Fatal bullous pyoderma gangrenosum in a patient with Klinefelter's syndrome.

Sir, Pyoderma gangrenosum (PG) is an idiopathic neutrophilic dermatosis may occur in association with a wide variety of disorders, including inflammatory bowel disease, haematological and rheumatological conditions, and states of perturbed immune function. In addition to the classical ulcerative form of PG, less common variants exist. Among these, bullous PG was first described in 1972 as a painful superficial blistering and eroded dermatosis in patients with leukaemia (1). Because of their clinical similarity, some authors believe that bullous PG and atypical Sweet’s syndrome represent points on a continuum of diseases that have in common dermal neutrophilia (2). We describe here a male patient with Klinefelter’s syndrome who developed bullous PG resistant to various immunosuppressive regimens and died following the occurrence of septicaemia.