Williams Grant M., Lincoln Mark
Keesler AFB
Brooke Army Medical Center
Acrokeratosis verruciformis of Hopf (or simply acrokeratosis verruciformis: AKV) is a rare genodermatosis characterized by keratotic lesions on the dorsum of the hands and feet. AKV most commonly develops during early childhood, but occasionally develops as late as the fifth decade. The natural history of the disease is a chronic course without spontaneous remission. It exhibits an autosomal dominant inheritance pattern and shows incomplete penetrance. Because of this, there may not always be a family history. There is no gender predilection. Hopf first described AKV in 1931. The probable etiology is a mutation in the ATP2A2 gene located on chromosome 12q24,, which is the same gene affected in Darier disease. The diseases are distinct but may coexist in the same patient. Clinically acrokeratosis verruciformis of Hopf appears as flat-topped keratotic papules and plaques on the dorsum of the hands and feet. Less frequently, the lesions may occur on the legs or arms. The disease spares sebaceous areas, like the frontal scalp, flexural surfaces, and oral mucosa. There are associated nail changes to include thickening of the nail plate, leukonychia, and longitudinal ridges. Histopathologically, the lesions will have papillomatosis, acanthosis, hyperkeratosis, and hypergranulosis. The lesions will not have parakeratosis.
霍普夫疣状肢端角化病（或简称为疣状肢端角化病：AKV）是一种罕见的遗传性皮肤病，其特征是手足背部出现角化性损害。AKV最常见于儿童早期发病，但偶尔也会在五十多岁时才发病。该病的自然病程为慢性，不会自发缓解。它呈现常染色体显性遗传模式，且表现为外显不全。因此，并非总是有家族病史。该病无性别倾向。霍普夫于1931年首次描述了AKV。可能的病因是位于12q24染色体上的ATP2A2基因突变，这与 Darier病中受影响的基因相同。这两种疾病不同，但可能在同一患者中共存。临床上，霍普夫疣状肢端角化病表现为手足背部的平顶角化性丘疹和斑块。较少见的情况下，损害可能出现在腿部或手臂。该病不累及皮脂腺丰富的区域，如额部头皮、屈侧表面和口腔黏膜。还会出现相关的指甲改变，包括甲板增厚、白甲和纵嵴。组织病理学上，损害会有乳头瘤样增生、棘层肥厚、角化过度和颗粒层增厚。损害不会有角化不全。
