with focal and minimal erythrocytic extravasations and an interstitial eosinophil granulocytic infiltrate. The reticular dermis was infiltrated by a large number of prevalently perivascular lymphocytic elements and numerous perivascular and interstitial eosinophil granulocytes, which also extended along the interlobular hypodermal septa and, to a lesser extent, the hypodermic lobules. The reticular dermis also showed some small and isolated flame figures (Fig. 2). The diagnosis of Wells’ syndrome was made on the basis of the clinical picture and the histological findings, together with a negative direct immunofluorescence test (5). Having excluded pharmacological, infective, vasculitic and inflammatory causes, the subsequent instrumental and laboratory investigations were aimed at identifying a possible relapse of the patient’s previous neoplastic disease. Complete abdominal ultrasonography, chest radiography and colonoscopy were negative, as was a search for tumour markers. The physical examination findings of numerous swollen inguinal and axillary lymph nodes therefore drew our attention to a possible underlying lymphoproliferative disease, and a subsequent lymph node biopsy revealed a picture compatible with a diffuse, small-cell non-Hodgkin’s B lymphoma/ B-cell CLL, which was confirmed by a bone marrow biopsy.