Yasuki Hata

Eosinophilic pustular folliculitis (Ofuji's disease): indomethacin as a first choice of treatment.

Eosinophilic pustular folliculitis (EPF) is characterized by erythematous patches of large follicular papules and pustules involving mainly the face. Although various treatments have been attempted for EPF, including systemic and topical steroid, diaphenylsulphone, colchicine, minocycline as well as UVB phototherapy, there is no consensus on the first choice of treatment. We report a typical case and summarize 25 patients with EPF treated in our hospital between 1978 and 1998. Indomethacin was most frequently used (12/25) and showed clinical improvement in the majority of the cases (11/12). The effect of indomethacin was usually observed within 1–2 weeks after initiation of treatment. Decrease of peripheral blood eosinophils accompanied the clinical improvement. Thus, indomethacin should be considered as a first choice of treatment for EPF.

Production of the entire extracellular domain of BP180 (type XVII collagen) by baculovirus expression

Bullous pemphigoid (BP) is an acquired autoimmune skin disease, and its target antigens are a 230 kDa plaque protein (BP230) and a 180 kDa transmembrane protein with interrupted collagenous domains (BP180, type XVII collagen), which localize at the hemidesmosome. In this study we have attempted to express the entire extracellular domain of BP180 (rBP180EC) as a secreted protein by baculovirus expression. Seventy out of 83 BP sera (84.4%) showed positive reactivity against rBP180EC by immunoblot analysis, and 56 out of 83 BP sera (67.5%) were positive against rBP180EC by ELISA. These figures were comparable with those when a bacterial recombinant protein encoding the NC16a domain of BP180 (rNC16a) was used as an antigen source. Reactivity of BP sera against rBP180EC by ELISA was completely abolished or significantly reduced by immunocompetition with rNC16a in 11 out of 14 BP sera tested, while the reactivity was not altered in the rest of the three sera. These findings indicate that the NC16a domain represents the major epitopes on the extracellular domain of BP180, although there are some other minor epitopes outside of NC16a which are uniquely expressed by rBP180EC. rBP180EC will be useful to develop a diagnostic tool for BP as well as to dissect a molecular role for BP180 in interactions of keratinocytes with epidermal basement membrane.

A Case of pemphigus foliaceus which occurred after five years of remission from pemphigus vulgaris.

A 77-year-old Japanese female developed pemphigus foliaceus (PF) after 5 years of remission from pemphigus vulgaris (PV). The patient had painful erosions in her mouth and flaccid blisters of the skin and was diagnosed as having PV, which responded well to corticosteroid treatment. She was then free from any lesion of PV for 5 years with a low dose of corticosteroid. Then she developed scaly erythematous lesions on the skin and was diagnosed as suffering from PF. Enzyme-linked immunosorbent assay (ELISA) using recombinant desmoglein 1 (Dsg-1) and Dsg-3 revealed that she had anti-Dsg-3 IgG in the PV stage, no antibodies during remission and anti-Dsg-1 IgG in the PF stage. These findings indicate that the target antigen was shifted from Dsg-3 to Dsg-1 along with the phenotype after a 5-year interval in this patient.

Trichophyton Tonsurans Infection Manifesting as Multiple Concentric Annular Erythemas

We report a case of dermatophytosis in a Judo wrestler caused by Trichophyton tonsurans (T. tonsurans) with clinical features that mimicked the concentric rings of tinea imbricata. Tinea imbricata is a unique dermatophytosis caused by Trichophyton concentricum (T. concentricum), observed endemically in subtropical to torrid zones and characterized by impressive concentric rings. We found three similar cases of the dermatophytosis in the literature that were reported as tinea pseudoimbricata or tinea indecisiva. All of these cases were associated with systemic or local immunosuppression, perhaps simulating the mechanism of tinea imbricata, which is known to involve the lack of delayed type hypersensitivity to T. concentricum. These cases imply that iatrogenic immunosuppression may perhaps play an important role in the development of the unique clinical features mimicking tinea imbricata. Furthermore, three of the four cases, including the presented case, were caused by T. tonsurans. It may be necessary to consider T. tonsurans infection when multiple concentric erythemas are encountered.

