Archive
Spring 2016, Vol. 24 No. 1
Hong Kong J. Dermatol. Venereol. (2016) 24, 29-32
Case Report
Circumscribed juvenile pityriasis rubra pilaris
局限性幼年型毛髮紅糠疹
OM Moreno-Arrones, B Perez-Garcia, A Pecharroman
Abstract
Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory dermatosis of unclear aetiology. Its most remarkable clinical findings are palmoplantar hyperkeratosis, orange-red scaling plaques and hyperkeratotic follicular papules. Its extension may vary from localised subtypes to generalised skin involvement with well-demarcated areas of spared skin ("islands of sparing") to erythroderma. Griffiths' classification is used to distinguish PRP in five clinical subtypes. Diagnosis is based on clinical and histopathological findings. The main differential diagnosis in a patient with circumscribed juvenile PRP (type IV) is psoriasis although keratosis pilaris, lichen spinulosus and follicular ichthyosis must also be ruled out. Treatment of pityriasis rubra pilaris remains a controversial topic due to the lack of large-scale clinical randomised trials and its unpredictable spontaneous resolution.
毛髮紅糠疹（PRP）是一種罕見及病因不明的丘疹鱗屑性炎性皮膚病。其最顯著的臨床表現是掌蹠角化過度、橙紅色的鱗片剝落狀斑塊和過度角化的毛囊性丘疹。它的影響範圍變化可以由局部亞型至泛發型伴隨著界限清晰而不受影響的正常皮膚部份（"倖免的島嶼"），甚至是全身的紅皮病。常用的格雷佛氏分類把毛髮紅糠疹分為五個臨床亞型，診斷是建基於臨床表現和組織病理學的發現。局限性幼年型毛髮紅糠疹（第四型）患者的首要鑑別診斷是銀屑病，其他要排除的皮膚病包括毛髮角化病、小棘苔蘚和濾泡型魚鱗癬。毛髮紅糠疹的治療仍有爭議之處，源於其缺乏大規模的臨床隨機對照試驗及本身難以預計的自我痊癒可能。
Keywords: Children, differential diagnosis, papulosquamous dermatosis, pityriasis rubra pilaris
關鍵詞: 兒童、鑑別診斷、丘疹鱗屑性皮膚病、毛髮紅糠疹
