A 38-year-old Japanese man with ATL was reported. A diagnosis of chronic ATL was made based on the following findings; positive HTLV-1 antibodies, positive proviral DNA in peripheral blood cells, lymph nodes (tissues), peripheral blood pictures with 54% small atypical cells and 0.5% flower cells, surface marker studies revealing 2% CD25 positive cells and 24% HLA-DR positive cells, lymphadenopathy and no hepatosplenomegaly. Interferon-α (IFN-α) was given, only to develop marked lung infiltration 3 days later. The treatment was then changed for VEPA, a combined chemotherapy. The patient died of a cytomegalovirus infection one year and three months after the initiation of treatment by which time a total of 680 mg of adriamycin had been given. Therefore, for a differential diagnosis between the acute and chronic types of ATL the morphology of leukemic cells, the pathological findings of skin lesions and lymph nodes, as well as marker studies of peripheral blood cells all proved useful. This case was characterized by large atypical and CD25 positive cells only in the T-zone of the lymph nodes, and IFN-α induced lung infiltration. Both the diagnosis of the type of ATL and its treatment were difficult in this case.

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A rare case of chronic adult T-cell leukemia/lymphoma (ATLL) complicated with malignant melanoma and renal cell carcinoma is reported. A 79-year-old man, born in Fukuoka prefecture, noticed a black-brownish macule on his right sole 20 years before his first visit to our clinic. After admission, continuous leukocytosis (13,000/mm³) with flower cells were found in the peripheral blood. The findings which included increased antibody titers to HTLV-1, positive HTLV-1 proviral DNA, and an appearance of ATLL cells in the cerebrospinal fluids, all support the diagnosis of chronic type ATLL. In addition, renal carcinoma (clear cell type) was histopathologically detected. The patient was treated by five sessions of cryosurgery and immunochemotherapy (dacarbazine, interferon-β), since sole’s macule and inguinal lymph node swelling were consistent with the diagnosis of malignant melanoma (level IV, stage III). These treatments were sufficiently effective to eventually case the macule on his sole to disappear. No appropriate therapies have yet been reported for either ATLL or renal cell carcinoma regarding the patient’s quality of life.

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Herpes zoster associated encephalitis is an uncommon complication of herpes zoster. Herpes zoster involving the right trigeminal nerve and the brain (herpes zoster associated encephalitis) developed in a 78-year-old Japanese male. At admission, chronic lymphatic leukemia was also discovered. The immune suppression in chronic lymphatic leukemia might have aggrevated both the patient’s clinical course and encephalitis.

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Few cases of malignant rheumatoid arthritis (MRA) have been reported in the dermatological field. A 68-year-old man was admitted to our hospital because of general fatigue, high fever, peripheral edema, purpura and blisters on both legs. There was a 5-year-history of rheumatoid arthritis and a 15-year-history of diabetes mellitus. The laboratory data were as follows: CH₅₀ 20.4 U/ml, serum immune complex 67.8 ug/ml, RAHA ×5120. The patient was thus diagnosed as having MRA according to its criteria. The purpuras and blisters developed into large and deep leg ulcers, and finally necrosis of the muscles and bones was found. Although some obstruction of the blood vessels was seen, no typical necrotizing vasculitis was found histopathologically.

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Anti-nRNP antibody, which was first reported as a serological marker of mixed connective tissue disease, has also been known to be detected in various other diseases such as Raynaud’s phenomenon, systemic sclerosis, systimic lupus erythematosus, and polymyositis. We herein report two cases of mixed connective tissue disease that were initially diagnosed as rheumatoid arthritis (RA). The first patient, a 70-year-old woman, was initially diagnosed as RA because of arthralgia and a positive rheumatoid factor. One year later, she consulted us for contact dermatitis and still had arthralgia. Antinuclear antibody tests revealed the presence of anti-nRNP antibodies. She had slightly swollen fingers and her chest X-ray showed mild pulmonary fibrosis. She was treated with prednisolone (10mg/day), resulting in an improvement in the arthralgia. The second patient, a 27-year-old woman, was also initially diagnosed as RA because of arthralgia and a positive rheumatoid factor. One year later, she presented with Raynaud’s phenomenon and swollen fingers. Antinuclear antibody tests demonstrated the presence of anti-nRNP antibody. Neither patient fulfilled the criteria for RA as proposed by the American Rheumatism Association. A total of 52 patients with anti-nRNP antibodies were examined to reveal first manifestations. Arthralgia was observed in 10/52(19.2%). In addition, rheumatoid factor was reported to often be detected in patients with anti-nRNP antibodies. Therefore, it appears that patients with anti-nRNP antibodies may initially be misdiagnosed as RA when they have arthralgia as a first manifestation and a positive rheumatoid factor.

