A 33-year-old man whose medical history included acute disseminated encephalomyelitis was admitted to our hospital with acute tonsillitis in February 2008 with high fever and diarrhea. Antibiotics and antivirals were not effective. Three days after hospitalization, his level of consciousness worsened and he was diagnosed with encephalitis. When he consulted a dermatologist, he had many erythematous eruptions with erosion on his face and his legs. Histopathologic examination of the erythema on the eyelid of the patient demonstrated dermal infiltration with numerous neutrophils. FLAIR MRI imaging showed high signal intensity. A final diagnosis of neuro-Sweet Disease was made by brain MRI and skin biopsy. After systemic administration of corticosteroids therapy, his tonsillitis and erythema improved, although slight fever remained. All his symptoms disappeared after starting colchicine. On HLA typing, B54 and Cw1 were positive. Careful attention should be paid to neurological symptoms when examining a patient with neuro-Sweet disease.Skin Research, 11: 197-201, 2012

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A 7-year-old girl developed skin eruption of maculopapular, erythematous and purpuric lesions on the face, elbows, and knees. In addition, palpable purpuras were present on the legs and the dorsum of the feet. Antineutrophil cytoplasmic autoantibodies (ANCA) with antimyeloperoxidase specificity (MPO-ANCA) were positive. Microscopic hematuria was detected but a renal biopsy specimen disclosed no abnormalities. A skin biopsy from a palpable purpura showed leukocytoclastic vasculitis of the small vessels in the entire dermis. The patient was diagnosed as having microscopic polyangitis (MPA). After methylprednisolone pulse therapy, treatment with oral prednisolone was started, resulting in complete regression of all symptoms, including skin lesions and disappearance of MPO-ANCA. However, the nodules on the knee reappeared with a concomitant rise in MPO-ANCA. The dose of prednisolone was increased and mizoribine was added. The combined therapy improved the nodules on the knee with disappearance of MPO-ANCA. However, the nodules on the knee have never completely regressed and they have intermittently worsened and are associated with slight elevation of MPO-ANCA. In the present patient, the severity of skin lesions correlated well with the levels of MPO-ANCA, suggesting that MPO-ANCA may play a role in the pathogenesis of the skin lesions.Skin Research, 11: 202-208, 2012

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Monozygotic twin brothers, age 2, presented with vast erythema and pustules on their whole body, accompanied with 38°C fever after taking amoxicillin for a common cold. Skin biopsy showed subcorneal pustules. They were successfully treated with systemic prednisolone. They were diagnosed with acute generalized exanthematous pustulosis (AGEP), on the basis of positive patch test results with amoxicillin. Three months later, the younger sibling showed a sudden onset of high fever, vast erythema and pustules without any prior medication. He was diagnosed with pustular psoriasis due to his clinical presentation. Three months later, the older sibling also showed the same symptoms. They are being treated with cyclosporine and methotrexate, but show occasional relapse. There is no generally established theory concerning the relationship between AGEP and psoriasis, but from this monozygotic twin case, we suspect that there may be a genetic similarity in the two diseases.Skin Research, 11: 209-214, 2012

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We report a 4-month-old male infant with dermatosis induced by biotin deficiency. He had been fed by hydrolyzed milk since a few days after birth, because of frequent vomiting after each breast feeding. He was suspected to have food protein-induced enterocolitis syndrome. At 4 months of age, well-demarcated erosive erythema at intertriginous areas and hair loss of the scalp appeared and gradually worsened. Biotin deficiency was suspected as he had been fed only hydrolyzed milk. His skin symptoms were significantly improved after two weeks administration of biotin 2mg/day. After finishing weaning at 8 months of age, cessation of oral administration of biotin induced no relapse of skin lesions. Nutritional deficiency of biotin is quite rare as it is contained in various foods and also synthesized by enteric flora. However, in Japan more than 30 infantile cases have been previously reported. Japanese milk formula for allergic or metabolic disorders rarely contains biotin because the addition of biotin to breast-milk substitutes is not legally allowed. Infants who have disorders that require nutritional support by only such formulas from the early newborn period have a high risk of biotin deficiency in Japan.Skin Research, 11: 215-219, 2012

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Abstract: A 51-year-old man went mandarin orange picking near Lake Hamanako in Shizuoka Prefecture 15 days before the initial consultation to our hospital, followed by fishing at a pond in Nagoya City, Aichi Prefecture. He developed high grade fever and headache 5 days after fishing. When he visited our hospital, he had fever of 38°C, headache, faint erythema on the trunk and extremities, a crust bite mark on the left lower leg, and enlarged lymph nodes in both inguinal regions. We suspected tsutsugamushi disease; therefore, a blood sample was subjected to PCR. Kawasaki genotype was identified. Close to full recovery was achieved after a two-week treatment with minocycline hydrochloride initiated at the initial consultation. When we see a patient with fever and eruptions, it is necessary to keep in mind the possibility of tsutsugamushi disease. It is important to make a rapid diagnose with PCR and start medical treatment in this disease.Skin Research, 11: 220-223, 2012

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