Sézary syndrome managed with histone deacetylase inhibitor followed by anti-CCR4 monoclonal antibody.

A 70-year-old man presented to our clinic with a 10-year history of recurrent pruritic erythema and plaques on his trunk and limbs. Based on the pathological findings and monoclonal rearrangement of the T-cell receptor (TCR)-Cß1 gene, mycosis fungoides (T2N0M0B0 stage IB) was diagnosed. Despite combination therapy including histone deacetylase inhibitor (vorinostat), the symptoms slowly evolved into Sézary syndrome (SS; T4N1M0B2) over 4 years, with dense infiltrates due to atypical lymphocytes expressing CCR4 developing in the entire dermis. Anti-CCR4 monoclonal antibody (mogamulizumab) treatment was started. After seven courses, the CCR4-positive atypical lymphocytes decreased in the dermis to levels below those seen at the outset of treatment. To our knowledge, there is no previous report of a case of SS managed with vorinostat followed by mogamulizumab demonstrating such a remarkable change in the pathological state following treatment.

A case of lichen planus pemphigoides with autoantibodies to the NC16a and C-terminal domains of BP180 and to desmoglein-1.

Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease that occurs in association with lichen planus (LP). This report describes a 59-year-old Japanese female patient with LPP. The patient first showed LP lesions on her hands, and subsequently developed bullae on her extremities and erosions of the oral mucosa. The patient's serum was positive for IgG autoantibodies against the BP180 NC16a domain, the BP180 C-terminal domain and desmoglein-1. However, a serum sampled one and a half years before the diagnosis of LPP was negative for autoantibodies against BP180 NC16a and BP180 C-terminal domains. These findings strongly suggest that the damage to the basal cells in the LP lesions exposed a sequestered antigen or formed neoantigens, leading to the production of pathogenic autoantibodies for LPP. Most of the previous cases of LPP have produced autoantibodies to the NC16a domain of BP180. This is the first case in which autoantibodies to the C-terminal domain of BP180 were detected. The oral mucosal symptoms in this case may have been caused by autoantibodies to the BP180 C-terminal domain.

H3(+) spectroscopy and the ionization rate of molecular hydrogen in the central few parsecs of the galaxy.

We report observations and analysis of infrared spectra of H3(+) and CO lines in the Galactic center, within a few parsecs of the central black hole, Sgr A*. We find a cosmic ray ionization rate typically an order of magnitude higher than outside the Galactic center. Notwithstanding, the elevated cosmic ray ionization rate is 4 orders of magnitude too short to match the proton energy spectrum, as inferred from the recent discovery of the TeV γ-ray source in the vicinity of Sgr A*.

The spin temperature of NH3 in Comet C/1999S4 (LINEAR).

A high-dispersion spectrum of Comet C/1999S4 (LINEAR) was obtained in the optical region with the high-dispersion spectrograph on the Subaru telescope when the comet was 0.863 astronomical units from the Sun before its disintegration. We obtained high signal-to-noise ratio emission lines of the cometary NH2 bands from which an ortho-to-para ratio (OPR) of 3.33 +/- 0.07 was derived on the basis of a fluorescence excitation model. Assuming that cometary NH2 mainly originates from ammonia through photodissociation, the derived OPR of NH2 molecules should reflect that of ammonia, which provides information on the environment of molecular formation or condensation and of the thermal history of cometary ices. Assuming that the OPR of ammonia in comets was unchanged in the nucleus, the derived spin temperature of ammonia (28 +/- 2 kelvin) suggests that a formation region of the cometary ammonia ice was between the orbit of Saturn and that of Uranus in the solar nebula.

Scoring of reflux symptoms associated with scleroderma and the usefulness of rabeprazole.

