Morgado-Carrasco Daniel, Feola Hernán, Fustà-Novell Xavier
Department of Dermatology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona.
Dermatol Online J. 2020 Apr 15;26(4):13030/qt8j7208hm.
Porokeratosis is a heterogeneous group of dermatoses with alterations of keratinization. Histologically, they are characterized by the presence of cornoid lamellae. Eruptive pruritic papular porokeratosis (EPPP) or the inflammatory form of disseminated superficial porokeratosis (or eruptive disseminated porokeratosis) is an infrequent variant, characterized by pruritic erythematous papules or annular lesions. We present a 72-year-old woman with EPPP, exhibited by pruritic lesions on the extremities and back, and review the literature concerning this condition. We found 32 cases of EPPP or inflammatory disseminated superficial porokeratosis (including the current case) reported in the literature, with a median age of 66 years (range, 13-84); 59.3% were men. Eruptive pruritic papular porokeratosis was associated with various neoplasms in 31.2% of cases. Six patients had an associated viral infection. Response to treatment was poor in most cases. Eruptive pruritic papular porokeratosis resolved spontaneously in 75% of cases. Median time to resolution was 6 months (range, 1-24). Eruptive pruritic papular porokeratosis (or inflammatory disseminated superficial porokeratosis/eruptive disseminated porokeratosis) is an infrequent variant of porokeratosis characterized by intense pruritus and spontaneous resolution in most individuals. Eruptive pruritic papular porokeratosis can be associated with neoplasms and screening for malignancies is recommended if clinically indicated.
汗孔角化症是一组具有角化异常的异质性皮肤病。在组织学上，它们的特征是存在鸡眼样板层。发疹性瘙痒性丘疹性汗孔角化症（EPPP）或播散性浅表性汗孔角化症的炎症形式（或发疹性播散性汗孔角化症）是一种罕见的变异型，其特征为瘙痒性红斑丘疹或环状损害。我们报告了一名72岁患有EPPP的女性，其表现为四肢和背部的瘙痒性损害，并复习了关于此病的文献。我们发现文献中报道了32例EPPP或炎症性播散性浅表性汗孔角化症（包括本例），中位年龄为66岁（范围13 - 84岁）；59.3%为男性。31.2%的发疹性瘙痒性丘疹性汗孔角化症病例与各种肿瘤相关。6例患者伴有病毒感染。大多数病例对治疗反应不佳。75%的病例中发疹性瘙痒性丘疹性汗孔角化症可自发消退。消退的中位时间为6个月（范围1 - 24个月）。发疹性瘙痒性丘疹性汗孔角化症（或炎症性播散性浅表性汗孔角化症/发疹性播散性汗孔角化症）是汗孔角化症的一种罕见变异型，其特征为大多数个体有剧烈瘙痒且可自发消退。发疹性瘙痒性丘疹性汗孔角化症可与肿瘤相关，如果有临床指征，建议筛查恶性肿瘤。
