角膜下脓疱性皮肤病(Sneddon-Wilkinson病):一例罕见病例

Rina Gustia, Yosse Rizal, Dwi Sepfourteen
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摘要

摘要角膜下脓疱性皮肤病(SPD)是一种罕见的慢性复发性脓疱性皮肤病。SPD与急性泛发性脓疱病(AGEP)和广泛性脓疱性银屑病(GPP)仅从临床表现上难以区分。我们报告一位48岁女性患有角膜下脓疱性皮肤病。她抱怨说,从4天前开始,她的脖子、胸部、腹部、手臂、腹股沟和腿部都有小水泡,红色的斑块上有烧灼感,并蔓延到脖子、胸部、腹部、手臂、腹股沟和腿部。患者既往无皮肤病变史。体格检查未发现其他病理,皮肤呈环状红斑斑块,脓疱形成,易合并形成脱屑斑块,在胸部、腹部、背部、手臂、臀部和腿部出现糜烂。组织病理学检查显示角膜下有脓疱。角膜下脓疱充满中性粒细胞,真皮有轻度中性粒细胞浸润。这一特点揭示给SPD。临床上SPD与AGEP和GPP可能难以区分。经组织病理学检查可鉴别此病,患者给予强的松40 mg、达泊森100mg治疗,4周后病情好转。
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Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease): A Rare Case
Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent, pustular eruption. It may be difficult to differentiate SPD from Acute Generalized Exanthematous Pustulosis (AGEP) and Generalized Pustular Psoriasis (GPP)just from clinical appeareance. A 48 years old woman with subcorneal pustular dermatosis was reported. She was complained there were tiny blisters containing pus on the reddish patches that felt burning and became widespread to neck, chest, abdomen, arms, groin and legs since 4 days ago. Patient had no history of skin lesion before. From physical examination revealed no other pathologies and on the skin showed erythematous patches with annular lesion, pustules formation that tended to coalesce to form desquamative plaques, erosions, on chest, abdomen, back, arm, buttock and leg. Based on histopathology showed there are subcorneal pustules. The subcorneal pustule is filled with neutrophils and on the dermis, there are mild infiltration of neutrophil cells. This feature revealed to SPD.Clinically it may be difficult to differentiate SPD from AGEP and GPP. Histopathology examination can distinguished these disease,patient was treated with prednisone 40 mg and dapson 100 mg and had improvement after 4 weeks.
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