Edwards Alan Martin, Capková Stepánka
The David Hide Asthma and Allergy Research Centre, St Mary's Hospital, Newport, Isle Of Wight, UK.
BMJ Case Rep. 2011 Jun 29;2011:bcr0220113910. doi: 10.1136/bcr.02.2011.3910.
Diffuse cutaneous mastocytosis (DCM) is a rare, severe, variant of cutaneous mastocytosis. The authors report the case of a male infant who developed maculae and maculopapulae on his legs and abdomen when aged 3.5 months, which spread to all body surfaces within weeks. Diagnosis of DCM was made at the age of 6 months when he had developed extensive bullous eruptions, generalised pruritus, flushing and abdominal pain. Treatment was started with oral dimethindine maleate. At the age of 18 months, oral sodium cromoglicate (SCG) was introduced. At the age of 23 months, additional treatment was started with a cutaneous emulsion containing 4% SCG. Continued treatment with oral dimethindine maleate, oral SCG with the dose maintained at 25 mg/kg/day, and SCG 4% cutaneous emulsion applied two to four times daily has resulted in a steady improvement of symptoms and skin appearance.
弥漫性皮肤肥大细胞增多症(DCM)是皮肤肥大细胞增多症的一种罕见、严重的变异型。作者报告了一例男婴病例,该男婴在3.5个月大时腿部和腹部出现斑疹和斑丘疹,数周内蔓延至全身。6个月大时,当他出现广泛的大疱性皮疹、全身性瘙痒、潮红和腹痛时,被诊断为DCM。治疗开始时使用口服马来酸氯苯那敏。18个月大时,开始使用口服色甘酸钠(SCG)。23个月大时,开始额外使用含4%SCG的皮肤乳剂进行治疗。持续使用口服马来酸氯苯那敏、将口服SCG剂量维持在25mg/kg/天,并每天使用2至4次4%SCG皮肤乳剂进行治疗,使症状和皮肤外观得到了稳步改善。