de Carvalho Jozélio Freire
Serviço de Reumatologia da Faculdade de Medicina da Universidade de São Paulo, São Paulo - SP, Brasil.
Acta Reumatol Port. 2009 Apr-Jun;34(2A):246-50.
Degos' disease or malignant atrophic papulosis is a rare vasculopathy characterized by the presence of a typical skin lesion and visceral vascular involvement of small vessels, mainly of the digestive tract or central nervous system. The most interesting fact in this disease is the benign appearance of cutaneous lesion, hiding the occlusion of skin and visceral vessels. The author reports the case of a female patient with systemic lupus erythematosus for eight years. During her follow up, generalized skin papules were observed on the trunk and limbs, sparing her face, hands and feet, compatible with Degos' disease. Additional imaging investigation excluded systemic involvement of the disease. Treatment with acetylsalicylic acid prevented the appearance of new cutaneous manifestations and the patient remains clinically stable on the Outpatient Clinic without complications, until this moment. Malign atrophic papulosis is a rare disease with a poor prognosis. However, its association with systemic lupus erythematosus seems to follow a more benign course, without the typical visceral involvement.
德戈斯病或恶性萎缩性丘疹病是一种罕见的血管病,其特征是存在典型的皮肤病变以及小血管的内脏血管受累,主要累及消化道或中枢神经系统。该病最有意思的一点是皮肤病变外观呈良性,掩盖了皮肤和内脏血管的闭塞情况。作者报告了一例患系统性红斑狼疮八年的女性患者。在随访期间,在其躯干和四肢观察到全身性皮肤丘疹,面部、手部和足部未出现,符合德戈斯病表现。进一步的影像学检查排除了该病的系统性受累。阿司匹林治疗预防了新的皮肤表现出现,直至目前,该患者在门诊临床情况稳定,无并发症。恶性萎缩性丘疹病是一种预后较差的罕见疾病。然而,它与系统性红斑狼疮的关联似乎病程更为良性,无典型的内脏受累情况。