Case Report: Gianotti-Crosti Syndrome Associated with Human Herpesvirus-6 Infection

We report a case of Gianotti-Crosti syndrome associated with human herpesvirus-6 (HHV-6) infection. An eight-month-old girl developed monomorphous papules on her cheeks, buttocks, and extremities after the subsidence of exanthema subitum. Viral antibody analysis confirmed primary HHV-6 infection. HHV-6 may be added to the list of causative agents of Gianotti-Crosti syndrome.     

Treatment of scleromyxoedema with hydroxychloroquine

Background: Scleromyxoedema is a rare disease of unknown aetiology that is characterized by deposition of mucin and sclerotic induration of the skin; it is associated with paraproteinaemia. Patients suffer from progressive disability due to immobilization and cosmetic disfigurement. Treatment of scleromyxoedema is a therapeutic challenge. The antimalarial hydroxychloroquine has a rapid and reliable effect in reticular erythematous mucinosis. Patients and methods: Four consecutive patients (two women, two men; median age: 50 years) with scleromyxoedema, three of them with IgG λ paraprotein, were treated with hydroxychloroquine. Treatment was initiated with 600 mg p. o. for 10 days, followed by 400 mg for at least 4 weeks, and 200 mg thereafter. Results: Complete remission of skin manifestations was achieved in one patient, whereas three patients achieved a partial remission of 61+, 5 and 25 months' duration. Notably, three patients felt increased mobility and reduced firmness of skin during the first week of treatment, which was reflected in a rapid reduction in dermal thickness. In one patient, dysphagia was reverted as evidenced by normalization of oesophageal clearance. Paraproteinaemia was not influenced at all. Side effects included one case of electroretinogram abnormalities after 19 months of therapy and one case of leucopenia after 3 months. Conclusion: Hydroxychloroquine is an effective form of therapy for scleromyxoedema, leading to rapid and prolonged alleviation of symptoms.     

Adult T-cell leukemia/lymphoma accompanying follicular mucinosis: a case report with review of the literature

Follicular mucinosis is recognized as one of the histopathological reaction patterns characterized by the accumulation of mucin within follicular epithelium. It is induced by various causes including inflammatory diseases, and more than half of the cases are associated with malignant lymphoma, mainly mycosis fungoides. Herein, we describe the third documented case of adult T-cell leukemia/lymphoma (ATLL) accompanying follicular mucinosis. A 72-year-old Japanese male presented with persistent erythema in his arm and neck. Laboratory tests demonstrated positivity for human T-cell leukemia virus (HTLV)-1 antibodies. Histopathological study of the biopsy specimen from the neck revealed superficial perivascular, nodular, and intrafollicular lymphocytic infiltrations. These lymphocytes were small- to medium-sized and had convoluted nuclei. Mucoid material deposition was observed within the hair follicles, and it was digested by hyaluronidase. Immunohistochemically, these lymphocytes were positive for CD3, CD4, CD25, and Foxp3. Accordingly, an ultimate diagnosis of ATLL accompanying follicular mucinosis was made. The skin is the most common extralymphatic site of involvement of ATLL. The present case clearly demonstrated that albeit extremely rare, ATLL can cause follicular mucinosis. Therefore, ATLL should be included in the differential diagnostic consideration of follicular mucinosis.     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

肢端持续性丘疹性黏蛋白沉积症

报告1例肢端持续性丘疹性黏蛋白沉积症。患者男,62岁。双前臂、双手背丘疹10年。皮损组织病理检查:真皮浅中层黏蛋白沉积,未见成纤维细胞增生。黏蛋白阿新蓝染色阳性。     

Reliability and validity of dermatology life quality index： assess- ment of quality of life in human immunodeficiency virus/acquired immunodeficiency syndrome patients with pruritic papular erup- tion

OBJECTIVE： To test the reliability and validity of dermatology life quality index（DLQI） in simplified Chinese language by assessing the quality of life（QoL） in HIV（human immunodeficiency virus）/AIDS（Acquired immunodeficiency syndrome） patients with pruritic papular eruption（PPE）. METHODS： A study of simplified-Chinese-versiond from10 questions with the principal component analysis and the contribution rate of the 3 common factors was 59.95%. CONCLUSION： The simplified-Chinese-version DIQL is an acceptable and valid scale for HIV/AIDS patients with PPE. It can be used to evaluate the QoL of HIV/AIDS patients with PPE in China.