Wananukul S, Pongprasit P
Department of Pediatrics, Chulalongkorn University, Bangkok, Thailand.
Int J Dermatol. 1999 Jan;38(1):29-35. doi: 10.1046/j.1365-4362.1999.00618.x.
Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature.
All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes
The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good.
Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.
报告了5例天疱疮患儿:3例寻常型天疱疮,1例增殖型天疱疮,1例落叶型天疱疮。文献中仅发表过1例青少年增殖型天疱疮。
3例寻常型天疱疮患者均接受口服皮质类固醇治疗;2例加用硫唑嘌呤以发挥激素节省作用。增殖型天疱疮患者临床表现类似寻常型天疱疮,但肛周病变愈合为肥厚性肉芽组织。他接受了口服皮质类固醇、硫唑嘌呤和皮损内注射皮质类固醇治疗。落叶型天疱疮患者表现为剥脱性皮炎,接受口服皮质类固醇治疗;后来加用甲氨蝶呤以节省激素。
对患者进行了1至4年的随访;儿童天疱疮预后良好。
需要长期随访以检测疾病复发及免疫抑制药物的副作用。