Hypocalcemia occurs in patients with psoriasis vulgaris, generalized pustular psoriasis of von Zumbusch, and impetigo herpetiformis. In most cases, hypocalcemia is caused by accompanying hypoalbuminemia. We report the rare case of a 24-year-old woman with idiopathic hypoparathyroidism in whom hypocalcemia precipitated typical generalized pustular psoriasis of von Zumbusch. The pustular lesions over her entire body rapidly cleared when her serum calcium was corrected by therapy with oral calcium and an active form of vitamin D₃. Thereafter, the lesions of psoriasis vulgaris reappeared on her chest and arms. From these findings mentioned above, this case was diagnosed as generalized pustular psoriasis, but not impetigo herpetiformis. There are reported cases that hypocalcemia induced a generalized pustular psoriasis of von Zumbusch in the English literature. The present report may be the first of this disease to be reported from Japan.

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A 69-year-old man had an erythematous-bullous eruption on the nape, lateral side of the neck, left hand and forearm after beeing on piroxicam for 3 days. He was not exposed to sunlight and stopped taking the piroxicam, nevertheless, the erythema spreaded rapidly on the unexposed skin. The MED values to UVA and UVB were within a normal range. A photopatch test using UVA showed a positive reaction at the site of piroxicam application. After the ingestion of piroxicam for 3 consecutive days, as an oral provocation test, edematous erythema developed in the same site as at the admission, and exposure of UVA irradiation to his back, an area with no erythema, led to erythema lesions. A lymphocyte transformation test for piroxicam was positive. These results suggest that a photoallergic reaction may play a role in the piroxicam-induced photosensitivity, except for the time course.

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Two patients with acral lentiginous melanoma with prominent lymphatic invasion were treated. One was considered to be in clinical stage Ib because of the absence of inguinal lymph node metastasis. However, a small subcutaneous nodule in connection with a black stripe, later found to be a metastasis of melanoma, and lymphatics filled with tumor cells were detected at the lower edge of the femoral triangle. Similar lesions were also found in another individual. Histopathological examination of those lesions were in-transit metastasis of melanoma and lymphatic invasion of melanoma. Dye injection with selective penetration into the lymphatics revealed that the main lymphatics stream from the sole ran along with the large saphenous vein. Although the prognostic significance of lymphatic invasion of melanoma remains to be determined, these findings suggested that the main stream of lymphatics be removed in surgical treatment of plantar malignant melanoma. The significance of lymphatic invasion and the surgical treatment of plantar malignant melanoma are discussed.

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A 16-year-old girl with proliferating trichilemmal cyst (PTC) was admitted to hospital with a complaint of a painless lump on the nucha. It had gradually enlarged during the past two years. The excised 5×4×4 cm tumor weighed 30 gr and was well demarcated from the surrounding tissue by a thin fibrous capsule. Microscopically, the tumor was composed of squamous epithelium proliferating in irregularly-shaped and various-sized lobules with central areas of trichilemmal keratinization and foci of calcification. Epithelial cells were small and palisaded at the periphery of the lobules, and in the inner layer they are large and clear with glycogen in the cytoplasm. Foci of inflammatory reaction with foreigh-body giant cells were present. There was no evidence of malignancy. Eighty-six reported cases of PTC collected from the Japanese literature were reviewed, together with the present case. There were 65 benign PTCs and 22 malignant PTHs. More than a half the number of patients with benign PTC were elderly women (55%), whereas the majority of the patients with malignant PTC were males (73%). The benign PTCs occurred mostly on the scalp (82%), but the malignant lesions were often present in sites other than the scalp (73%). Although benign PTCs were sharply demarcated and often encapsulated, malignant PTCs showed invasive growth, cellular atypia and frequent mitoses, occasionally metastasizing to the regional lymph nodes.

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IgG λ type myeloma associated with systemic amyloidosis occurred in a 65-year-old man. He had noticed an abnormal sensation in the fingers preceding asymptomatic papules on the perioral region and upper eyelids about one year before coming to our dermatology clinic. Amyloid materials were observed throughout the dermis, particularly around the small vessels and beneath the epidermis, using light microscopic examination. The amyloid deposits were enclosed by an attenuated processes of fibroblasts and a moderate amount of collagen fibers and elastic fibers was present in some amyloid deposits. Around some vessels, the amyloid did not precipitate beneath the basement membrane. These findings suggested that amyloid did not always precipitate beneath the basement membranes. At autopsy, massive amyloid depositions were present in all organs and tissues. Elastic fibers in the amyloid deposits were present beneath the endocardiium, but not beneath the pericardium. Thus, elastic fibers do not always precipitate amyloid materials.