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We herein report a case of dermatomyositis with esophageal dysfunction. A 60-year-old woman who had complained of urticarial eruptions for several years was admitted to our hospital because of marked erythema on the face and trunk associated with general fatigue and muscle weakness. Histological examination of the erythematous macules showed epidermal spongiosis, a hydropic degeneration of the basal cells and mild lymphocytic infiltration. A biopsy specimen from the deltoidal muscles also showed atrophy of muscle fibers. She complained of severe dysphagia. Esophageal manometry and a barium-study with a cine-esophagram, as well as the pH measurement in the lower esophagus showed a dysfunction of lower pharynx and both the upper and lower esophagus: diminished peristalsis and a lack of the expected relaxation of the upper esophageal sphincter muscles, vallecula and pyriform pooling, and frequent regurgitation from the stomach. The muscle weakness hardly improved and cardiac involvement appeared in spite of the treatment with 45-60 mg/day predonisolone for more than two months. Thereafter, she was successfully treated with 50 mg/day of azathioprine combined with predonisolone.

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A 50-year-old woman visited our hospital in September 1988 with erythematous eruptions on her face, extremities and back, and also demonstrated muscle weakness. A clinical diagnosis of dermatomyositis was made and predonisolone (60 mg/day) was administered. An improvement of the cutaneomuscular symptoms was observed, but a sudden development of interstitial pneumonitis failed to respond to high-dose corticosteroid therapy including pulse therapy. This patient eventually died 8 days after the onset of pulmonary symptoms. In spite of the normal level of CPK and LDH in the laboratory examinations, this case had a poor prognosis. We emphasize the fact that the pulmonary disease may sometimes occur in dermatomyositis and the CPK level in the serum does not always reflect the activities of dermatomyositis.

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We describe a 39-year-old male patient with malignant melanoma on his right lower leg of five years’ duration in association with numerous pigmented spots on all body sites. A microscopic examination of the lesion on the lower leg, which had been resected at another hospital, revealed nodular melanoma, Clark’s level IV with a maximal tumor thickness of 3.5 mm. There was a metastasis in the right inguinal lymph node. A physical examination revealed a total of 177 pigmented macules ranging from 2 to 12 mm in diameter. Two of these pigmented macules with either a reddish hue or a notched border were histologically diagnosed as dysplastic nevi due to the lentiginous nested hyperplasia of the melanocytes, the bridging of nests, a dermal lymphocytic response and lamellar fibroplasia. This is the eighth report of a Japanese patient developing malignant melanoma in association with dysplastic nevi.

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A case of nerve sheath myxoma on the right buttock of a 16-year-old female is reported. The tumor was skin-colored and elastic hard in consistency. A similar nodule was palpable in the dermis. No café-au-lait spot was observed anywhere on the body. A lobulation was noticed on the cut surface of the resected mass, and light microscopy revealed mucinous lobules gathered in the dermis. Spindle-shaped cells were noticed in each lobule and showed little pleomorphism, thus leading to the diagnosis of nerve sheath myxoma. Immunohistochemically, S-100 protein was weakly positive and epithelial membrane antigen was negative in the tumor cells, raising the possibility that this tumor might be derived from Schwann cells.

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A 53-year-old Japanese male was first examined in July 1991, because of a painful dome-shaped, dark red nodule on the left temporal scalp. The surface of the nodule was smooth and it was 17×15×11 mm in size. The tumor was excised surgically and examined by histopathological and immunohistochemical studies. The histological features revealed a well-demarcated solitary mass localized in the upper and middle dermis. A connection to the epidermis through a comedone-like conduit was seen. Tubular structures were lined by two layers of epithelial cells, and the inner layer of cells showed decapitation secretion. The luminal continent was PAS positive and diastase resistant, but the mucicarmine was negative. Immunohistochemically, CEA and S100 protein were both positive at the luminal surface of the tumor cells.

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A case of asymptomatic lichen amyloidosus which occured on the lower legs of a 37-year-old man was in reported. Histopathologically, the features of the HE-stained specimens were compatible with those of lichen amyloidosus. Under light microscopy, however, the deposits stained negatively with Congo red and only faintly showed positive with Dylon. The same sections did not demonstrate birefringence under polarized light either. These findings were thus quite inconsistent with amyloid. However, when the Congo red and Dylon stained sections were viewed under fluorescence microscope, the deposits demonstrated an orange fluorescence. Immunohistochemically, the deposits reacted positively with anti-amyloid P component antibodies and anti-keratin antibodies. The findings of the electron microscope were compatible with amyloid. These results therefore confirmed that the deposits were amyloid. The skin condition of the patient improved after discontinuing the use of a nylon towel for bathing and applying steroid ointments. We believe that an accurate diagnosis may be quite impossible in such a case without the use of a fluorescence microscope.

