Hideoki Ogawa

Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity.

Generalized pustular psoriasis (GPP) is a rare but notoriously recalcitrant cutaneous diseases. Therefore, there have been few reports of more than ten patients with GPP who were treated at the same institution. The severity of this disease and its response to each therapeutic modality vary among patients. In some GPP is life-threatening, but in others it may show a benign, chronic course for a long period of time. Before starting treatment, a knowledge of the therapeutic efficacy and side effects of each drug used in the treatment of GPP is necessary. In our multicenter study, we compared the effectiveness of and adverse reactions to several systemically administered drugs. Following the development of a unique classification of the disease severity based on scoring the clinical symptoms and the laboratory findings, we propose here therapeutic guidelines for the treatment of GPP.

History of the establishment and revision of diagnostic criteria, severity index and therapeutic guidelines for pemphigus in Japan.

We summarize the process of establishing and revising the diagnostic criteria, severity index and therapeutic guidelines for pemphigus in Japan (including the results of a nationwide survey regarding these guidelines). We also summarize the content and present an evaluation of the utility of these guidelines. Due to the publication of these documents throughout the Japanese medical community, it appears that patients with pemphigus have recently begun to receive appropriate treatment, dramatically improving their quality of life and prognosis. Continuous examination of these criteria by means of follow-up studies of patients treated according to the guidelines is necessary to determine the long-term efficacy of treatment. To this end, it is hoped that these guidelines will be more extensively disseminated among the medical community.

A Case of Unilateral, Systematized Linear Hair Follicle Nevi Associated with Epidermal Nevus-Like Lesions

Multiple hair follicle nevi are an extremely rare condition. In 1998, a case of unilateral multiple hair follicle nevi, ipsilateral alopecia and ipsilateral leptomeningeal angiomatosis of the brain was first reported from Japan. Very recently, hair follicle nevus in a distribution following Blaschko’s lines has also been reported. In this paper, we observed a congenital case of unilateral, systematized linear hair follicle nevi associated with congenital, ipsilateral, multiple plaque lesions resembling epidermal nevi but lacking leptomeningeal angiomatosis of the brain. These cases implicate the possibility of a novel neurocutaneous syndrome. Additional cases should be sought in order to determine whether this condition is pathophysiologically distinct.

Recurrent E413K Mutation of hHb6 in a Japanese Family with Monilethrix

Monilethrix is an autosomal dominant hair disorder characterized by a beaded appearance of the hair due to periodic thinning of the shaft. This disorder has been reported to be caused by mutations in the helix termination motif of two type II cortex keratins, hHb1 and hHb6. Here we describe a Japanese monilethrix family that has the most frequent mutation, the E413K mutation in hHb6, so far found in 26 families. Genotype/phenotype correlation was not obvious in our case or in the previously reported cases.

A case of Bowen's disease showing a clinical tendency toward spontaneous regression

We present a case of Bowens disease showing a clinical tendency toward spontaneous regression. The patient, a healthy 86‐year‐old woman, complained that erythema had appeared on her left forearm two years earlier and had gradually enlarged. At the first examination, we observed a well‐demarcated, 4 × 3 cm, erythematous plaque, partically covered with crusting and erosions near the wrist. Diagnosis of Bowens disease was confirmed by a biopsy. Since the patient refused surgery and also discontinued hyperthermic treatment with disposable chemical pocket warmers after a brief trial, we decided to continue the observation of disease progression without any treatment. Two years after the initial visit, the lesion showed a clinical tendency toward spontaneous regression, with a fine erythematous plaque that showed the obscurely demarcated border of the lesion. After three years, although the patient exhibited a similar symptomatic improvement, a skin biopsy showed a few residual tumor cells. At the patients request, we chose to observe the progress of the lesion. We review the literature of cases of Bowens disease that have shown a similar tendency toward spontaneous regression, which have been rare.

Superficial mucoceles of the soft palate.

Introduction Mucoceles of the minor oral salivary glands are common lesions that result from damage to the excretory duct that leads to pooling of mucus into the connective tissue [1]. They frequently occur in the lower labial mucosa. Mucoceles in the mucosa vary from 1–2 mm to 1 cm in diameter. The clinical appearance of the mucoceles depends on the depth; with a larger diameter, the mucocele is likely to protrude from the mucosa and have a more domed shape. Extravasation of mucus within the tissues, epithelium or immediately below the epithelium generates a small blister, creating a lesion known as a ‘superficial mucocele’ [2]. Superficial mucoceles occur most often in the soft palate and buccal mucosa and can be misdiagnosed as bullous lichen planus, herpes simplex and mucous membrane pemphigoid. We report a case of superficial mucoceles and discuss its pathogenesis and mechanism.