Velloso E D, Beitler B, Chamone D A
Disciplina de Hematologia e Hemoterapia, Hospital das Clínicas da Faculdade de Medicina, Universidade de São Paulo.
Rev Hosp Clin Fac Med Sao Paulo. 1991 Mar-Apr;46(2):94-8.
The case histories of two patients with benign systemic mastocytosis with skin involvement are presented. The first patient had urticaria pigmentosa diagnosed at the age of 2 months, and developed systemic disease within two years. The second presented urticaria pigmentosa at the age of 22 years while benign systemic mastocytosis was diagnosed 30 years later. The clinical findings in both cases were: skin involvement, hepatosplenomegaly and abdominal pain. The second patient had myelofibrosis. There was a favorable response to H1 and H2 histamine antagonist and ketotifen.
本文报告了两例伴有皮肤受累的良性系统性肥大细胞增多症患者的病历。首例患者在2个月大时被诊断为色素性荨麻疹，并在两年内发展为全身性疾病。第二例患者在22岁时出现色素性荨麻疹，30年后被诊断为良性系统性肥大细胞增多症。两例患者的临床表现均为：皮肤受累、肝脾肿大和腹痛。第二例患者患有骨髓纤维化。患者对H1和H2组胺拮抗剂以及酮替芬反应良好。
