Microsoft Word - DRM220BF

E. Telegdy, I. Schneider, Department of Dermatology, University Medical School, Pécs (Hungary) Sir, The association of cutaneous T cell malignant lymphoma (CTCL) and a vesiculobullous disease is extremely rare. Kaposi [1] in 1887 presented the first report of a pemphigus-like dermal lesion associated with mycosis fungoides. Since then a few associations of CTCL and other autoimmune bullous diseases have been published but according to the literature the association of CTCL with bullous pemphigoid has not been reported previously. We have diagnosed the joint occurrence of these two diseases in a 61-year-old woman. Case History. Pyelonephritis and chronic uremia have been known for 5 years. In March 1987, there was an attack of herpes zoster on the right side of the trunk. The maculous dermal symptoms accompanying the pruritus have persisted essentially unchanged since then. in spite of oral antihistamine and local steroid treatment. On admission the skin was dry and scaly. Pigmented brownish plaques, partly livid, irregular in size and shape, and slightly elevated, were seen more on the trunk. back and abdomen and less on the extremities. Their surface was scaly or eczematous and in some places the plaques were infiltrated. The patient was not taking drugs regularly. Other physical examinations revealed no pathological changes. Laboratory studies showed moderately elevated BSR (20 mm/h). renal function tests (CN: 20.4 μmol/l, serum creatinine: 389 μmol/l) and 16% eosinophilia in the peripheral blood smear. All the routine laboratory findings were within normal range. In the urine Escherichia coli and Proteus vulgaris infections were detected which were treated with erytromycinum (2.000 mg daily), on the basis of the antibiogram. Because of the basic disease (CTCL) ‚ a treatment with 90 mg/day of oral prednisolone and a single dose of 25 mg methotrexate (MTX) was initiated. On the 6th day following the MTX therapy, the patient began to complain of a burning sensation in the lips and of a mild erythema all over the body. Lentil-sized, tense vesicles appeared on the left flexor forearm, abdomen, waist and on the edge of the tongue. The Nikolsky sign was negative. Since the possibility of a drug side effect, but also of an autoimmune vesiculobullous disease arose as concerns the symptoms, a histology of the bullous lesion was performed too. The histologic picture of the plaque was interpreted as mycosis fungoides stage I–II. The histologic sections of the bullous process were diagnosed as bullous pemphigoid. Direct immunofluorescence shows an intense linear band of IgG along the basement membrane zone (fig. 1). IIFtest was negative. Therapy: in response to an orally administered tapered dose of steroid, complete recovery was observed in the bullous dermal process within 1 month.