Rosario Andrea, Bhat Ramesh M
Department of Dermatology, Venereology and Leprology, Father Muller Medical College, Mangalore, Karnataka, India.
Indian J Dermatol. 2016 May-Jun;61(3):347. doi: 10.4103/0019-5154.182473.
Mastocytosis is a disorder characterized by the clonal proliferation of mast cells and their accumulation in skin, bone marrow, liver, and spleen. Cutaneous mastocytosis presents in children in over 90% of the cases and any cutaneous manifestation in an adult is the earliest sign of the systemic disease. A 45-year-old patient presented with itchy dark lesions over the body since childhood and Darier's sign was positive. Skin biopsy showed features of mastocytosis and immunohistochemistry was positive for CD34. Since the patient was refractory to treatment with antihistamines and psoralen-ultraviolet A therapy, injections of interferon alpha were given - 3 million IU twice weekly subcutaneously as they have been proven to improve constitutional symptoms. Very few reports of successful treatment of cutaneous mastocytosis using interferon alpha have been published.
肥大细胞增多症是一种以肥大细胞克隆性增殖并在皮肤、骨髓、肝脏和脾脏中积聚为特征的疾病。超过90%的儿童会出现皮肤肥大细胞增多症,而成人出现的任何皮肤表现都是系统性疾病的最早迹象。一名45岁患者自童年起全身出现瘙痒性深色皮损,达里埃氏征阳性。皮肤活检显示肥大细胞增多症的特征,免疫组化CD34呈阳性。由于该患者对抗组胺药和补骨脂素-紫外线A疗法治疗无效,因此给予干扰素α注射——每周两次皮下注射300万国际单位,因为已证明其可改善全身症状。关于使用干扰素α成功治疗皮肤肥大细胞增多症的报道极少。