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Case 1 was a 54-year-old man with a little finger tip-sized and elastic soft cystic swelling on the scaphoid fossa of the left auricle. Histopathologically the cyst was present in the auricle cartilage. The cystic wall was fibrous tissue with no epithelial tissue. Case 2 was a 49-year-old man with a cyst on the right auricle. Their lesions were similar clinically and histologically and were diagnosed as a pseudocyst of the auricle. In case 1, the fluid of the cyst was removed by puncture and a bolster mattress suture was made using roll gauze. In case 2, after evacuating the fluid of the cyst, 0.1% Decadron solution was injected several times into the cystic cavity. In both cases, satisfactory results were obtained. We considered that in the pseudocyst of the auricle, the lysosomal enzymes were implicated the degenerative process of the cartilage. Steroid solution seems to be the first choice of treatment.

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A forty-five-year-old man with organoid nevus involving the scalp had three different adnexal tumors within the lesion, for a few years. Histologically, they were confirmed as basal cell epithelioma, syringocystadenoma papilliferum, and sweat apparatus-derived benign tumor. The last tumor microscopically showed numerous dilated channels lined with a double layer of epithelial cells that form papillations projecting into the lumen. There was no evidence of decapitation and some lumina were filled with amorphous eosinophilic material. This routine finding prompted us to carry out immunohistochemical investigations with eccrine sweat gland-associated antibodies. SKH1 and anti-CEA antibodies stained only the inner layer of the dilated channels and EKH4 stained only the flattened outer layer of the cells, thereby suggesting these dilated channels are derived from the eccrine sweat gland. Our final diagnostic term of this tumor was eccrine papillary adenoma. Statistically, the 28 cases of organoid nevus that have been reported developed more than two secondary arising tumors within the lesion, and only 7 cases had more than three distinct tumors, in the Japanese literature from 1965 to 1987. Our statistical survey showed few cases of organoid nevus in association with eccrine sweat apparatus derived tumors. Our present report seems to be the first of an association of organoid nevus with eccrine papillary adenoma.

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A 60-yea-old woman had a tender nodule with an ulcer on her right palm. The histological diagnosis was eccrine duct carcinoma. She had other papules and nodules on her left wrist, abdomen, thighs and buttocks. Histologically, Bowen’s disease was diagnosed. One nodule resembled basal cell epithelioma. Because of the multiple skin cancers located mainly on the trunk and keratosis on the palms and soles, we speculated that these lesions were provoked by arsenic. The nodule on her right palms was resected and the other lesions disappeared after ODT with 5 FU ointment or after cryosurgery with liquid nitrogen. Adjuvant chemotherapy of pepleomycin and mitomycin C was prescribed. At this writing 8 months later, she is free from a recurrence.

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We treated a 61-year-old woman with pseudoxanthoma elasticum with typical cutaneous eruptions, ocular lesions and intermittent claudication. The intermittent claudication was caused by arterial stenosis as the result of a calcinosis of the right femoral artery. There was no pulsation of the right dorsal pedis artery and the flattened waves on sphygmograms of the right toe were marked. Concerning the incidence and site of cardiovascular disorders associated with this disease, the related literature states that a higher frequency occurs among Caucasians.

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A Case of idiopathic benign form of follicular mucinosis is reported. Multiple erythematous infiltrative plaques and papules were present on the scalp, face, back, abdomen and both upper arms. Light microscopic examination showed that the hair follicles and sebaceous glands were edematous and the epithelial cells appeared spindle-shaped or stellate. There were mucin filled cystic intercellular spaces of the pilosebaceous apparatus and between the collagen fibers in the upper dermis. Inflammatory cell infiltration was observed near the pilosebaseous apparatus and numerous S100 protein positive cells were evidenced. Electron microscopic examinations revealed that the affected follicular epithelial cells were rich in rER and Langerhans cells had unusual amorphous materials in the cytoplasm.

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The effect of immunomodulative drugs on ADCC was examined by a plaque assay, since ADCC was reported to be affected in some skin diseases. Monocyte-depleted effector lymphocyte (K cell) from human peripheral blood, target sheep red blood cell (SRBC) and anti-SRBC IgG were added to a poly-L-lysine treated microtestplate, and immediately after making monolayers, incubated at a 37°C for 3 hours. The number and the size of hemolytic plaque (HP) of SRBC were examined in the presence of drug and/or culture medium. In absence of the drug, the percentage of plaque forming cell (%PFC) was 6.3±0.4, and the size of HP was 53.4±1.3 μm². The treatment of effector lymphocytes with recombinant interferon-αA (rIFN-αA) resulted in the augmentation of activity of individual K cell (2×10 U/ml of rIFN-αA: HP 76.0±2.6μm², p<0.001), but not in the increase of number of K cell, at any concentration. Enhancement of both %PFC and HP was induced by unique immunopotentiators Staphylococcal phage lysate (SPL) and Vaccina virus products (NSP). (2×10 U/ml of SPL: %PFC 10.4±0.6, p<0.001. HP 65.8±2.0 μm², p<0.001; 1×10 μg/ml of NSP: %PFC 8.9±1.1, p<0.05, HP 67.2±1.8 μm², p<0.001). On the contrary, prednisolone (PS) suppressed the number of K cells, dose dependently (1×10 μg/ml of PS: %PFC 3.0±0.8, p<0.01). The average area of HP was significantly smaller (HP 43.6±1.3 μm², p<0.001). Methyl B₁₂ induced no significant changes on ADCC. Thus, the plaque assay is valuable to elucidate the effect of immunomodulative drugs on ADCC.

