Paraneoplastic pemphigus associated with follicular lymphoma

Abstract

A 52-year-old woman was referred for a retroperitoneal mass, the biopsy of which revealed follicular lymphoma. Before admission, she had developed a polymorphic bullous eruption and painful oral ulceration. Examination showed several oral ulcers (especially on the lateral surfaces of the tongue and on the cheeks), gingivitis and erosions of lips with adherent whiteyellow exudate (top left). There were numerous erosions with erythematous bases on the trunk, from 0AE5 to 3 cm in diameter, with crusts, multiple flaccid or tense blisters and lichenoid lesions suggestive of erythema multiforme (top right). Conjunctivitis with yellowish secretions was present (bottom left). Skin biopsy showed suprabasal and subepidermal clefts with scattered necrotic keratinocytes (bottom right). Eosinophilic infiltration of dermis and epidermis and epidermal acantholysis were observed. Direct immunofluorescence (IF) studies showed deposition of IgG and C3 on the surface of keratinocytes and in the basement-membrane zone. Indirect IF studies showed binding of serum antibodies to rat bladder. The patient’s serum was positive for anti-desmogleins 1 and 3 antibodies by enzyme-linked immunosorbent asssay. The diagnosis of paraneoplastic pemphigus was established. A spectacular improvement of skin lesions was observed following the first cycle of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone). Oral ulcerations were much more resistant to treatment despite obtaining a very good haematological partial response after four cycles and maintenance of steroids. After eight cycles, oral improvement was finally observed. As paraneoplastic pemphigus requires specific and prolonged therapy, the diagnosis of this life-threatening affection should be considered by haematologists when faced with polymorphous eruption and oral ulcerations.