Dermatitis herpetiformis in Japan: an update.

BACKGROUND: Although dermatitis herpetiformis (DH) is a relatively common disease in Caucasian populations, it is rare in Asian populations including the Japanese. We encountered a Japanese case of DH which showed granular IgA and C3 deposits in the papillary dermis and which was associated with gluten-sensitive enteropathy but no HLA-B8/DR3/DQ2. OBJECTIVE: The purpose of this study is to describe the characteristics of Japanese DH cases, since most of them have been reported in Japanese language and dermatologists outside Japan are not familiar with the characteristics of Japanese DH. METHODS: We have reviewed all 34 Japanese DH cases reported previously. RESULTS: We found several features of Japanese DH compared with Caucasian DH, such as a high frequency of the fibrillar pattern, rarity of gluten-sensitive enteropathy and an absence of the HLA-B8/DR3/DQ2 haplotype. CONCLUSION: There might be significant differences in pathophysiology between Caucasian and Japanese DH cases.

Hair-discoloration of Japanese elite swimmers.

To understand hair-discoloration in relation to swimming, we examined sixty-seven elite swimmers of the Japan National Swimming Team and fifty-four, age-matched subjects as controls. The incidence of hair discoloration (61%) in the swimmers' group was significantly higher than that in controls (0%) (p<0.0001). Interestingly, surface damage of the nail plates coexisted in the swimmers with the scalp-hair discoloration. The hairs picked from the eight swimmers and two age-matched individuals as controls were examined by electron microscope (EM) and EM X-ray microanalyzer. The swimmers' discolored, golden hair revealed complete disappearance of hair cuticle both by scanning EM (SEM) and transmission EM (TEM). The quantity of melanosomes in the cortex decreased, and their diameter was smaller than that of controls. In addition, irregularly shaped melanosomes with variable electron density and less electron-dense melanosomes with white haloes were frequently observed in the swimmers' golden hair. The X-ray elemental spectrograph by SEM revealed that the content of sulfur in all the swimmers' discoloured hair was lower than that in the normal controls and that the content of chlorine in the male swimmers' discoloured hair was higher than that in the female swimmers and the normal controls. The X-ray elemental microanalysis by TEM focused on melanosomes in the cortex of the cross section and detected elemental chlorine in all swimmers' golden hairs. It did not detect any element in the control hairs. The 14C-tyrosine uptake test of hairbulbs found no significant difference between the swimmers and the normal controls. These findings suggest that hair discoloration was mainly due to cuticle damage by friction with water. Hypochlorous acid in the swimming pool water can penetrate to the hair cortex through the cuticle. It can oxidize and degenerate melanosomes there.

Immunohistochemical diagnosis of a Merkel cell tumor in a dog.

We examined the clinical and immunohistochemical features of a Merkel (neuroendocrine) cell tumor on the skin between the eyes of a 12-year-old male Yorkshire Terrier dog. The tumor was characterized by locally expansive dermal nodules composed of solid nests or clusters of epithelioid cells surrounded by fine fibrous stroma. Basal cell epithelioma, Merkel cell tumor, and extramedullary plasmacytoma were also considered as diagnoses. Because the cytoplasm of the tumor cells stained positively for cytokeratin and chromogranin A but not for immunoglobulins, the tumor was diagnosed as a Merkel cell tumor. An electron-microscopic study of a tissue specimen revealed electron-dense granules approximately 200 nm in diameter. These granules were irregularly dispersed throughout the cytoplasm of the tumor cells, which would be expected in neuroendocrine cells. Twelve months after resection, a 0.8-cm-diameter tumor recurred at the original site. However, further follow-up of 22 months revealed no evidence of additional tumor growth, invasion, or metastasis, so we concluded that this tumor was benign.

Expression of fibrogenic cytokines in desmoplastic malignant melanoma.

Desmoplastic malignant melanoma (DMM) consists of amelanotic spindle-shaped melanoma cells and is accompanied by desmoplasia with fibrous stromata. It has a strong tendency for local infiltrative growth and recurrence and a propensity for neurotropism. It is not yet known which cytokine is responsible for the desmoplasia in DMM. In the present study, we investigated the roles of several fibrogenic cytokines and cytokine receptors in DMM: basic fibroblast growth factor (bFGF), connective tissue growth factor (CTGF), transforming growth factor-beta, platelet-derived growth factor (PDGF) and PDGF receptors. Immunostaining and in situ hybridization were conducted in four cases of DMM and four cases of amelanotic malignant melanoma (AMM) as negative controls for desmoplasia. PDGF-beta receptor, bFGF and CTGF were intensely expressed in the DMM specimens in comparison with the AMM specimens. The reaction of PDGF-B ligand and CTGF to PDGF-beta receptor, in addition to the expression of bFGF, may contribute to the desmoplasia in DMM.

Dystrophic form of inherited epidermolysis bullosa in a dog (Akita Inu).

We report a dog with dystrophic epidermolysis bullosa. This 4-year-old female Akita Inu, a species of Canis familiaris var. japonicus Temminck, had a 3-year-history of ulcers and scars over the pressure areas on the limbs, and dystrophic nails, since the age of 1 year, which corresponds to early adulthood in humans. Electron microscopy of a blister revealed separation beneath the lamina densa, and a reduction in the number of anchoring fibrils. The NC-1 domain of type VII collagen was positively stained with monoclonal antibody LH7.2 at the basement membrane zone. These findings indicate that humans and dogs have a similar response to antibody LH7.2, which may aid the development of an animal model for this disease.

Werner's syndrome associated with malignancies: five case reports with a survey of case histories in Japan.

We present 5 cases of Werner's syndrome associated with malignancies and a survey of 26 cases in the Japanese literature. Though tumors of mesenchymal origin have been reported in Werner's syndrome, 14 of the 31 cases cited in this paper developed carcinomas. Carcinoma of the thyroid gland was relatively high in frequency. The significance of carcinomas in Werner's syndrome should be further investigated.

Acquired cutis laxa (generalized elastolysis): light and electron microscopic studies.

The case is described of a 44-year-old man with acquired cutis laxa. The primary clinical manifestation was an erythema of the chest. Gradually there developed persistent circumscribed lesions in other areas of the skin, spreading into large erythematous plaques with loose skin. Slight restrictive ventilatory insufficiency, elongation of the vocal cord and diverticulosis of the colon were also found, indicating a chronic and widespread disorder. Skin biopsies showed diminution and degeneration of the elastic fibres. The light-microscopic features were subdivided in relation to the various skin manifestations. Ultrastructure of the skin showed various amounts of electron-dense, amorphous material and loosely bound microfibrils in and around elastic and collagen fibres as well as some microfibrils with knobs in the interfibrouer, were found normal. It is suggested that the first changes in the elastic fibres induced an inflammatory response and mild mucinous changes of the collagen fibres. Furthermore elastic fibres seemed to be replaced by newly formed collagen.

Dermatological study of 47,XYY males.

Dermatological features of five 47,XYY males are presented. Port-wine stains were observed in 2 cases. As the incidence of port-wine stains among the general population is believed to be 0.5%, this result would seem to indicate that the association of 47,XYY males with port-wine stains is more than a coincidence, though the survey of previous studied failed to reveal any 47,XYY cases with port-wine stains.