A 36-year-old woman at 28 weeks of gestation was referred to our clinic for evaluation of purpura on her lower limbs. A physical examination revealed palpable purpura spots on her buttock and lower extremities. Urinalysis revealed proteinuria and hematuria. A histopathological examination showed numerous extravasated red blood cells and neutrophilic infiltration in the dermis, as well as fibrinoid degeneration in the vascular wall. Direct immunofluorescence technique showed immunoglobulin A deposition in the vascular wall. A diagnosis of Henoch-Schönlein Purpura was made. We decided to initiate treatment with oral corticosteroids, and her symptoms disappeared. At 39 weeks of gestation, she delivered a healthy baby. During pregnancy, it is difficult to choose a treatment because of potential adverse effects in the fetus. According to earlier 24 case reports, Henoch-Schönlein Purpura often causes severe nephritis, and some cases resulted in fetal death. We should, therefore, pay attention to the conditions of both the patient and fetus in the Henoch-Schönlein Purpura cases in pregnant women. If there is a possibility for development of a serious condition in patients with Henoch-Schönlein Purpura, it is important to explain the risk carefully to the patient and perform the appropriate treatment.

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A 56-year-old man had felt pain and numbness of the left foot for 6 months before visiting our hospital. A physical examination revealed livedoid lesions on his lower legs and feet, and cyanosis with ulcerations on his left toes. A histopathological examination showed vascular extension and hyperplasia in the upper dermis, and hypertrophy of the vascular wall in the middle dermis. Routine blood analysis showed a marked increase of platelet counts. The ensuing investigation was concluded with a diagnosis of essential thrombocythemia. A treatment using hydroxyurea led to improvement of the digital ulcers along with a decrease of platelet counts. We should note that livedo or cyanosis with ulcerations of the feet may be caused by essential thrombocythemia.

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A 54-year-old Japanese man with diabetes mellitus and chronic renal failure developed multiple finger and hand necroses. He had received hemodialysis for 13 years. Six months before the appearance of his current symptoms, an arteriovenous shunt on his left forearm became occluded by local bacterial infection. A new arteriovenous shunt was created on the right forearm. However, blood flow to the right hand gradually decreased even though percutaneous transluminal angioplasty was performed several times to prevent shunt stenosis. Multiple finger and hand necroses occurred and spread after a slight injury. An angiogram showed increased blood flow after pressing the arteriovenous shunt, which indicated a diagnosis of blood steal syndrome. Brachial-radial artery bypass successfully overcame his ischemic problem, and the necrotic ulcers rapidly healed after debridement and negative pressure wound therapy. Skin lesions of dialysis patients are difficult to diagnose and treat, and it is necessary to observe them carefully.

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A 58-year-old woman had previously visited another clinic with symptoms of her right hand, which was swollen with itching lasting about 1 month, despite the absence of particular triggers. Although the patient received topical steroid therapy, her symptoms did not improve, leading to a referral to our department. Three elastic hard subcutaneous nodules over 1 cm in diameter were palpated on the dorsum of the right hand. X-ray and computed tomographic images of the dorsum of her right hand showed no marked changes, but magnetic resonance imaging revealed areas of low intensity on T1- and T2-weighted images at the sites of the subcutaneous nodules. A skin tissue examination of a portion of one of the nodules revealed a naked granuloma, and a chest X-ray showed bilateral hilar lymphadenopathy. Moreover, gallium-67 scintigraphy detected abnormal accumulations in the pulmonary hilum, the dorsum of the right hand, and a parotid gland, which led to a diagnosis of subcutaneous sarcoidosis. Oral administration of tranilast 300 mg/day was initiated, and it achieved resolution of the clinical features. In this manner, subcutaneous nodules are often of great diagnostic value for making a diagnosis of sarcoidosis. Especially, MRI imaging was not only a diagnostic aid but could also provide useful information about the number and location of nodules. Interestingly, oral administration of tranilast 300 mg/day could lead to resolution of the clinical features in the present case.

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We report two cases of hypomelanosis of Ito. Case 1) A 7-month-old boy presented with mitral incompetence, opened arterial ligament of right aortic arch, cryptorchidism, left-ear deafness, overlap of both 5th fingers, and unusual facial contours since birth. Tenioid hypopigmented whorls or streaks following Blaschko lines were found on the trunk and limbs with Unna's nevus on the forehead and the regio occipitalis capitis. Erythema or vesicles were not observed throughout his clinical course. We diagnosed the patient as hypomelanosis of Ito. A chromosomal analysis was normal, and magnetic resonance imaging of the head showed no apparent abnormalities. Histologically, Fontana-Masson staining showed a decreased concentration of melanin granules in the region of the hypopigmentation without any incontinentia pigmenti. In the affected hypopigmented area, a marked decrease of c-KIT-, MITF- and tyrosinase-positive cells was evident compared with the surrounding normal skin. Case 2) A 1-year-old girl presented with aproctia, both talipes calcaneovalgus, syndactyly of the right 3rd, 4th and 5th toes, syndactyly of the left 4th and 5th toes and left hypoplastic kidney since birth. Tenioid hypopigmented whorls or streaks following Blaschko lines were observed on the limbs, leading to a diagnosis of hypomelanosis of Ito. Hypomelanosis of Ito is frequently associated with serious complications such as abnormalities of the central nervous system. Careful dermatological observation is mandatory for diagnosing hypomelanosis of Ito.