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A sixteen-year-old female patient with intractable atopic dermatitis is reported, who showed marked beneficial response to the oral administration of a Chinese drug, Tsu-Do-San. In addition to the clinical effects, Tsu-Do-San therapy reduced her serum LDH level, serum IgE level and RAST scores. Other previously reported prescriptions by Chinese drugs for atopic dermatitis are also briefly summarized.

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The alkaline DNA extraction method was applied for the isolation of human papillomavirus from warts. Tissue samples from common and plantar warts were digested with proteinase K, and the extrachromosomal circular covalently-closed form of HPV-DNA was observed to be rapidly extracted by both alkaline sodium dodecyl sulphate and phenol-chloroform treatment. The recovery of HPV-DNA from the tissue was sufficient for a determination of endonuclease restriction patterns by agarose gel electrophoresis.

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The suitable experimental conditions for a MTT assay in order to assess the cytotoxic effect of various drugs on a human malignant melanoma cell line (YU GEN 8) was detetmined. The following conditions were found to be suitable: (1) an initial cell density of YU GEN 8; 30×10⁴ cells/ml, (2) a quantity of MTT solution (5mg/ml); 20μl. Using this system, the effect of three kinds of drugs on the cell growth was evaluated. Bu-3818E (BMY-28565, feldamycin) and Bu-3819E (BMY-28566, melanostatin), both of which were potent melanin synthesis inhibitors for mouse B16 melanoma cells, did not suppress the growth of the YU GEN 8 cells. However, Bu-3862T (BMY-28647, eponemycin), which had been previously reported to suppress mouse B16 melanoma cells, also suppressed the growth of human YU GEN 8 cells. In conclusion, the growth of the human melanoma cells appeared to be independent of the melanin synthesis of the cells.

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Sun protection factor (SPF) is one of the objective guides for evaluating sunscreens. In this study, we investigated the SPF of a newly designed sunscreen against UVA+B obtained from a solar simulator. We also determined the preventive effect of delayed tanning (DT) and immediate pigment darkening (IPD). The geometric mean of the SPF was either 15.0 or greater. The protection factor (PF) with DT was 12.0 or greater and the PF with IPD was 85.3. This sunscreen was thus found to provide a high degree of protection against erythema, as well as DT and IPD.

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We carried out a clinical observation of 24 patients with measles who visited the Division of Dermatology, Maizuru Kyosai Hospital from February to May in 1992.
1) The ages of the patients ranged from 13 to 29 years old (the mean age was 18.7 years old). Eleven cases were males and 13 were females. Six patients had been previously vaccinated against measles.
2) As to symptoms, fever, cough and Koplik’s spots were seen in 100%, 96% and 79% of the cases, respectively. Hemorrhagic eruptions were seen in 13% of the cases. The symptoms were mild in some of the vaccinated patients.
3) In a cytodiagnosis of the oral mucosa, multinucleated giant cells were observed in 67% of the cases. It was considered to be a useful examination for differentiating measles from rubella and erythema infectiosum.
4) Regarding the antibodies to measles, hemagglutination-inhibiting (HI) antibody increased significantly in 95% of the cases and IgM antibody was detectable in 95% of the cases.
5) Immediately after the onset of the disease, a laboratory examination showed leukocytopenia, lymphocytopenia, thrombocytopenia and a nuclear shift of neutrophil to the left. About one week after onset, it showed an increase of atypical lymphocytes in 83% of the cases and an elevation of LDH value in 79% of the cases.
6) As for complications, otitis media, herpes simplex and encephalitis were each observed in one case. As for liver dysfunction, an elevation of the GOT and GPT values was observed in 63% of the cases, while many cases showed a mild elevation.
7) One of the cases was in pregnancy. The course of pregnancy was uncomplicated and a normal healthy baby was delivered at term.

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The clinical effect of 20% azelaic acid ointment on hyperpigmented disorders such as chloasma, ephelides and reticulate acropigmentation of Kitamura was investigated. Azelaic acid ointment was applied to the patients twice a day, and the clinical effect was evaluated using a color-difference detector. A clinical benefit was observed in 14 out of 20 patients. On the other hand, skin irritation was observed in 2 out of 20 patients. This study shows that azelaic acid ointment can be useful for patients with hyperpigmented disorders.

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A 22-year-old woman with atopic dermatitis consulted us with a chief complaint of facial erythema. Since the erythema was resistent to ordinary therapies for 8 weeks, we additionally administered two types of Chinese herbal medicine, Orengedoku-to (EK-15) and Kikyo-sekko (K324). After 3 weeks the erythema improved obviously. The clinical effect of these Chinese herbal medicines on facial erythema in this case is supposed to have been brought about by the anti-inflammatory, anti-allergic or anti-bacterial activity of the drugs and their components.

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[in Japanese]

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