Anti-early endosome antigen 1 autoantibodies were detected in a pemphigus-like patient but not in the majority of pemphigus diseases.

Although the major autoantigens in classic pemphigus are desmogleins, sera from various types of pemphigus react with a number of other molecules, including desmocollins and plakin proteins. However, other novel pemphigus-related autoantigens remain to be identified. In this study, immunoblotting for serum from an atypical autoimmune bullous disease patient identified an unknown 175 kDa protein. Subsequent studies using two-dimensional gel electrophoresis, immunoblotting and mass-spectrometry identified the 175 kDa protein as early endosome antigen 1 (EEA1). This finding was confirmed by subsequent immunological studies, including indirect immunofluorescence of skin and cultured keratinocytes, two-dimensional gel electrophoresis and immunoblotting with anti-EEA1 polyclonal antibody, and preabsorption with EEA1 recombinant protein. Finally, we developed a novel BIOCHIP assay using full-length EEA1 recombinant protein to detect anti-EEA1 antibodies. However, none of 35 sera from various types of pemphigus showed anti-EEA1 antibodies in the BIOCHIP assay, with the exception of the serum from the index case. In addition, various findings in the index case did not suggest pathogenic role of anti-EEA1 autoantibodies. Therefore, although we successfully identified the 175 kDa protein reacted by a serum of an atypical pemphigus-like patient as EEA1, novel BIOCHIP study for other pemphigus sera indicated that EEA1 is not a common and pathogenic autoantigen in pemphigus.

The First Human Case of Rickettsia tamurae Infection in Japan.

A case of Rickettsia tamurae infection in Japan is reported. A 76-year-old Japanese male had a tick bite which developed to local skin inflammation on his left leg. Anti-rickettsia antibodies were detected in his serum, and R. tamurae DNA was identified in his blood, the lesional skin, and the tick.

Erythrodermic lymphomatoid granulomatosis: a case report.

A 70-year-old man was admitted to our hospital for evaluation of a rapidly progressive erythrodermia. He had superficial lymph node swelling and gluteal/inguinal nodosum-like lesions. A skin biopsy of the erythrodermia showed dense mixed infiltrates distributed throughout the whole dermis, predominantly consisting of small lymphocytes and histiocytes with multinucleated giant cells presenting with a granulomatous appearance. The dense infiltrates showed a characteristic angiocentric pattern surrounding the upward vasculature interconnecting the subcutaneous/subpapillary plexus in the dermis. Some infiltrating lymphocytes showed mild atypia with somewhat irregularly shaped nuclei. Their immunologic staining profiles supported the diagnosis of lymphomatoid granulomatosis. Despite the dense angiocentric infiltration in the dermis, typical angiodestructive infiltration with necrotic changes was not seen on pathological examination. In this case, in situ hybridization yielded negative findings for Epstein-Barr virus-encoded RNAs. Three months after the onset of erythrodermia, the patient developed pulmonary lymphomatoid granulomatosis. Corticosteroid pulse therapy was effective for the treatment of severe pulmonary infiltrations and erythrodermia. However, there had been mild recurrence of the condition or hypereosinophilia during the 4 years of follow-up. Low maintenance doses of cyclophosphamide and corticosteroid provided the patient symptomatic relief to date.