Cathepsin D expression in skin metastasis of breast cancer

Cathepsin D, an aspartic proteinase, correlates with invasion and metastasis in breast cancer and with poor prognosis. In the present study, we examined the immunohistological expression of cathepsin D in both primary (5 cases) and skin‐metastatic breast cancers (13 cases) and compared it to those in gastric (2 cases) and lung (4 cases), and primary eccrine cancers (3 cases). All breast and gastric cancers were adenocarcinomas. The 2 gastric cancers were poorly differentiated, while the 4 lung cancers consisted of 2 poorly differentiated adenocarcinomas, 1 poorly differentiated large cell carcinoma, and 1 moderately to poorly differentiated squamous cell carcinoma. We also surveyed the immunohistological distribution of cathepsin B, carcinoembryonic antigen, gross cystic disease fluid protein‐15, c‐erbB‐2, and estrogen receptor. In almost all breast cancer samples, the cancer cells demonstrated strong expression of cathepsin D in the cytoplasm, but weak staining patterns with other antibodies. Gastric and lung cancer cells did not respond with cathepsin D (except one metastatic lung cancer) or the other immunohistological markers. Since cathepsin D is strongly expressed in primary and metastatic lesions of breast cancer, cathepsin D could be useful as an adjunct to a panel of immunohistochemical stains in determining the primary site of origin of metastatic cancer in the skin.

Keratoacanthoma with glandular proliferation.

A case of keratoacanthoma (KA) with glandular proliferation was reported. The tumor was a firm, dome‐shaped, elevated nodule on the cheek of an 82‐year‐old Japanese male. Generally, the tumor showed the typical histopathological features of KA; slight nuclear atypia and mitotic figures were present in a cup‐shaped proliferation of keratinocytes and, in the center of the lesion, a keratin‐filled crater with nests of dyskeratotic and acantholytic cells was seen. In the bottom of the lesion, a glandular structure forming branching, thin lumina was observed. The epithelium of the lumina was made up of two or more layers of columnar or cuboidal cells without keratinization. Tall columnar cells with oval nuclei were located in the luminal row and small cuboidal cells with round nuclei and scanty cytoplasm were in the outer layer. Immunohistochemical staining revealed that only the glandular structure was carcinoembryonic antigen positive. Lectin binding patterns observed in the glandular proliferation were similar to those reported for the apocrine gland. KA sometimes exhibits an adenoid structure in its lesion because of acantholytic changes. However, KA associated with true glandular proliferation has not been reported as far as we know, and our case is the first reported one. KA is thought to be a tumor of follicular origin, and the glandular proliferation observed in the present case seemed to have certain characteristics that suggest its apocrine origin.

Case of primary cutaneous adenoid cystic carcinoma: Expression of c-KIT and activation of its downstream signaling molecules.

between low-risk and high-risk BCC. Given that IGF-1R regulates the biologic process involved in malignancy, it is possible that IGF-1R involvement explains the aggressive behavior of high-risk BCC subtypes. In addition, we suggest that IGF-1R may be a useful marker in the differentiation of high-risk BCC from low-risk BCC. We previously found nuclear IGF-1R in poorly differentiated cutaneous squamous cell carcinoma. Nuclear IGF-1R originates from the cell surface, not from an intracellular pool of newly synthesized receptors. The fact that nuclear IGF-1R binds to enhancer regions and activates transcription raises the possibility that nuclear IGF-1R may contribute to deregulated gene expression and thereby play a pathophysiological role in cancer cells. However, we did not find IGF-1R expression in the nucleus, but mainly in the membrane of tumor cells of high-risk BCC. Aberrant activation of Sonic hedgehog (Shh) signaling is considered to be a major signal transduction pathway in the BCC tumorigenesis. IGF-1 cooperates with deregulation of the Shh pathway via Akt activation. IGF-IR associate with Smo, suggesting that Shh and IGF-1 are integrated at the receptor level. Therefore, we speculate that IGF-1R may participate in the pathogenesis of high-risk BCC, although further experiments are warranted.