Paraneoplastic Pemphigus Presenting with Prominent Lichanoid Tissue Reaction

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  • 苔癬型反応を主体とするparaneoplastic pemphigus
  • タイセンガタ ハンノウ オ シュタイ ト スル paraneoplastic pemphigus

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Abstract

We report a 62-year-old woman with paraneoplastic pemphigus who developed severe mucosal ulceration and widespread keratotic papules. Skin biopsies revealed epidermal vacuolization, keratinocyte necrosis, and a band-like mononuclear cell infiltration, showing a lichenoid reaction. IgG and C 3 deposition was observed in intercellular spaces of the epidermis and along the basement membrane zone on direct immunofluorescence. An indirect immunofluorescence study showed intracellular IgG staining in the normal human epidermis, and IgG deposition in the basement membrane zone and intercellular spaces of rat bladder epithelium. By immunoblotting, polypeptides with molecular weights of 250-, 190- and 130-kD, were recognized in the patient’s serum. Antibodies against desmoglein 1 and 3 were also found in the serum by ELISA. B cell lymphoma was diagnosed by a lymph node biopsy. Acute respiratory failure developed and caused the patient’s death. In our patient, lichenoid tissue reactions were prominent and no bulla formation was seen. This allows us to hypothesize that both cell-mediated and humoral immune reactions are important in the pathogenesis of paraneoplastic pemphigus. We consider that the diagnosis of the disease should be made by multiple immunological examinations including indirect immunofluorescence using rat bladder, immunoblotting and ELISA.

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