van der Waa J D, Vredenborg A D
MohsA Huidcentrum, Eindhoven.
Contact: J.D. van der Waa (
Ned Tijdschr Geneeskd. 2020 Aug 17;164:D5098.
Polymorphic maculopapular cutaneous mastocytosis (PMCM) is caused by proliferation of mast cells in the skin. It is commonly seen in infants. Although mastocytosis usually only affects the skin, systemic mastocytosis should be ruled out in cases with extensive presentation. PMCM resolves spontaneously before puberty in most cases. A 6-month-old male presented with asymptomatic cutaneous erythematous patches all over the skin. Darier's sign was positive. A diagnosis of polymorphic maculopapular cutaneous mastocytosis was made. Because of the extensive skin lesions, the patient was referred to a paediatrician. Tryptase levels were elevated but blood tests did not suggest systemic involvement. The parents were given an EpiPen Jr because of increased risk of anaphylactic shock.
多形性斑丘疹性皮肤肥大细胞增多症（PMCM）是由皮肤中肥大细胞增殖引起的。常见于婴儿。虽然肥大细胞增多症通常仅累及皮肤，但对于表现广泛的病例应排除系统性肥大细胞增多症。大多数情况下，PMCM在青春期前会自发消退。一名6个月大的男性全身出现无症状性皮肤红斑。Darier征阳性。诊断为多形性斑丘疹性皮肤肥大细胞增多症。由于皮肤病变广泛，该患者被转诊给儿科医生。类胰蛋白酶水平升高，但血液检查未提示有全身受累。由于过敏性休克风险增加，给患儿父母配备了儿童肾上腺素自动注射器。