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We observed numerous squamoid cell nests in a biopsy specimen taken from a BCE lesion in a 52-year-old man. In the cells, abundant tonofilaments, desmosomes and keratohyalin granules were seen electron-microscopically. Immunohistochemically, the cytoplasm of the cell reacted to antihuman keratin antibody. From these findings, we diagnosed our case as one of the keratotic type of BCE. This seems to be the second such case to be reported in the world literature.

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We investigated by segregation anlysis the mode of inheritance of psoriasis vulgaris with 93 psoriatic families. Out of the 93, 7 families of normal x affected mating and 83 families of normal x normal mating were collected by single selection and 3 families of normal x affected mating were ascertained through parents (complete selection). Out of the 83, 9 have two or more affected children. The hypotheses of single locus models (single dominant and single recessive models) for psoriasis were ruled out from the results of χ² analysis. On the other hand, the multifactorial model for psoriasis was not rejected by using Edwards’ simulation model. The double recessive model, as one of the polygenic models for susceptibility to psoriasis was acceptable, determined in 9 multiplex families with psoriasis. Major genes having major effects related to psoriasis were not evidenced.

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Histopathological and immunohistological studies were done on ten patients with dermatofibroma. There were foam cells and spindle-shaped clefts in the central areas. Histopathologically, we grouped the lesions into three classes, according to the combination of foam cells and spindle-shaped clefts. In the immunohistological studies of lysozyme, all lesions were negative. In the electron microscopical study the foam cells possessed numerous granules resembling lipid droplets. There was also fusion of the granules and degeneration of the foam cells. The spindle-shaped clefts were giant nonstructural materials. The clefts were presumably the result of degeneration of the foam cells.

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Serum IgE level, specific antibodies for 13 kinds of food antigens, anti-IgE auto antibody, peripheral blood lymphocytes monoclonal antibodies of OKT4 and OKT8 and lymphocyte transformation stimulated by Concanavalin A were evaluated in children with severe atopic dermatitis. Children with severe atopic dermatitis showed high IgE levels, positive IgE antibodies for food antigens and high anti-IgE auto antibody level, comparing with findings in case of bronchial asthma. There were no significant differences between severe atopic dermatitis and bronchial asthma concerning results of lymphocytes OKT4, OKT8 positive cells and OKT4/OKT8 ratio. In children with severe atopic dermatitis, lymphocytes transformation stimulated by Concanavalin A showed lower levels than seen in those with bronchial asthma.

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We cultured human hair follicle cells from follicles of the surgically resected scalp skin. In the presence of calcium (1.96 mM), a physiological concentration, the cells piled up, keratinized and rapidly displayed a terminal differentiation, as seen in skin epidermal cells. Only when they were cultured from the start with a physiological calcium concentration medium and then with a low calcium concentration one (0.43 mM) did monolayered, polygonal and large nucleated basal cell-like cells appear, and were retained for about 20 days. To remove the fibroblasts which grow rapidly and surround hair follicle cell colonies in the course of culture, we scraped them off with a rubber policeman and the growth was suppressed to some extent.

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A palatine tonsillectomy was performed on patients with pustulosis palmaris et plantaris (PPP) plus chronic tonsillitis. From the clinical courses of these patients, the following results were obtained.
1) The tonsillectomy was effective therapy for PPP.
2) When the patients with retracted palatine tonsils with pus plugs and inflamed mucosa of the fauces underwent tonsillectomy, the prognosis was favorable.
3) The results of the tonsillar provocation test and the tonsillar annulation test (Impretol test) did correlated with the postoperative prognosis of PPP more than did those of peripheral blood cell counts and serological tests, including ASO.
4) The patients with the shorter periods between the onset of PPP and the tonsillectomy showed a tendency toward earlier postoperative improvement.

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Statistical surveys on new cases of out-patients seen in our clinic for 10 years (from October 25, 1976 to the end of 1986) revealed the following: The total new cases accounted for 16,717 (7,832 males and 8,885 females). The most frequent 10 cases consisted of 1) eczema-dermatitis, 2) fungal diseases, 3) urticaria-pruritus-prurigo, 4) nevi, 5) viral diseases, 6) physiological and chemical trauma, 7) diseases of pilosebaceous organs, 8) inflammatory dyskeratosis, 9) drug eruption and toxicodermia, and 10) non-inflammatory dyskeratosis. Other cases include psoriasis (290), alopecia areata (299), vitiligo vulgaris (164), basal cell carcinoma (55), squamous cell carcinoma (44), Bowen’s disease (30), pemphigus-pemphigoid (21), collagen diseases (127) and melanoma (22). Cases of sporotrichosis and chromomycosis were relatively rare, meanwhile cases of Recklinghausen’s disease were relatively numerous, compared to the average values of all over Japan. This shows the geographical characteristics of Yamagata Prefecture in Tohoku District.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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