Skin Research, 15: 49-50, 2016

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A 44-year-old female was referred to our dermatology department with generalized eczema. She had been diagnosed with atopic dermatitis in childhood and was treated with corticosteroid ointments, but she had refused to use them for 10 years. As her eczema exhibited seasonal exacerbation, we performed a heat-loading sweat test, which revealed severe hypohidrosis her patient's face and trunk. As she had many tooth caries, we suspected that she had Sjögren's syndrome. A blood test produced positive results for anti-SS-A and anti-SS-B antibodies, and a lip biopsy showed numerous infiltrates of mononuclear cells around the minor salivary gland ducts. We administered topical therapy for atopic dermatitis, which resulted in the patient's hepatic enzyme levels rising. As she had Sjögren's syndrome, we searched for other autoimmune diseases, which led to her being diagnosed with primary biliary cirrhosis. Although hypohidrosis is a common manifestation of atopic dermatitis, clinicians should consider the possibility that it might be a complication of Sjögren's syndrome in severe cases. We also discuss the Th1/Th2/Th17 balance in relation to hepatic dysfunction during the treatment of atopic dermatitis.Skin Research, 15: 51-56, 2016

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A 34-year-old male noticed erythema on his bilateral lower legs in March 2009. The erythema gradually expanded to his upper limbs and he subsequently developed a fever. Although he was treated with cefazolin, his elevated d-dimer levels and reduced platelet count did not improve, and he was diagnosed with disseminated intravascular coagulation (DIC) of unknown cause. The DIC improved after the administration of nafamostat mesilate, but the administration of imipenem, oral steroids, and pulsed intravenous methylprednisolone were ineffective against the patient's sustained fever. Infection, a malignant tumor, and collagen disease were excluded after appropriate examinations. The patient was diagnosed with adult-onset still's disease (AOSD) based on the Yamaguchi diagnostic criteria. He was treated with various agents including oral corticosteroids, steroid pulse therapy, oral methotrexate, oral cyclosporine A, double filtration plasmapheresis, and oral colchicine; however, these treatments did not completely abrogate his condition and frequent repeated episodes of aggravation were observed. Meanwhile, he was found to have a high serum interleukin-6 level, and we administered tocilizumab together with oral steroids. Nevertheless, his condition still did not improve. Finally, complete remission was achieved with a combination of tocilizumab, oral steroids, and methotrexate. Further investigation is needed in order to evaluate the effectiveness of tocilizumab against refractory AOSD.Skin Research, 15: 57-62, 2016

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A 43-year-old female, who was taking 13mg oral prednisolone for systemic lupus erythematosus, visited our facility with a chief complaint of a vesicular rash on her buttocks. After admitting her based on a diagnosis of herpes zoster infection, an acyclovir infusion was administered at a dose of 250mg 3 times a day. Residual urine (750mL) was observed, but the patient did not exhibit any subjective symptoms, and a urine test confirmed the presence of pyuria. Despite treatment with oral cefdinir and urethral catheterization, the patient's body temperature increased to ＞38°C on day 12, and she exhibited hypotension, leading to a diagnosis of septic shock caused by a urinary tract infection. As Pseudomonas aeruginosa was detected in a urine culture, ceftazidime treatment was initiated. Subsequently, the shock and urinary tract infection were resolved; however, as neurogenic bladder persisted, she was discharged with a uterine balloon in place. When treating herpes zoster in the lumbar and sacral regions, it might be necessary to confirm the presence/absence of residual urine by chronologically measuring it.Skin Research, 15: 63-67, 2016

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A 73-year-old Japanese male was referred to us with intractable eruptions that were resistant to oral betamethasone and topical clobetasol propionate. The patient had noticed nodules on his neck and erythematous plaques on his trunk one year and three months earlier. Corticosteroid injections had been partially effective against the neck nodules. However, facial swellings and erosive lesions had subsequently appeared. A physical examination revealed slightly pruritic, dark reddish, indurated, and coalescing erythematous macules and plaques on the patient's face, trunk, and extremities. A biopsy specimen from the patient's neck showed follicular and perifollicular lymphocytic infiltration and mucin deposition, which resulted in a diagnosis of follicular mucinosis. Oral photochemotherapy was only briefly effective. The third biopsy specimen, which was obtained at 3 months after the first examination from a recurrent nodular lesion on the patient's neck, showed infiltrating atypical lymphocytes in and around the hair follicles. The eruptions were diagnosed as folliculotropic mycosis fungoides (FMF). Chemotherapy with pirarubicin, cyclophosphamide, vincristine, and prednisolone (THP-COP) was ineffective, and a metastasis to the brain was considered at 4 months after the initiation of chemotherapy, following the death of the patient. No previous studies have reported an association between brain metastasis and FMF in the literature. Cases of FMF involving peculiar clinical lesions and various histopathological findings can take a while to correctly diagnose. However, the present report suggests that cases of FMF with an aggressive course should be diagnosed and treated as soon as possible.Skin Research, 15: 68-74, 2016

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A 57-year old male was admitted to our hospital complaining of erythema, induration, and increasing left leg pain. Four days before his admission, he had developed a small erosive lesion in the interdigital region of his foot, the cause of which was unclear. His dorsum pedis subsequently became erythematous, swollen, feverish, and painful. The increasing pain made it hard for him to walk, and he visited a general hospital after being referred by a nearby clinic. He was hypotensive, and the inflammatory region had expanded rapidly. He was sent to our hospital with a suspected diagnosis of necrotizing fasciitis. On examination, his dorsum pedis was swollen and erythematous, and a region of brownish-red discoloration extended from his ankle to his lower leg. Based on a clinical examination, he was diagnosed with necrotizing fasciitis. He was given antibacterial drugs and taken to the operating room for surgical debridement. His condition improved soon afterwards. Six days after the debridement, negative pressure wound therapy (NPWT) was started. Twenty days later, the wound bed was covered with granulation tissue, and the NPWT was discontinued. We successfully prepared a wound bed for a split-thickness skin graft. On hospital day 50, he was discharged. No adverse events occurred, and the wound healed completely. Necrotizing fasciitis is a necrotizing soft tissue bacterial infection of the muscle fascia and subcutaneous fat. It has the potential to become quite severe. In such cases, surgical treatment is often required to save the patient's life. Here, we report a case in which necrotizing fasciitis was successfully treated using NPWT and skin grafting after rapid debridement.Skin Research, 15: 75-79, 2016

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A 77-year-old female was referred to us because of a gradually progressing erythematous and purplish indurated plaque on her right breast. She had been diagnosed with breast cancer at the age of 70. Breast-conserving surgery had been carried out, and the patient had subsequently received postoperative radiotherapy (targeted at the right breast). A physical examination revealed an asymptomatic, poorly demarcated, erythematous, variously gradated purplish, indurated plaque on the patient's right breast together with a blackish nodule, which measured 2cm in diameter. A biopsy specimen from the plaque showed vascular-like channels composed of pleomorphic cells together with faint luminal differentiation in the upper and middle dermis. We diagnosed the eruption as an angiosarcoma. The recent increase in the use of breast-conserving surgery and radiotherapy to treat breast cancer is likely to result in a rise in the frequency of radiation-induced angiosarcoma in Japan. In patients that receive radiotherapy for breast cancer, periodic examinations would help to prevent radiation-induced angiosarcoma progressing.Skin Research, 15: 80-84, 2016

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