HLA markers in familial Lichen Sclerosus

Background: Lichen sclerosus (LS) has been identified with increased frequency in families,often associated with HLA markers, mainly DQ7. A genetic co‐etiology seems likely in this setting. Moreover, there is an association of LS with autoimmune disorders, such as the presence of anti‐thyroid peroxidase autoantibodies (anti‐TPO), a hallmark of autoimmune thyroid diseases. Patients and Methods: In 3 families affected by LS, we verified their HLA markers, and identified previously undiagnosed cases of LS and autoimmune disorders. 30 individuals were examined with history, skin biopsy, HLA class I and II typing by PCR‐SSP, and measurement of anti‐TPO, free thyroxine and thyroidstimulating hormones (TSH) levels. Results: There were 8 cases of LS, 50 % of them anti‐TPO+. Autoimmune disorders were found in 40 % (total) and in 87.5 % of those affected. Most common HLA markers were B*15, B*57, CW*03, CW*07, CW*18, DRB1*04, DRB1*07, DRB4*. The three latter have been previously associated with LS. Conclusion: New cases of LS and autoimmune disorders can be detected in first degree relatives of patients with LS. The presence of anti‐TPO antibodies strongly suggests autoimmune thyroiditis. There is intra‐familial association between the haplotype HLA‐B*15 ‐DRB1*04 ‐DRB4* and anti‐TPO,emphasizing their link with thyroiditis. New familial approaches might help to make clear the pathogenesis of LS and its association with autoimmune diseases.     

扁平苔藓和丙型肝炎病毒

扁平苔藓是一种原因不明的炎症性皮肤病,其患病率为0.5%～2%.目前认为,扁平苔藓可能是一种自身免疫性疾病.丙型肝炎病毒被证明其伴随着一系列肝外表现,包括扁平苔藓,为此,综述扁平苔藓和丙型肝炎病毒的关系,以及病毒感染如何导致扁平苔藓的发生,从而为扁平苔藓合并丙型肝炎病毒感染的临床治疗及早期诊断提供一种思路.     

免疫荧光技术在口腔扁平苔藓和慢性盘状红斑狼疮诊断中的价值

采用免疫荧光技术对口腔扁平苔藓（ＯＬＰ）和慢性盘状红斑狼疮（ＤＬＥ）的免疫病理学进行了研究，结果表明，ＩｇＧ和纤维蛋白原荧光抗体在上皮基底膜区沉积的阳性率两病之间有显著性差异；而胶样小体中出现的阳性率无显著性差异；两病患者的血清抗核抗体阳性率之间亦无显著性差异。     

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.     

Two cases of lichen planus pigmentosus presenting with a linear pattern

We report two cases of lichen planus pigmentosus (LPP) that developed in a unilateral linear pattern. The patients presented with unilateral linear brown macules on the extremities. Skin biopsy showed orthokeratosis, basal hydropic degeneration with scarce lymphohistiocytic infiltrates, and numerous melanophages in both patients. These patients, to the best of our knowledge, are the first cases of LPP presenting with a linear pattern. LPP should be considered in the differential diagnosis of linear hyperpigmented skin lesions.     

Orale pr?kanzer?se Konditionen—eine übersicht

Zusammenfassung Präkanzeröse Konditionen (Zustände) umfassen Erkrankungen, die mit einem signifikant erhöhten Krebsrisiko verbunden sind. Dazu gehören die sideropenische Dysphagie, der orale Lichen planus (OLP), die orale submuköse Fibrose (OSF), die Syphilis, der diskoide Lupus erythematodes (DLE), das Xeroderma pigmentosum sowie die Epidermolysis bullosa. Die Zahl publizierter Fälle von oralen oder perioralen Transformationen zu Karzinomen ist für einige dieser Erkrankungen klein. Auch sind Zungenkarzinome oder Karzinome der Mundhöhle bei Spätsyphilis eher von historischem Interesse, obwohl in den 90er Jahren immer noch auf eine mögliche Assoziation einer positiven Syphilisserologie und dem Auftreten eines Zungenkarzinoms hingewiesen wurde. Der OLP ist eine nach wie vor kontrovers diskutierte präkanzeröse Kondition. Transformationsraten werden zwischen 0 und 5,6% angegeben. Neuere Untersuchungen, v. a. prospektive Studien, weisen darauf hin, dass möglicherweise die orale lichenoide Reaktion (OLR) Ausgangszustand einer Transformation ist und nicht der klassische OLP. Die Differenzierung zwischen OLP und OLR ist sowohl vom klinischen als auch vom histopathologischen Aspekt nach wie vor schwierig. Zweitwichtigste präkanzeröse Kondition ist die OSF, die durch das Kauen von Betel in Süd- und Südostasien bedingt wird. Mit 7,6% liegt für die OSF die höchste Transformationsrate aller präkanzerösen Konditionen vor.

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外阴硬化性苔癣与T淋巴细胞免疫的关系

目的 :探讨外阴硬化性苔癣与免疫功能的关系。方法 :采集患者静脉血 ,检测血清中CD3+ 、CD4 + 、CD8+ 、CD16 + T细胞水平 ;对照组采用正常献血妇女静脉血。结果 :患者组CD4 + T/CD8+ T细胞比值明显低于正常对照组 ,且差异有显著性。结论 :硬化性苔癣的患者体内存在免疫应答过度 ,且造成机体自身组织的损伤。     

Lichen Striatus Occurring after Allogenic Peripheral Blood Stem Cell Transplantation in an Adult with Aplastic Anemia

Lichens striatus (LS) is an acquired, self-limiting inflammatory dermatosis that follows the lines of Blaschko. The etiology of the eruption is unknown, but several theories have been proposed with focus on environmental factors, viral infection, cutaneous injury, hypersensitivity, and genetic predisposition. We describe a 19-year-old woman who developed a unilateral linear eruption 17 months after allogenic peripheral blood stem cell transplantation. Histopathology revealed features, which were consistent with LS. To the best of our knowledge, our patient is the first case describing the appearance of LS occurring after allogenic stem cell transplantation. We speculate that this condition represents an unusual form of localized, chronic graft-versus-host disease.     

NB-UVB及羟氯喹和糖皮质激素三联疗法治疗泛发性扁平苔藓1例

患者男,57岁。口腔溃烂、手足及躯干起疹1月余。背部皮损组织病理可见胶样小体,诊断:泛发性扁平苔藓。予NB-UVB联合糖皮质激素口服及羟氯喹治疗,8周后皮疹消退。     

Case for diagnosis

Idiopathic Atrophoderma of Pasini and Pierini (IAPP) is a rare, exclusively cutaneous disease. It is more frequent in females, with incidence peak in the second and third decades of life. The etiopathogenesis remains unknown. IAPP most commonly affects the back, abdomen and proximal regions of the limbs. Lesions may be rounded, oval or circular; single or multiple. The evolution is variable and the course is initially progressive. Collagen changes such as atrophy, thinning, condensation and sclerosis may be observed in the papillary dermis. This paper describes a case of Idiopathic Atrophoderma of Pasini and Pierini with histopathologic findings.