OBJECTIVE: The high frequency of gastroesophageal reflux disease (GERD) as a complication of scleroderma (systemic sclerosis, SSc) calls for treatment with powerful acid suppressants such as proton pump inhibitors (PPI). The present study used a GERD-specific questionnaire to assess the symptoms of GERD in SSc patients, and examine the effectiveness of rabeprazole (RPZ) for treating the symptoms of GERD. METHODS: The Frequency Scale for the Symptoms of GERD (FSSG), a medical questionnaire developed in Japan for evaluating GERD, and the Visual Analogue Scale (VAS) were used to evaluate GERD symptoms and the degree of pain, respectively, in 151 SSc subjects. These tools were also used to assess the effect of 8 weeks' treatment with the PPI RPZ (10 mg/day). RESULTS: Data on age and gender, and FSSG and VAS scores before treatment and after 4 and 8 weeks' RPZ treatment, were available for 84 subjects. The mean FSSG score was 13.9+/-9.7 before treatment, 8.3+/-8.1 after 4 weeks of treatment, and 7.0+/-7.0 after 8 weeks of treatment; the score reduction was significant (p<0.001) indicating the effectiveness of RPZ in improving subjective GERD symptoms. The VAS scores revealed a significant improvement in pain after both 4 and 8 weeks compared with baseline scores. Six subjects experienced adverse effects and five discontinued the analysis during the period. CONCLUSION: Administration of RPZ 10 mg/day is effective for the control of the symptoms of GERD associated with SSc. In addition to assessing the symptoms of GERD, the FSSG questionnaire can be used to evaluate the therapeutic effect of drugs.

Development of a health guidance support system for lifestyle improvement. Committee of Health Evaluation Support System Council of Japan AMHTS Institutions.

OBJECTIVE: To provide automated advice for lifestyle adjustment based on an assessment of the results of a questionnaire and medical examination or health checkup data. METHODS: A system was developed that gathers data based on questions regarding weight gain, exercise, smoking, sleep, eating habits, salt intake, animal fat intake, snacks, alcohol, and oral hygiene; body mass index, resting blood pressure, fasting blood sugar, total cholesterol, triglycerides, uric acid and liver function tests. Based on the relationships between the lifestyle data and the health checkup data, a health assessment sheet was generated for persons being allocated to a multiple-risk-factor syndrome group. RESULTS: Health assessment and useful advice for lifestyle improvement were automatically extracted with the system, toward the high risk group for lifestyle related diseases. The system is operational. CONCLUSIONS: We developed a health guidance support system for lifestyle improvement. In comparison with conventional, limited advice methods, we developed a practical system that defined the necessity for lifestyle improvement more clearly, and made giving advice easier.

Isolated mitral valve injury from non-penetrating cardiac trauma. Report of a case with successful repair.

Isolated mitral valve injury from non-penetrating cardiac trauma was low in incidence. A successfully operated case of a patient with acute mitral insufficiency from non-penetrating cardiac injury due to a traffic accident was reported and discussed. This isolated mitral valve lesion, of which the posterior leaflet was torn away from the annulus with intact chordae tendineae and papillary muscle, was very rare in the literature. And, particularly, the preoperative non-invasive findings such as echocardiogram and ACG were mostly useful and important in our case. This case was the fourth successful repaired case on blunt isolated mitral valve injury in the literature.

Immunogenetic study of three Japanese families with neonatal lupus erythematosus.

We encountered three Japanese families with neonatal lupus erythematosus. None of the three fathers showed any signs of collagen disease. The three mothers were found to suffer from Sjögren's syndrome; they all tested positive for anti-SSA and SSB antibodies and had lymphocyte infiltration into the small salivary gland. In two families, one child had neonatal lupus erythematosus while a sibling was normal; in the third family, both children had neonatal lupus erythematosus. Thus, a mother with positive anti-SSA and SSB antibodies can give birth to one infant with and one infant without or have two infants with neonatal lupus erythematosus. We conducted HLA typing of all 12 members of the three families in order to clarify the immunologic factors involved. We found no increased frequency of any HLA phenotype in the three mothers and their four children with neonatal lupus erythematosus; however, HLA-DR4 was present in three of the children with neonatal lupus erythematosus.

[Recurrent annular erythema--cutaneous manifestation of Sjögren syndrome with anti SS-A (Ro) and anti SS-B (La) antibodies].

Eight cases of Sjögren syndrome (SjS) with anti-SS-A and anti-SS-B antibodies are reported. They developed erythema annulare centrifugum-like annular erythema which mainly appeared on the face. Laboratory tests showed similar serologic changes. RA factor, speckled type antinuclear antibody, anti SS-A & SS-B antibodies (DID & ELISA) were observed but complements and anti-double stranded DNA antibodies were not detected. In spite of mild sicca symptoms, sialogram, lip biopsy, Shirmer test and Rose Bengal staining showed typical changes of SjS. It is considered that the recurrent annular erythema is a specific skin manifestation of SjS with anti SS-A/SS-B antibodies.

Basal cell epithelioma occurring in an epidermal nevus.

An unusual case is presented of a thirty-year-old woman in whom basal cell epithelioma developed from a preexisting localized epidermal nevus in the scalp. The epidermal nevus was not associated with any abnormal pilosebaceous complexes.