Nielsen P G
Department of Dermatology, Göteborg University, Sweden.
Acta Derm Venereol Suppl (Stockh). 1994;188:1-60.
Clinical reports of hereditary palmoplantar keratoderma are generally based on a limited number of patients. In 1967 the prevalence in the northernmost county of Sweden (Norrbotten) was shown to be 0.55%. In 1982 it was possible to trace half of the original propositi from that study. Among these families, a severe clinical form with a presumed recessive inheritance could be distinguished. The clinical pictures in relatives of the original propositi were described, and other diseases were listed together with those in patients from previously performed studies. The frequency of dermatophytosis was 36.2%, which was equal to a prevalence of 37.6%. T. mentagrophytes occurred significantly more often and immunological factors, such as increased presence of blood group A, specific dermatophyte IgG antibodies, precipitating antibodies and an immunological in vitro reaction to keratin, supported differences in the distribution of dermatophytes. However, the amount of keratin was considered the most important factor for the affinity of dermatophytes to the palms and soles. A vesicular eruption along the hyperkeratotic border and a mononuclear cell infiltrate were often reported. Such reactions were interpreted as immunological reactions to dermatophytosis. Scaling and fissuring were considered clinical signs of dermatophyte infections and not a part of the originally reported clinical picture. Results of the histopathological study corresponded to previously reported descriptions of the Unna-Thost variety. However, it has recently been reported that the histopathological picture of this variety was based on histopathological features of epidermolytic palmoplantar keratoderma. The existence on the Continent of the Unna-Thost variety was therefore questioned. Histopathological features of epidermolytic palmoplantar keratoderma were not found in the County of Norrbotten and the designation "Diffuse HPPK type Norrbotten" has therefore been proposed. The histopathological picture of the presumed recessive variety did not differ from that of the dominant variety but ultrastructural characteristics differentiated it from Mal de Meleda and the dominant variety. It was therefore concluded that a new variety with a presumed recessive inheritance was found.
遗传性掌跖角化病的临床报告通常基于有限数量的患者。1967年，瑞典最北部的诺尔伯特县的患病率显示为0.55%。1982年，有可能追踪到该研究中一半的最初提出者。在这些家族中，可以区分出一种假定为隐性遗传的严重临床类型。描述了最初提出者亲属的临床症状，并列出了其他疾病以及先前研究中患者的疾病。皮肤癣菌病的发生率为36.2%，相当于患病率为37.6%。须癣毛癣菌出现的频率明显更高，免疫因素，如血型A的增加、特定皮肤癣菌IgG抗体、沉淀抗体以及对角蛋白的免疫体外反应，支持了皮肤癣菌分布的差异。然而，角蛋白的量被认为是皮肤癣菌对掌跖亲和力的最重要因素。经常报告沿角化过度边缘的水疱性皮疹和单核细胞浸润。这种反应被解释为对皮肤癣菌病的免疫反应。脱屑和皲裂被认为是皮肤癣菌感染的临床症状，而不是最初报告的临床症状的一部分。组织病理学研究结果与先前报道的尤纳 - 托斯特型描述相符。然而，最近有报道称，该类型的组织病理学图像是基于表皮松解性掌跖角化病的组织病理学特征。因此，尤纳 - 托斯特型在欧洲大陆的存在受到质疑。在诺尔伯特县未发现表皮松解性掌跖角化病的组织病理学特征，因此提出了“诺尔伯特弥漫性HPPK型”的名称。假定的隐性类型的组织病理学图像与显性类型没有差异，但超微结构特征将其与梅勒达病和显性类型区分开来。因此得出结论，发现了一种假定为隐性遗传的新品种。
