Since the discovery that inactivation of the human patched gene is responsible for the nevoid basal cell carcinoma syndrome and sporadic forms of basal cell carcinoma (BCC), a role for dysregulation of hedgehog signaling in tumorigenesis of BCC has been firmly established. Other key members of this pathway, such as sonic hedgehog, smoothened, and the Gli family of transcriptional factors, have also been shown to be involved in tumor formation of BCC. Recently, the plant-derived teratogen cyclopamine has been shown to inhibit the hedgehog response and is expected as a potential `mechanism-based’ therapeutic agent for the treatment of BCC. Dysregulation of hedgehog signaling was also demonstrated in trichoepithelioma, which has morphological similarity to BCC. However, whether other appendageal tumors including trichoblastomas and those arising from nevus sebaceous also include hedgehog signaling abnormalities is controversial. For extramammary Paget’s disease, new therapeutic approaches targeting the erbB-2 oncogene and possible androgen dependence of this tumor have been suggested. An innovative technology of DNA microarrays, which allows the mathematical analysis of expression of thousands of genes, has recently been introduced in genetic analysis for melanoma. This analysis may identify unrecognized subtypes of cutaneous melanoma with different biological potentials.
In this paper, we report three cases of atheroscleroris obliterans with ischemic foot ulcer who were successfully treated with vascular reconstruction. These patients complained of recalcitrant ulcers with coldness and severe ischemic pain in their feet. Topical ointment treatment and repeated intravenous injections of prostaglandins were ineffective. They had histories of cerebral infarction or congestive heart failure, and two of them concomitantly had diabetes mellitus. Angiography demonstrated multiple stenosis or occlusion of the femoral or popliteal arteries. The clinical manifestations improved along with the improvement in the foot blood flow after performing vascular reconstruction. Therefore, vascular reconstruction should be considered for the management of critical ischemia in atherosclerosis obliterans for limb salvage.
We describe a 51-year old female patient with amyopathic dermatomyositis complicated by interstitial pneumonia and pneumomediastinum and successfully controlled by steroid therapy. She had been suffering from amyopathic dermatomyositis associated with Sjögren syndrome and autoimmune hepatitis and was hospitalized because of exertional dyspnea due to interstitial pneumonia. The pneumonia improved immediately after the pulse administration of a large amount of methylpredonisolone, but pneumomediastinum then developed. It disappeared after 4 months rest and the administration of 40 mg/day of oral prednisolone. Although pneumomedastinum associated with dermatomyositis usually has a poor prognosis, she has been in remission with a maintenance dose of 10 mg/day of oral predonisolone.
A 46-year-old male had chronic non-pruritic urticaria, recurrent fever, lymphadenopathy and a monoclonal IgM dysproteinemia, but no malignant lymphoplasmacytic proliferation was detected by bone marrow biopsy, lymph node biopsy or systemic examination. Therefore, the patient was diagnosed as having Schnitzler’s syndrome (SS). His urticaria, fever and lymphadenopathy were not alleviated by administration of nonsteroidal anti-inflammatory drugs and antihistamine drugs but were greatly improved by steroid pulse therapy. However, the symptoms recurred upon the reduction of steroids. Double filtration plasmapheresis (DFPP) was then performed. The urticaria was alleviated immediatedly after DFPP but recurred during prolonged intervals between treatments. The patient then developed elevation of the serum IgM level, fever and aggravation of urticaria and was treated by 3-cool chemotherapy (DMVM-IFNα therapy). The urticaria was alleviated by the chemotherapy but recurred shortly these after. The serum IgM level was still controlled as of 5 months after the chemotherapy, and, after the last reduction of prednisolone, no fever has arisen. Although a treatment method for SS has not been established, the combination of the above 3 characteristic treatments was considered useful for a control of the syndrome.
We report a case of 6 year-old girl with pyoderma gangrenosum and hyperzincemia. She had been suffering from fever, elevation of CRP and liver dysfunction and followed for e latent Epstein Barr virus infection since infancy. She developed painful undermined ulcers surrounded by itchy erythema on her head, neck, and lower extremities at the age of four. Laboratory findings showed anemia, thrombopenia, and elevation of CRP. Phagocytosis of neutrophils was decreased, but T cell function was normal. Serum zinc was extremely elevated, although the urine zinc level was normal. Hepatosplenomegaly was shown in abdominal CT. Skin biopsy specimen revealed a dense infiltration of neutrophils in the dermis. A deep ulcer developed at the biopsied site. We diagnosed this case as pyoderma gangrenosum and started systemic steroid therapy. We could not find any inflammatory bowel disease, lymphoproliferative disorder, autoimmune disease, or severe immunodeficiency. Recently, hyperzincemia has been reported in immunocompromised cases, in some of whom cutaneous lesions, pyoderma gangrenosum, and vasculitis have appeared. We describe herein an association between hyperzincemia and pyoderma gangrenosum with a review of the literature.
We described a 67-year-old man with relapsing, linearly arrayed papules and vesicles on the left leg for the previous three years. Light microscopic observation revealed epidermal acantholysis with inflamatory cell infiltration. The patient was diagnosed as having transient acantholytic dermatosis (TAD) with linear configuration following the lines of Blaschko. Only two TAD cases have been reported with a zostoriform distribution. Our case may be a first report of TAD with a linear pattern.
For patients with atopic dermatitis, long-term treatment is usually inevitable and various limitations are subsequently placed on their daily life. Therefore, the influence on the patient on the new innovative pharmacotherapy for atopic dermatitis is thought to be extremely significant from an economical viewpoint. In this study, we attempted the measurement of WTP for the newly developed therapeutic agent, “Tacrolimus”. A group of 1,000 adult men and women and living in the Tokyo metropolitan area were selected by random sampling for the interview survey, and a total of 431 interviews were completed. The results indicate that the average person would be willing to pay up to 11,500~11,700 yen a month for pharmacotherapy with “Tacrolimus”. This information is considered to be beneficial for policy decision-making such as reimbursement decisions.