Bulat Vedrana, Mihić Liborija Lugović, Situm Mirna, Buljan Marija, Blajić Iva, Pusić Jana
University Department of Dermatology and Venereology, Sestre milosrdnice University Hospital, Zagreb, Croatia.
Acta Clin Croat. 2009 Mar;48(1):59-64.
The term mastocytosis is referred to as an array of uncommon, usually sporadic, heterogeneous clinical illnesses that result from the hyperplasia of tissue mast cells. It comprises many different clinical manifestations varying from indolent cutaneous forms to systemic and malignant conditions. The characteristic presentation of mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or less commonly, diffuse cutaneous mastocytosis. Urticaria pigmentosa is the most common manifestation of cutaneous mastocytosis that manifests as a generalized eruption of round or oval erythematous macules, papules and plaques with variable amounts of brown pigment, usually on the trunk, but may also occur in all regions of the body including face and mucous membranes. Pruritus, dermographism and Darier's sign are additional features of these eruptions. Mastocytosis may also be manifested as mastocytoma, a rare, benign, pediatric tumor that results from hyperplasia of mast cells in papillary dermis in the first few weeks of life. The clinical course of mastocytosis is variable. The prognosis for the majority of pediatric patients with urticaria pigmentosa is extremely good, and over half of cases clear completely by adolescence, while those with aggressive systemic mastocytosis or mast cell leukemia show a progressive course, usually with a fatal outcome.
肥大细胞增多症这一术语指的是一系列不常见的、通常为散发性的、异质性的临床疾病，这些疾病由组织肥大细胞增生引起。它包括许多不同的临床表现，从惰性皮肤形式到全身性和恶性疾病。肥大细胞增多症的特征性表现包括皮肤表现：孤立性肥大细胞瘤、色素性荨麻疹，或较少见的弥漫性皮肤肥大细胞增多症。色素性荨麻疹是皮肤肥大细胞增多症最常见的表现，表现为全身性的圆形或椭圆形红斑、丘疹和斑块，伴有不同程度的褐色色素沉着，通常出现在躯干，但也可能发生在身体的所有部位，包括面部和黏膜。瘙痒、皮肤划痕症和 Darier 征是这些皮疹的其他特征。肥大细胞增多症也可能表现为肥大细胞瘤，这是一种罕见的良性儿科肿瘤，由出生后最初几周乳头真皮内肥大细胞增生引起。肥大细胞增多症的临床病程各不相同。大多数患有色素性荨麻疹的儿科患者预后非常好，超过一半的病例在青春期前完全消退，而那些患有侵袭性全身性肥大细胞增多症或肥大细胞白血病的患者则呈进行性病程，通常预后不良。
