Hiroko Gomi

Oesophageal involvement in pemphigus vulgaris

Oesophageal involvement of pemphigus vulgaris had been considered an exceptional event. However, our endoscopic study found oesophageal lesions in seven of eight (87.5%) patients with pemphigus vulgaris.

In vitro phototoxicity of new quinolones: production of active oxygen species and photosensitized lipid peroxidation

To elucidate photosensitization potentials of new quinolone antibacterial agents, production of active oxygen species and peroxidation of squalene after ultraviolet A exposure were investigated. Production of singlet oxygen and/or hydrogen peroxide was estimated by bleaching of p‐nitroso‐N, N‐dimethylaniline. Lomefloxacin showed the greatest ability to produce active oxygen species, and this ability was reduced by the addition of the singlet oxygen quencher sodium azide. Ciprofloxacin and fleroxacin also had strong activity. Photosensitized peroxidation of squalene was evaluated by measurement of thiobarbituric acid‐reactive substances. Lomefloxacin was the strongest sensitizer, followed by fleroxacin and ciprofloxacin. These results suggest that certain new quinolones are involved in phototoxicity via the mechanism of active oxygen species.

Autoerythrocyte Sensitization Syndrome with Thrombocytosis

A case of autoerythrocyte sensitization syndrome in a 59-year-old female is reported. She had several episodes of circular ecchymoses on her left thigh. Intradermal injection of autoerythrocytes reproduced a similar ecchymosis. Thrombocytosis was detected and treated with busulfan which induced a delayed improvement of the ecchymosis outbreaks over 2 years of follow-up.

Pemphigus erythematosus: Detection of Anti-Desmoglein-1 Antibodies by ELISA

Kawakami et al. [1] report on 2 patients with ichthyosis and mental retardation, who are diagnosed as having (incomplete) Sjögren-Larsson syndrome (SLS). We disagree with the authors. The SLS is a highly characteristic neurocutaneous disorder with congenital ichthyosis, spasticity, mental retardation and retinal glistening dots [2–4]. The ichthyosis is generalized, with the trunk, flexures and the dorsal aspects of hands and feet the most severely affected sites. The ichthyotic skin is colored yellowish-brown and gives rise to pruritus. Spasticity and mental retardation are severe, but not progressive, in most cases. SLS is caused by a deficiency of microsomal fatty aldehyde dehydrogenase (FALDH) that catalyzes the conversion of mediumand long-chain fatty aldehydes to their corresponding fatty acids [5]. The FALDH gene has been identified, and mutations have been found in this gene in SLS patients. Curative treatment strategies are not available. The ichthyosis in the patients described by Kawakami et al. [1] differs from the ichthyosis seen in SLS, as the authors state themselves. Mental retardation is the only additional feature in both patients; however, its severity is not mentioned in patient 1, while it is reported to worsen gradually in patient 2. Spasticity and retinal glistening dots are present in the vast majority of SLS patients but are not found in patients 1 and 2. Biochemically, FALDH activity in patient 2 is in the range of SLS heterozygotes. Patient 2 is reported to have the same level of FALDH activity, but no quantitative data are given. Contrary to the statement of the authors, partial enzyme deficiency has never been reported in SLS patients, especially not in their references 2, 19, 20 and 21 [6–9]. Moreover, it has been established that SLS heterozygotes, i.e. carriers of FALDH deficiency, are asymptomatic [9]. The clinical features in patients 1 and 2 do not resemble SLS, and their FALDH activity is above the upper limit of SLS homozygotes. Taken together, these data indicate that the diagnosis of (incomplete) SLS is highly unlikely both on clinical and biochemical grounds. A more plausible explanation for the biochemical findings in these patients is that one of the parents is an asymptomatic carrier of FALDH deficiency and that the same heterozygous state, not explaining the clinical features, has been demonstrated in both patients. Parental FALDH activities should be measured and reported additionally, to close the controversy. Furthermore, molecular analysis which can now be done routinely in various centers including our own, should be performed. Dietary therapy with medium-chain triglycerides has been demonstrated to be ineffective in SLS [10]. This fact, and not the patients’ development delay, should be the reason for not performing dietary therapy in SLS. In conclusion, we object to the diagnosis of (incomplete) SLS in the patients described by Kawakami et al. [1]. A diagnosis of SLS, including so-called atypical or incomplete SLS, has to be proven by demonstrating a FALDH activity in the range of SLS homozygotes. Dietary therapy with medium-chain triglycerides should not be performed in SLS patients.

Giant vascular eccrine spiradenoma mimicking calcifying epithelioma.

dence of metastasis at 9 months after the final operation. The deep lymph vessels of the posterior loin follow their corresponding blood vessels and end in the superior gluteal node near the intrapelvic part of the superior gluteal artery, adjacent to the superior border of the greater sciatic foramen. This is the first report to describe surgical anatomy of the superior gluteal node, which belongs to the deep nodes.

Pyridoxine-Induced Photosensitivity and Hypophosphatasia

We describe a case of photosensitivity due to pyridoxine hydrochloride (vitamin B6) in a heterozygote of hypophosphatasia. Photopatch tests using pyridoxine hydrochloride and pyridoxal 5′-phosphate, compounds referred to as vitamin B6, with ultraviolet light A irradiation were positive. Laboratory examination showed low serum alkaline phosphatase. Tissue-nonspecific alkaline phosphatase exon amplification from DNA of the patient’s lymphocytes detected deletion 1154–1156 hypophosphatasia mutation, indicating that this patient was diagnosed to be a heterozygote of hypophosphatasia. The seric pyridoxal 5′-phosphate level of this patient with hypophosphatasia was higher than in normals. Furthermore, after oral administration of vitamin B6 this level increased greatly and long-lastingly, and this might be related to the low level of alkaline phosphatase in this patient. Photosensitivity in this patient may have been caused by abnormal metabolism of vitamin B6 under the hypophosphatic condition.

A Case of Esophagitis Associated with Stevens-Johnson's Syndrome

Abstract: Stevens‐Johnsons syndrome (SJS) is a severe type of erythema multiforme with involvement of the mucous surfaces of the mouth, nose and conjunctiva. Although various complications have been described, reports of digestive tract lesions associated with SJS are rare. A 21‐year‐old woman developed erythematous lesions on the limbs three days after receiving antibiotic and anti‐inflammatory agent treatment for common cold‐like symptoms. On initial consultation at our hospital, endoscopy was performed to investigate the cause of a disagreeable sensation in the esophagus and mucosal exfoliation of a white membrane‐like substance was recognized as involving the entire esophagus. Sporadic white mucosal protrusions were also observed, suggesting exfoliative esophagitis. No abnormal findings were noted in the stomach or duodenum. After admission, the skin lesions rapidly expanded to involve the entire body including the mucous membranes of the lips, oral cavity, conjunctiva and vulva. Nikolskys sign was positive. A diagnosis of SJS was made and systemic corticosteroid administration was initiated. Both the esophagitis and the skin lesions improved within 2 months of starting treatment. Esophageal lesions constitute a relatively rare manifestation of SJS, and only 10 cases have been reported in the international literature.