Anti-TNFα (tumor necrosis factor-α) agents, adalimumab and infliximab, are the first biologics approved for the treatment of psoriasis in Japan. Although these agents are expected to have remarkable effects on psoriasis and/or psoriatic arthritis, and to improve QOL (quality of life) of patients with psoriasis, there is growing concern regarding the potential serious adverse reactions such as infections including tuberculosis as well as bacterial and Pneumocystis pneumonia. The guideline and safety manual have been developed to ensure that anti-TNFα agents are properly used by board-certified dermatologists experienced in practice of psoriasis. Requirements for physicians and medical institutions, eligible patients for treatment with anti-TNFα agents, dosage and administration of each anti-TNFα agents, general considerations for administration, contraindications, safety precautions for high-risk patients for adverse reactions, and recommendable combination therapies with anti-TNFα agents are referred.
A surgical resection continues to be the basic treatment for skin cancer, but, as the numbers of elderly patients and patients with complications have increased, the numbers of cases that are not ideal candidates for surgery have also been increasing. In 2006, we adopted topical photodynamic therapy (PDT) using 5- aminolevulinic acid as one type of noninvasive treatment that can be performed on such patients. We herein report that we performed PDT using pulsed dye laser with a skin-cooling device on 7 cases of extramammary Pagetʼs disease with good results. These results were evaluated by clinical findings, photodynamic diagnoses, and skin biopsies；the results included CR in 2 cases and PR in 5 cases. Three of the PR cases were resolved or substantially ameliorated by surgical resections that were possible with local anesthesia due to reductions in the size of the lesions. Such reductions in the sizes of the lesions were observed in all of the cases. Although it is therefore necessary to be careful when selecting candidates, however, PDT is nevertheless considered to be a useful treatment for patients with extramammary Pagetʼs disease.
We report a case of mutilans-type psoriatic arthritis complicated by secondary amyloidosis. The patent was a 75-year-old man diagnosed with psoriasis vulgaris 15 years previously. He was admitted to our hospital for evaluation of a large area of psoriatic lesions and severe leg edema. Laboratory examination revealed normocytic anemia, renal dysfunction, and proteinurea. He had been suffering from arthralgia and joint deformity, for 8 years. Based on X-ray and bone scintigraphy examinations, he was diagnosed with mutilans-type psoriatic arthritis. Biopsy specimens from the lip mucosa revealed Dylon-stained amyloid deposits. Together, these findings indicate that systemic amyloidosis related to psoriatic arthritis led to renal dysfunction, causing leg edema, anemia, and proteinurea.
A 59-year-old male had amelanotic melanoma arising in hyperkeratotic lesions of the heel which had been present since childhood. He sometimes shaved them. There were no such lesions on his palms. The same lesions were recognized in his mother, brother and two of his children. Based on the positive family history and clinical manifestations, the hyperkeratosis is strongly suspected to be hereditary plantar keratoderma. He had noticed erosions and a red granulomatous tumor on his right heel year before the first visit to our hospital. A biopsy specimen obtained from the tumor histologically revealed malignant melanoma. Subsequently, the tumor was surgically removed completely with a 30 mm margin. Sentinel lymph node biopsy revealed positive nodes in the inguinal and popliteal areas; 15 inguinal lymph nodes and 2 popliteal lymph nodes were dissected, and 3 inguinal lymph node and 2 popliteal lymph node metastases were found, leading to the diagnosis of stage IIIC (pT4bN3M0). After the operation, five courses of DAV-Feron chemotherapy were followed by continuous local Feron injection twice a month. One and a half years later, right external iliac lymph node metastasis was found. and the intrapelvic lymph nodes were dissected. Two years after the operation, multiple lung and liver metastases were evident, and he died of liver failure. The association of malignant melanoma with hereditary palmoplantar keratoderma is relatively rare. Chronic mechanical irritation may have affected the onset of malignant melanoma of hereditary plantar keratoderma in this case.
A 54-year-old woman was diagnosed with primary Sjogren syndrome (SjS) in 2001. She has been receiving predonisolone per os, but some erythemas and papules over her lower abdoman to genitalia appeared in January of 2007. These eruptions were repeatedly excised at a former hospital, but they continued to increase, chiefly in the anogenital area. Histological examination showed that the skin lesions demonstrated dense and nodular infiltrations of mononuclear cells, and biopsies from skin and minor salivary glands revealed large amounts of amyloid deposition. Computed tomography of her chest and parotid gland revealed an involvement of amyloid deposition in her parotid gland. In addition, Southern blot hybridization assays of skin lesions demonstrated a monoclonality, as well as an immunoglobulin heavy chain gene rearrangement. These findings yielded a diagnosis of primary cutaneous marginal zone B-cell lymphoma (MZL) and amyloidosis in a patient with SjS. So far as we can determine, this is the first report of a case consisting of these 3 disorders; their mechanisms and interactions still remain to be clarified.