Case 1: An infant boy, whose mother had been treated for hyperthyroidism with thiamazole until the 24th week of pregnancy, was born with a skin defect in the parietal region. The lesion spontaneously healed with reepithelization and an alopecic scar. Case 2: An infant boy, whose mother had been treated with thiamazole throughout her pregnancy, presented with ulcers surrounded by alopecia in the parietal region, together with fistulas in the face. We diagnosed these two cases as having aplasia cutis congenita. Previously, similarly congenital anomalies have been reported when fetuses were exposed in utero to thiamazole. Although it has not been demonstrated that thiamazole is a direct teratogen, the authors stress that hyperthyroidism patients who might get pregnant should refrain from taking thiamazole.
We present a case of Cowden disease with triple extramammary Paget’s disease. An 81-year-old man with red-colored plaques on the bisaxillary region and scrotum was referred to our clinic. Histological examination showed Paget’s cells in the epidermis. We diagnosed triple extramammary Paget’s disease. The patient also had multiple papules on the buccal mucosa and multiple keratotic papules on both hands. Gastrofiberoscopy and colonoscopy revealed multiple gastrointestinal polyps. Computed tomography images revealed a thyroid tumor. This patient was positive for a mutation in the PTEN gene, which resulted in Cowden disease. This syndrome is a systemic disorder involving hamartomatous overgrowth of tissues of all three embryonic origins and is associated with increased risks for thyroid, breast, and possibly other cancers. To our knowledge, this is a first case report of Cowden disease with extramammary Paget’s disease.
We report two cases of anaphylaxis due to ingestion of mite-contaminated Okonomi-yaki (Japanese pancake) mix. Case 1: A 50-year-old female developed anaphylaxis after eating Okonomi-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE against Dermatophagoides farinae (Der. f.), but did not demonstrate any wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to Der. f. antigen (Torii) but not to previously unopened Okonomi-yaki mix or wheat flour that had been stored in a freezer. Case 2: A 28-year-old female developed anaphylaxis after eating Monja-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE antibodies against Der. f. but no wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to the responsible Okonomi-yaki mix and Der. f. antigen (Torii), but not to wheat flour that had been stored in a freezer. The responsible Okonomi-yaki mix contained 50 heads/g of Der. f., and a high level (64.1 μg/g) of Der f1 antigen was detected by specific ELISA. Immunoblotting revealed a specific 25 kDa band in patient sera against the responsible Okonomi-yaki mix, which was absorbed by Der. f. crude antigen.
Generally, the symptoms of Behçet’s disease (BD) start with oral aphthous ulceration, and BD characteristically develops into a systemic disorder including the muco-cutaneous, ocular, gastro-intestinal, vascular and neurologic organs. Although it is not so difficult to diagnose clinically typical cases, cases without aphthous ulceration can be more problematic. The diagnostic guidelines provided by the Japanese Research Group for BD organized by the Ministry of Health, Labor and Welfare suggests using a prick test with oral streptococcal antigens as a diagnostic method, because BD patients are generally hypersensitive to streptococci. However, it may be difficult to prepare standardized bacterial antigens for the diagnosis of BD. Therefore, we propose a much simpler diagnostic method, because the so-called “pathergy test” does not seem to be highly specific in BD. It is well known that the saliva of BD patients contains many oral streptococci, including uncommon serotypes of Streptococcus (S.) sanguinis and/or S. oralis. We performed prick tests with self-saliva and saline as controls on the forearm skin of 7 BD patients, 3 recurrent aphthosis (RA) patients, a herpetic oral ulcer patient, 2 non-BD erythema nodosum patients, and 5 healthy controls. Six of the 7 BD patients and one RA patient showed erythematous reactions (more than 10 mm in diameter) with a small pustule 48 hours after pricks with self-saliva. This self-saliva prick test seemed to have the potential to be as helpful in diagnosing BD as the one using streptococcal antigens.