BI30: A bullous dilemma

M. Shetty, B. Gaglani and I. Logan

Ealing Hospital, London, UK

We aimed to highlight the association of chronic lymphocytic leukaemia (CLL) with erythema multiforme (EM) major. We present the case of a 52-year-old fit man with a 5-day history of a painful unilaterally grouped vesiculobullous eruption over multiple dermatomes T1–T4, rapidly evolving to cross the midline and forming widespread bullous targetoid eruptions and erosions sparing the palms and soles with some oral mucosal involvement, 3 days after the intake of naproxen. He had a background of hypertension with no associated B symptoms. On examination, he had cervical and inguinal lymphadenopathy. Initial suspicion was that of varicella zoster virus (VZV) infection with bullous drug eruption. This was treated with oral prednisolone, topical steroids and intravenous aciclovir. Naproxen was stopped. Investigations revealed lymphocytosis (40 × 10⁹ cells L^–1) with smear cells. HIV screening was negative. Viral swab polymerase chain reaction (PCR) was positive for VZV DNA, which was confirmed on serology. Herpes simplex virus (HSV) IgM and IgG were positive but PCR was negative. Of note, Epstein–Barr virus viral capsid antigen IgG and IgM were positive. He underwent computed tomography of the chest, abdomen and pelvis, which showed widespread lymphadenopathy and splenomegaly. Histological findings were likely consistent with a bullous drug reaction. The differential at this stage widened to include paraneoplastic, but immunofluorescence was negative. A new diagnosis of Binet stage B small lymphocytic lymphoma/CLL was made by the haematology team and confirmed with a lymph node biopsy. Based on the clinical picture and investigations, a final diagnosis of multidermatomal VZV infection and EM major was made. This was further substantiated by the absence of further eruptions many weeks after the patient restarted naproxen without clinical supervision. Our patient responded very well to treatment with no recurrence to date and is under follow-up with the haematology team. EM is known to be commonly associated with HSV infection. Our case represents an uncommon association of EM major with CLL and its simultaneous manifestation with multidermatomal VZV infection. Shankari et al. (Shankari SK, Jayanthi K, Gupta B. Erythema multiforme in a patient with recurrent non-Hodgkin’s lymphoma/chronic lymphocytic leukemia. J Indian Acad Oral Med Radiol 2012; 24: 168) described a case of EM associated with non-Hodgkin lymphoma and CLL-1. We highlight the importance of considering an underlying haematological malignancy in a patient with an atypical manifestation of overlapping skin conditions with EM. The two blistering skin manifestations occurring simultaneously can inadvertently be mistaken for a drug reaction; therefore, it is imperative to correlate this clinically with a detailed history and appropriate investigations. Further studies are needed to substantiate this association and the pathophysiology behind this.