Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Department of Pediatrics, Haematology and Oncology, Medical University of Gdańsk, Gdańsk, Poland.
Int J Dermatol. 2021 Oct;60(10):1253-1257. doi: 10.1111/ijd.15542. Epub 2021 Apr 27.
Mastocytosis is a heterogeneous group of myeloproliferative disorders characterized by accumulation of clonal mast cells in various tissues. The aim of this study was to determine the symptoms evolution and outcome after 10 years observation.
Fifty-five children with mastocytosis were included in the study group and monitored concerning mast cell mediator-related symptoms (MC MRSs) and clinical course of the disease for a period of ≥10 years.
Patients presented with a maculopapular cutaneous form of mastocytosis (MPCM) (n = 47) and diffuse cutaneous mastocytosis (DCM) (n = 8). The complete remission (CR) of skin lesions occurred in 10.3% of children after 10 years observation; no remission (NR) was observed in 17.9% children. The CR of skin specific MC MRS occurred in 69.2% children with MPCM and in 14.3% with DCM.
Most children with cutaneous mastocytosis (CM) eventually experience a major or partial regression of skin lesions, although complete regression before puberty is rare. The spontaneous remission of skin specific MC MRS is less frequent in children with DCM.
肥大细胞增多症是一组异质性的骨髓增殖性疾病，其特征是在各种组织中积累克隆性肥大细胞。本研究的目的是确定 10 年后观察到的症状演变和结果。
55 例肥大细胞增多症患儿纳入研究组，对其肥大细胞介质相关症状（MC MRSs）和疾病临床过程进行了≥10 年的监测。
患儿表现为斑丘疹性皮肤肥大细胞增多症（MPCM）（n=47）和弥漫性皮肤肥大细胞增多症（DCM）（n=8）。10 年后观察，10.3%的患儿皮肤病变完全缓解（CR）；17.9%的患儿无缓解（NR）。69.2%的 MPCM 患儿和 14.3%的 DCM 患儿皮肤特异性 MC MRS 缓解。
大多数皮肤肥大细胞增多症（CM）患儿的皮肤病变最终会出现明显或部分消退，尽管青春期前完全缓解很少见。DCM 患儿皮肤特异性 MC MRS 自发缓解较少见。
