We report a case of epidermolysis bullosa acquisita. A 31-year-old man was referred to our clinic for blisters on the trunk and head, and erosion on the oral mucosa. Enzyme-linked immunosorbent assay (ELISA) results of anti-BP180 antibody, anti-BP230 antibody, anti-desmoglein 1 antibody, and anti-desmoglein 3 antibody were within the normal range. Histopathological examination showed subepidermal blister. Direct immunofluorescence showed deposits of IgG at the cutaneous basement membrane zone. Indirect immunofluorescence on salt-split skin showed that the patient's serum reacted with the dermal side of the split skin. Immunoblotting showed that the patient's IgG antibodies reacted with 290-kDa antigen and ELISA showed positive reactivity with type VII collagen. The patient was diagnosed with epidermolysis bullosa acquisita. We started treatment with prednisolone at a dose of 15mg/day, which did not lead to significant improvement even after increasing the dose up to 30mg/day. After adding DDS at a dose of 75mg/day, the patient's skin improved. We slowly weaned the patient off systemic corticosteroids, and new lesions decreased. We have now reduced the DDS dose to 25mg/day. Mucosal involvement at the mouth has been reported in more than half of EBA patients. It has been reported that combination therapy of systemic corticosteroids and oral DDS is effective for EBA. This case responded well to the combination therapy, and is now only on DDS.Skin Research, 14: 1-5, 2015

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Ultrasonography (US) is useful not only in the search for metastases of malignant skin tumors, but also in the evaluation of their primary lesions. US using a high-frequency probe for superficial lesions enables detailed visualization of the tumor interior. High-intensity spots that reflect calcification, necrosis, and keratinization are observed in the interior of some basal cell carcinomas, making them useful in the diagnosis of these carcinomas. Our departments aggressively perform US before surgery in order to determine the extent of excision necessary. The depths of tumors, as estimated by ultrasound, and those determined by histological assessment were very similar in six patients with basal cell carcinoma who underwent preoperative US. Preoperative US was suggested to be useful in determining the extent of excision in patients with recurrent basal cell carcinoma or those at high risk.Skin Research, 14: 6-11, 2015

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In January 2011, a 70-year-old man developed numerous blisters on the extremities when he was under treatment for a corneal ulcer at the Department of Ophthalmology in our hospital. Histopathology showed subepidermal blisters. Direct immunofluorescence revealed linear deposits of IgG and C3 at the basement membrane zone (BMZ) of the epidermis. Indirect immunofluorescence of normal human skin detected circulating IgG anti-BMZ antibodies, which reacted with the epidermal side of 1M NaCl-split normal human skin. Enzyme-linked immunosorbent assay detected IgG anti-BP180 autoantibodies. Thus, the diagnosis of bullous pemphigoid (BP) was confirmed. He also presented polygonal adherent scales on the whole body and subsequent fluorescence in situ hybridization (FISH) analysis revealed a deletion of the steroid sulfatase (STS) locus on Xp22.3, which underlies recessive X-linked ichthyosis (RXLI). The vast majority of RXLI patients are associated with various complications, such as corneal disease, chondrodysplasia punctata, mental retardation, epilepsy, and gonadal dysgenesis, by deletions of the gene cluster around the STS locus. To date, only five cases (including our own) with the concurrence of congenital ichthyosis and bullous disease have been reported in the English and Japanese literature. In particular, the present paper is the first to report a case of concurrent RXLI and BP. Because it is not clear whether the development of BP is related to RXLI, further study is necessary.Skin Research, 14: 12-16, 2015

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A 47-year-old man was referred to our hospital in July 2010. He had noticed a painful tumor on the dorsum of his left foot 5 years previously. At the time of his initial visit, he had a dome-shaped subcutaneous nodule that was approximately 20mm in diameter; his mobility was good. Histopathologically, the tumor was composed of oval-to-spindle-shaped eosinophilic cells, which showed striking multilayered, concentric growth around lesional blood vessels. Blood vessels tended to be arborized and variable in size, forming a so-called hemangiopericytomatous pattern. Immunohistochemically, smooth muscle actin was positive, but desmin was negative. Our diagnosis was myopericytoma with characteristic histopathology. Myopericytoma is a concept newly defined in the World Health Organization (WHO) Classification of Tumours in 2002. In addition, according to the 2013 WHO classification, it is related to the family of pericytic (perivascular) tumors and classified into the morphological spectrum with myofibroma, angioleiomyoma, and glomus tumors.Skin Research, 14: 17-21, 2015

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