In 1935, Dr. Masao Ota, a Professor of Dermatology at Tohoku Imperial University, was asked to make a film. He himself wrote a scenario, which he asked Associate Professor Kanehiko Kitamura at Kumamoto Medical School to correct. The movie has never been completed. The original purpose of this film, “Spiral Devil,” was to educate the masses about syphilis. The script includes a comprehensive explanation of the history of syphilis, its symptoms, and its treatments. The story development is based upon the syphilis of a young architect and his wife. From the viewpoint of the history of dermatology, this is not only important but interesting.
The aim of the present study was to characterize the clinical features and prognosis of alopecia areata (AA) in pediatric patients. Subjects were 61 children with AA (30 males and 31 females) with 325 adult AA patients included as a comparison. On initial examination, 38 children had hair loss and 29 patients had atopic diathesis. The number of patients according to the pattern of hair loss was as follows: 4 mono, 11 oligo, 21 poly, 10 diffuse, 5 totalis, 7 universalis and 4 ophiasis. A total of 31 patients were treated with cryotherapy, 25 with topical SADBE, 11 with local injection with triamcinolone acetonide, and 9 with PUVA therapy. A total of 19 AA pediatric patients achieved complete hair regrowth. The convalescence rate in pediatric AA tended to be better than the convalescence rate in adult AA. Cryotherapy, topical SADBE and local injection with triamcinolone acetonide were effective in pediatric AA patients. The prevalence of atopic diathesis in children with AA may be higher than in adults with AA. We speculate that atopic diathesis may influence the prognosis of AA in children. Pediatric patients with universalis and diffuse AA did not reach convalescence; children with severe AA were more resistant to treatment than adult AA.
A 42-year-old man was diagnosed with multicentric Castleman’s disease at the age of 32 years. Laboratory data indicated polyclonal hyper-γglobulinemia, anemia, and elevated levels of C-reactive protein, erythrocyte sedimentation rate, and IL-6. A skin biopsy revealed the formation of many lymphoid follicles and lymphoplasmacytic infiltration cells in the dermis. No gene rearrangement was detected from the skin biopsy using both Southern blotting and PCR analysis techniques. A chest CT revealed a bilateral, peripheral, finely granular appearance, bilateral lymphadenopathy in the axilla and mediastinum, and paratracheal lesions. The patient was diagnosed with multicentric Castleman’s disease and treated with predonisolone and cyclophosphamide, but the clinical symptoms gradually progressed. Tocilizumab, an anti IL-6 receptor antibody, was administered every 2 weeks when the patient was 38 years old, and his clinical condition and laboratory data improved. Two years and 8 months later, the patient was found to have cervical lymphadenopathy, and, six months after that, a lymph node biopsy led to a diagnosis of mixed cellularity Hodgkin lymphoma. The administration of tocilizumab was suspended, and chemotherapy was given, resulting in complete remission. It has been reported that there is a significant incidence of malignant lymphoma in Castleman’s disease. Conversely, tocilizumab is apparently effective for Castleman’s disease when the patient presents with systemic symptoms. The relationship between tocilizumab and malignant lymphoma remains obscure. We examined the problem of administration of tocilizumab in Castleman’s disease.
Fabry disease is an X-linked disorder caused by a deficiency of lysosomal α-galactosidase, resulting in progressive multisystemic accumulation of glycosphingolipids, mainly globotriaosilceramide. The initial symptoms are mainly skin manifestations such as hypohidrosis, angiokeratoma, and acroparenthesias. Subsequent kidney failure, heart disease, and strokes lead to early death, typically at the age of 50 years. The prevalence of Fabry disease was estimated to be 1:500,000 births. However, recent neonatal mass screenings have shown the prevalence to be 1:1,250 to 1:3,100, which means Fabry disease is a relatively common disorder. Until the 2000s, treatment of Fabry disease targeted the symptomatic effects. Since Enzyme Replacement Therapy (ERT) became available in 2001, Fabry disease can be treated more fundamentally. Since 2001, we have treated 3 patients with Fabry disease using ERT. In two cases, we were able to begin the ERT treatment at the early stage of the disease, and no severe symptoms including heart failure, storokes and kidney failure have occurred to date. In the one case with proteinuria, even with ERT, the renal function gradually declined, resulting in dialysis therapy. Early initiation of ERT is important to slow disease progression. As the initial manifestations are mainly dermatological in Fabry disease, the role of dermatologists is important in early diagnosis.