Mitsuru Iwata

Epidermolysis bullosa acquisita: clinical response to plasma exchange therapy and circulating anti-basement membrane zone antibody titer

Epidermolysis bullosa acquisita has been recognized as a rare autoimmune mechanobullous disorder since the detection of immunoglobulin and complement deposits along the basement membrane zone. A circulating anti-basement membrane zone antibody has also been detected in some cases. We are reporting a case of epidermolysis bullosa acquisita in which clinical symptoms were well correlated with the circulating anti-basement membrane zone antibody titers. Although the patient initially responded very well to corticosteroid therapy, remission could not be maintained without increasing the dosage. Other therapies, including azathioprine, dapsone, vitamin E, and gold sodium thiomalate, produced no beneficial effects. Although a high dose of oral corticosteroid and cyclophosphamide decreased the antibody titer and blister formation, this therapy had to be terminated because of side effects. Plasma exchange therapy in combination with corticosteroid and low-dose cyclophosphamide resulted in a marked decrease of the anti-basement membrane zone antibody titer and clinical improvement. Thus plasma exchange therapy may be a useful adjunct to conventional treatments for patients with epidermolysis bullosa acquisita.

Anatomical distribution and immunological characteristics of epidermolysis bullosa acquisita antigen and bullous pemphigoid antigen

A Japanese patient with epidermolysis bullosa acquisita (EBA) was autopsied, and direct immunofluorescence (DIF) testing was performed. Using this patients serum (EBA serum) and three bullous pemphigoid (BP) sera, the anatomical distribution and immunological character‐istics of EBA antigen and BP antigen were investigated by indirect immunofluorescence (IIF). EBA antigen showed the same anatomical distribution as BP antigen in DIF and IIF studies; both antigens were limited to the skin, tongue, oesophagus, trachea, cornea and bladder. EBA antigen was located on the dermal side of both NaCI and PBS‐separated skin, whereas BP antigen was limited to the epidermal side. Ethanol fixation abrogated the antigenic stability of BP antigen, but not that of HBA antigen. No difference was found when acetone or formalin fixation was used. The separation methods and prefixation in ethanol could be useful techniques applicable to the classification of the bullous disorders which manifest circulating anti‐BMZ antibodies.

“Ectopic” extramammary Paget's disease affecting the lower anterior aspect of the chest

A case of extramammary Pagets disease that affected the lower anterior aspect of the chest of a 54-year-old man is reported. It is extremely rare that the disease arises in areas other than the anogenital region and the axillae.

Immunohistochemical studies of major histocompatibility antigens in a case of the bare lymphocyte syndrome without immunodeficiency

A case of the bare lymphocyte syndrome without apparent immunodeficiency was observed. The patient had, in addition, ichthyosis vulgaris and sinobronchial disease. A pustular lesion developed at first on the anterior aspect of lower part of the left leg. This lesion gradually increased in size and evolved into giant indurated, irregular adjoining plaques. On biopsy, the histologic findings were similar to necrobiosis lipoidica. No human lymphocyte antigen (HLA) class I antigens were detected on peripheral mononuclear cells; however, HLA-DR antigens were present on B lymphocytes. Immunohistochemical studies disclosed defective expression of class I antigens in the non-lesional skin, but positive expression was demonstrated in the lesional area. HLA-DR antigens were expressed on keratinocytes and on most infiltrating inflammatory cells in the affected skin. It is therefore speculated that class I antigen appearance and mononuclear cell infiltrate each induces the other and that together they play an important role in the formation and enlargement of the skin lesion.

Pemphigus vegetans presenting as scrotal tongue.

A case of scrotal tongue is described. Histological and immunofluorescent findings revealed that this case was pemphigus vegetans, which should therefore be added to the differential diagnosis of scrotal tongue.

Expression of Transferrin Receptor in Normal Human Skin, Psoriatic Skin and Various Skin Tumors

The distribution of transferrin receptor in normal human skin and its expression in psoriatic skin and various skin tumors have been investigated. Immuno‐peroxidase staining was performed on biopsy specimens using monoclonal OKT 9 antibody, which reacts with transferrin receptor.

A case of malignant trichilemmoma produced in solar keratosis by immunohistochemistry dyeing.

A 75-year-old woman had a nodule on her cheek. Histopathological examination revealed lobular proliferaton of clear cells in the center and basaloid in the peripheral area of the tumor. The clear cells were PAS positive, diastase sensitive. The basaloid cells showed palisading. Tumor cells were CEA (-) S-100 (-) . Nuclear atypio was seen. We diagnosed it as a malignant trichilemmoma.Immunohistochemical stining using various monoclonal anticytokeratin antibodies with formalin-fixed and paraffin-embedded block specimans was performed. All the tumor cells were positive to simple epithelial cytokeratin such as CK8, 18. Clear cells were positive to differentiated stratified epithelial cytokeratin such as CK1, and simple epithelial cytokeratin CK19. Staining pattern was different from epidemis, but similar to outer root sheath between is thmus and infundidulum.