Legrain V, Taïeb A, Bioulac-Sage P, Maleville J
Unité de Dermatologie pédiatrique, Hôpital Pellegrin, Bordeaux.
Ann Dermatol Venereol. 1994;121(8):561-4.
Cutaneous mastocytosis usually includes objective skin signs such as pigmented maculopapulae or skin infiltration. We report an unusual case of cutaneous mastocytosis without systemic involvement in a 9-month old infant. Clinical expression was entirely functional (pruritus, urticaria) with no permanent lesions. There were 22 times more mastocytes found in the skin biopsy than in similar biopsies obtained in healthy control subjects of the same age, which corresponds to the counts found in cutaneous mastocytosis. Diffuse cutaneous mastocytosis with no permanent lesions is an exceptional form of mastocytosis (3 cases reported in the literature). The long-term outcome is unknown. This syndrome should be distinguished from idiopathic anaphylaxis by quantification of the dermal mastocytes.
皮肤肥大细胞增多症通常包括色素沉着性斑丘疹或皮肤浸润等客观皮肤体征。我们报告了一例9个月大婴儿的非系统性累及的罕见皮肤肥大细胞增多症病例。临床表现完全为功能性(瘙痒、荨麻疹),无永久性皮损。皮肤活检发现肥大细胞数量比同年龄健康对照受试者的类似活检多22倍,这与皮肤肥大细胞增多症中的计数相符。无永久性皮损的弥漫性皮肤肥大细胞增多症是肥大细胞增多症的一种罕见形式(文献报道3例)。长期预后未知。该综合征应通过真皮肥大细胞定量与特发性过敏反应相鉴别。