We statistically examined 95 patients with extramammary Paget’s disease. These examinations were performed between 1962 and 2005 at the Division of Dermatology, National Cancer Center Hospital. In the results, we found that the survival rate of the patients with vessel invasion was 26.5%, but that without vessel invasion was 78.5%.The survival rate of the patients with one lymph node metastasis was 100%, but that with more than two lymph node metastases was only 13.5%. On the basis of these results, we adovocate the definition and future of TNM classification and staging of extramammary Paget’s disease.
Netherton syndrome is a rare, autosomally recessive genodermatosis characterized by the trias of congenital ichthyosis, hair abnormality and atopic diathesis. Recently, we experienced 4 pediatric cases whose ages ranged from 6 days to one year and a month. The patients developed generalized scaly erythroderma at or soon after birth, and were referred to our outpatient clinic, which is specific for keratinization disorders. Because all the patients presented generalized diffuse erythema and desquamation at birth, they were initially diagnosed with congenital (non-bullous) ichthyosiform erythroderma. In these patients, the face and head was notably covered by thick seborrheic scales during the neonatal period. However, the erythema and desquamation subsequently resolved during early infancy, and scaly erythema became localized primarily to the seborrheic and intertriginous areas. Hair abnormality and increased serum IgE were not detectable in two of 4 patients at 3 months after birth ; however, these symptoms appeared later during a follow-up period. Skin biopsy samples showed premature hyper-shedding of the stratum corneum cells. In addition, increased trypsin-like hydrolytic activity of the stratum corneum was revealed in all of the patients. This method seemed to be useful for the initial screening of NS. DNA analysis of the SPINK5 gene was also done and revealed mutations in two of the 4 patients. Together with the clinical and laboratory findings, we diagnosed all the patients as suffering from Netherton syndrome (NS). In this report, we summarized the clinical courses and laboratory findings in these patients with NS, and discussed their dermatological treatments and lifestyle guidance (i.e., skin care).
We report a case of amyopathic dermatomyositis with progressive outer retinal necrosis during therapy for interstitial pneumonia. The patient was a 58-year-old female. She had edematous erythema on her forehead, V-neck area, waist and back with no myopathy and developed interstitial pneumonia. We administered steroid pulse therapy under the diagnosis of amyopathic dermatomyositis, but the interstitial pneumonia worsened. Three days after the start of cyclosporin administration, she developed herpes zoster on her left waist, which disappeared after acyclovir administration. After two months, she suddenly complained of a disorder of her eyes, which was diagnosed as progressive outer retinal necrosis. In spite of the administration of gancyclovir and foscarnet sodium hydrate, it worsened quickly, and she finally lost her sight.
From 1962 through 2005, we have had new 95 patients of extramammary paget’s disease in the National Cancer Center Hospital. Among them, we examined the average survival rate of 11 patients that underwent chemotherapy. There were six kinds of chemotherapy employed, and the most often used chemotherapy was FECOM treatment. The average survival period was 15.4 months. Furthermore, for the patients that had metastasis to more than two lymph nodes, we compared the period until the patients had remote metastasis between the groups of patients that underwent chemotherapy and those with no chemotherapy. The former group showed an extension for the period. However, the chemotherapy that we used was not uniform, and we should standardize the chemotherapy for paget’s disease. As the result of this examination, we found that about half of the patients with remote unetastasis also developed hydronephrosis, and we infered how it occurred by using the preceeding picture for the analysis of the transition course in these cases.