Ben Ghorbel Imed, Litaiem Noureddine, Chelly Ines, Houman Habib
Department of Internal Medicine, University of Tunis El Manar, Tunis, Tunisia.
Department of Dermatology, University of Tunis El Manar, Tunis, Tunisia.
BMJ Case Rep. 2014 Jun 18;2014:bcr2014204339. doi: 10.1136/bcr-2014-204339.
A 27-year-old woman presented with persistent dryness of the mouth and eyes. She presented with permanent photodistributed rash involving the face and distal extremities. Laboratory tests showed positive Sjögren's syndrome (SS)-A and SS-B antibodies. Histological examination of minor salivary gland biopsy revealed inflammatory infiltration grade 4 according to Chisholm's classification. Skin biopsy showed acanthosis, hyperkeratosis in the epidermis and little inflammatory infiltrate in the dermis. There was an infiltration of CD4 T lymphocytes in the dermis. Based on the characteristics of the dermatitis and on a rapid response to niacin replacement, the diagnosis of pellagra was carried out. A complete resolution of the dermatological signs was obtained within 2 months. To the best of our knowledge, the association between primary SS and pellagra has never been reported. We emphasise the possible mechanisms of this association.
一名27岁女性出现持续的口干和眼干症状。她还出现了累及面部和四肢远端的永久性光分布性皮疹。实验室检查显示干燥综合征（SS）-A和SS-B抗体呈阳性。根据Chisholm分类，小唾液腺活检的组织学检查显示炎症浸润为4级。皮肤活检显示表皮棘层增厚、角化过度，真皮内炎症浸润较少。真皮内有CD4 T淋巴细胞浸润。基于皮炎的特征以及对烟酸替代治疗的快速反应，诊断为糙皮病。2个月内皮肤症状完全消退。据我们所知，原发性干燥综合征与糙皮病之间的关联从未有过报道。我们强调了这种关联的可能机制。
