Gamborg Nielsen P, Hofer P A, Lagerholm B
Department of Dermatology, Varberg Hospital, Sweden.
Dermatology. 1994;188(3):188-93. doi: 10.1159/000247136.
The frequency of autosomal dominant inherited palmoplantar keratoderma (HPPK) in the northernmost county of Sweden (Norrbotten) is 0.55%. Histopathological examination of 91 biopsies from patients with the dominant form of HPPK revealed no case of epidermolytic PPK. This finding is in contrast to the results of a re-examination of descendants of the original family published by Thost which showed the characteristic features of epidermolytic PPK, and re-evaluation of biopsies from other families has shown that it is the most frequent type. The existence of PPK type Unna-Thost in relation to epidermolytic PPK and to HPPK of the northernmost county of Sweden will be discussed. At the same time a revision of designation of this type is proposed. A dermo-epidermal mononuclear cell infiltrate belongs to the classical description of PPK Unna-Thost. It was shown that this cell infiltrate occurs significantly more often in patients with HPPK and dermatophytosis. Relapsing vesicular eruptions along the hyperkeratotic border are a clinical sign of the severity of dermatophyte infections. Such spongiotic vesicles together with a mononuclear cell infiltrate should be considered as eczematous reaction to dermatophytosis.
瑞典最北部的北博滕郡常染色体显性遗传性掌跖角化病（HPPK）的发病率为0.55%。对91例显性型HPPK患者的活检组织进行组织病理学检查，未发现表皮松解性掌跖角化病（PPK）病例。这一发现与托斯特发表的对原家族后代重新检查的结果形成对比，后者显示出表皮松解性PPK的特征性表现，并且对其他家族活检组织的重新评估表明它是最常见的类型。将讨论与表皮松解性PPK以及瑞典最北部郡的HPPK相关的昂纳 - 托斯特型PPK的存在情况。同时，提议对该类型的命名进行修订。真皮 - 表皮单核细胞浸润属于昂纳 - 托斯特型PPK的经典描述。结果显示，这种细胞浸润在HPPK患者和皮肤癣菌病患者中出现的频率明显更高。沿角化过度边缘反复出现的水疱性皮疹是皮肤癣菌感染严重程度的临床体征。这种海绵状水疱连同单核细胞浸润应被视为对皮肤癣菌病的湿疹样反应。
