Surgical Lymphoedema Treatment of Morbihan Disease: A Case Report.

ABSTRACT: Morbihan disease is a rare condition characterized by persistent erythema and solid edema of the upper two thirds of the face, leading to visual impairment and significant esthetic disfigurement. The underlying etiology of the disease remains uncertain, but its correlation with lymphoedema has been conjectured. Definitive treatment options are also lacking, and most current topical, systemic, and surgical interventions provide transient or partial results.In this report, we describe a case of Morbihan disease in a 32-year-old man. He suffered from erythema and swelling over the left periorbital region for 6 years, and was diagnosed with Morbihan disease. As the edema was unresponsive to drug therapy, he was referred to our department. Indocyanine green lymphography was performed, and the diagnosis of facial lymphoedema was confirmed. Manual lymphatic drainage was not effective, so we proceeded with surgical procedures. Lymphaticovenous anastomosis was performed to improve lymphatic drainage, and blepharoplasty was performed to debulk the enlarged tissue of lymphoedema. The visual field improved significantly, and the patient remains free from reexpansion of the lesion during the 1-year follow-up.The surgical approach for lymphoedema treatment can be a viable option for severe Morbihan disease unresponsive to nonsurgical therapies.

Topical immunotherapy in combination with anthralin in the treatment of refractory alopecia areata.

BACKGROUND: Treatment is often challenging in patients with alopecia areata. We often try topical immunotherapy to treat alopecia areata in Japan. Anthralin is sometimes used in other countries. OBJECTIVES: The aim of this study was to examine effectiveness of combination therapy with both topical immunotherapy with squaric acid dibutylester or diphenylcyclopropenone and anthralin in the treatment of refractory alopecia areata. METHODS: We treat four patients with refractory alopecia areata by topical immunotherapy and anthralin. Two patients had alopecia areata multilocularis and the other two patients had alopecia totalis. The entire scalp was treated with weekly application of squaric acid dibutylester or diphenylcyclopropenone and daily 0.5% anthralin ointment. Patients were followed up weekly, and adverse effects were recorded. RESULTS: One patient with multifocal patches of alopecia areata got complete hair regrowth at week 30, the other patient with multifocal patches of alopecia areata turned for the worse at week 30 and recovered at week 52. Hair regrowth was not seen in the other two patients with alopecia totalis. Localized pruritis and hyperpigmentation of the scalp were seen in two patients. CONCLUSIONS: To treat alopecia areata unresponsive to topical immunotherapy alone, topical immunotherapy in combination with anthralin is worth a try.

Novel and recurrent ATP2A2 mutations in Japanese patients with Darier's disease.

Darier's disease (DD, keratosis follicularis: OMIM#124200) is an autosomal dominant skin disorder characterized by multiple dark brown keratotic plaques and warty papules covered by thick crusts. Most cases of DD are caused by mutations in ATP2A2, which is expressed in both the skin and the brain. ATP2A2 encodes the cardiac muscle SERCA2a protein and the ubiquitously expressed SERCA2b. SERCA2 plays an important role as a calcium pump. It is thought that a mutation in ATP2A2 causes dyskeratosis and abnormality of cell-cell adhesion. Here, we report five DD patients from five independent families who presented or were referred to the Nagoya University Hospital in the past five years. We detected five mutations in ATP2A2, including a previously unreported mutation. We observed no apparent genotype/phenotype correlation between types and sites of the ATP2A2 mutations and DD phenotypes in the present series of DD patients. Genetic diagnosis from ATP2A2 mutation search is useful for the definite diagnosis of DD, although it is difficult to predict the severity and prognosis of skin symptoms from the results of the ATP2A2 mutation analysis in DD patients.

Antimicrobial susceptibility and molecular characteristics of 857 methicillin-resistant Staphylococcus aureus isolates from 16 medical centers in Japan (2008-2009): nationwide survey of community-acquired and nosocomial MRSA.

This study is a nationwide survey of all clinical methicillin-resistant Staphylococcus aureus (MRSA) isolates, including community-acquired MRSA (CA-MRSA), in Japan. A total of 857 MRSA clinical isolates were collected from the 16 institutions throughout Japan that participated in the survey (2008-2009). The drug susceptibility and staphylococcal cassette chromosome mec (SCCmec) typing and the presence of specific pathogenic genes were evaluated. The isolates comprised SCCmec type II (73.6%), type IV (20%), and type I (6%). The percentage of SCCmec type IV isolates was significantly higher in outpatients than in inpatients. Most of the isolated strains were sensitive to vancomycin (VCM, MIC ≤2 µg/mL), linezolid (MIC ≤4 µg/mL), and teicoplanin (MIC ≤8 µg/mL). Although most strains were sensitive to VCM, the MIC value of VCM for SCCmec type II strains was higher than that for SCCmec type IV strains. Only 4 (2.3%) of 171 SCCmec type IV strains were Panton-Valentine leukocidin (lukS/F-PV)-positive. Thus, this result indicates a unique feature of SCCmec type IV strains in Japan. The information in this study not only is important in terms of local public health but will also contribute to an understanding of epidemic clones of CA-MRSA.