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A 1-month-old girl presented with multiple confluent plaques of varying sizes that were slightly raised from the skin surface on the mid-back to the right side and had been present since birth. The plaques were generally pale-yellow in color with a granular surface. Histopathologically, the lesions showed immature hair follicles with mild papillomatosis and hyperplasia of the sebaceous glands, and organoid nevus was diagnosed. The distribution pattern appeared to be type III (phylloid pattern) of Happle's mosaic distribution pattern. At present, no other abnormalities besides skin symptoms have been observed, for example, in the central nervous system and skeletal system. However, keeping sebaceous nevus syndrome in mind, careful observation should be considered due to the pathognomonic distribution of plaques.

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Here we report on four cases of angiosarcoma in a lower leg ; the patients were admitted to Kyushu University Hospital between 2008 and 2012. One of the cases was a lymph node metastasis from a lower leg 15 years before the admission. All four cases were female, and their ages when they were first diagnosed as angiosarcoma were 39, 49, 67 and 76 years, respectively. Three of the four cases had a history of chronic lymphedema, so we diagnosed the condition as Stewart-Treves syndrome. Multimodal treatment using a combination of surgery, chemotherapy and radiotherapy was performed. Compared with the previously reported cases of angiosarcoma in the lower leg and with those in the head and neck region, relatively younger ages and female dominance were observed. Most cases of angiosarcoma in a lower leg were associated with chronic lymphedema, which is referred to as Stewart-Treves syndrome. We should be aware of this tumor when managing patients suffering from chronic lymphedema.

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A 59-year-old male presented with a dome-shaped protruding subcutaneous mass on his right upper arm. It had enlarged to 5 cm in diameter at 8 months. We diagnosed the tumor as undifferentiated high grade pleomorphic sarcoma based on the pathological findings. Systematic examination demonstrated no metastatic lesions at that time, so we performed secondary wide resection with safety margins. However, multiple lung metastatic lesions appeared 10 months after the wide resection. We conducted combination chemotherapy with adriamycin and ifosfamide. The metastatic lesions disappeared after five cycles of chemotherapy. However, the lesions relapsed after 10 months. Thereafter, the number of metastatic lesions increased, and the patient died 4 years and 6 months after the wide resection. The standard therapeutic modality for non-round cell soft tissue sarcoma, containing undifferentiated high grade pleomorphic sarcoma, is surgical resection and radiotherapy. Chemotherapy is needed for metastatic lesions, but there is no standard chemotherapy for non-round cell soft tissue sarcoma to date. However, there have been some studies reporting the effectiveness of chemotherapy in reducing the recurrence and death rates of localized resectable soft tissue sarcoma. In our patient, combination chemotherapy may have improved the prognosis.

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This study assessed the efficacy of once-daily layered application of a betamethasone butyrate propionate lotion (ANT-L) and a calcipotriol ointment (DOV-O) and the influence of this combination on the quality of life (QOL) of 59 psoriasis vulgaris patients with skin eruptions refractory to a topical treatment with a steroid ointment alone or in combination with an active vitamin D₃ (VD₃) ointment. For analysis, the subjects were stratified according to the previous treatment received. The previous treatment comprised monotherapy with a very strong (VG) or higher-class steroid ointment in 35 patients and combination therapy with a VG or higher-class steroid ointment plus a VD₃ ointment in 24 patients. In both groups, skin symptoms and QOL of the patients significantly improved after the study treatment. The study results indicate that oncedaily topical layered application of the DOV-O/ANT-L combination therapy is efficacious in treating psoriasis vulgaris refractory to a previous topical treatment. The changes in the study treatment from the previous treatment involved a change of the dosage form of the steroid medication from ointment to lotion as well as either addition of or change to DOV-O from a VD₃ ointment. Improved treatment compliance could be a major factor influencing the efficacy of the layered DOVO/ANT-L combination therapy.

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We conducted a post-marketing prospective surveillance of the efficacy and safety of famciclovir among herpes zoster patients with renal dysfunction. Fifty-three herpes zoster patients with a creatinine clearance (Ccr) of less than 90 ml/min, including 9 patients treated with hemodialysis for renal failure, were recruited. Following the recommended dosages in the package leaflet, famciclovir was administered to each individual according to their Ccr. The assessments of general physicians, the pain associated with the lesions, renal function, and side effects were recorded before and after the famciclovir treatment. Overall efficacy assessments of “Markedly improved” and “Improved” accounted for 90.4% of the findings, and no assessments of “Unchanged” or “Worsened” were recorded. The efficacies in the group with a Ccr of less than 20 ml/min and in the group receiving hemodialysis were 75.0% and 77.8%, respectively ; these efficacies were not statistically different when compared with those of the other groups. No adverse drug reactions were reported in the participants. Famciclovir did not alter the Ccr and did not have any adverse effects on renal function after herpes treatment. To our knowledge, this is the first prospective study to assess the efficacy and safety of anti-viral treatment for herpes zoster patients with renal dysfunctions. These data indicate that famciclovir is a safe and effective choice for patients with renal dysfunction.

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