Kondo Y, Umegaki N, Terao M, Murota H, Kimura T, Katayama I
Department of Dermatology Integrated Medicine, Osaka University Graduate School of Medicine, Suita-City, Japan.
Case Rep Dermatol. 2012 Jan;4(1):85-91. doi: 10.1159/000337751. Epub 2012 Mar 30.
Acanthosis nigricans (AN) is a hyperpigmented keratotic skin lesion known to be associated with malignant disease and endocrinopathy. We report a very rare case of generalized AN with Sjögren's syndrome- and systemic lupus erythematosus-like features but without type B insulin resistance. Neither internal malignancy nor other endocrinological disorders, including glucose intolerance, were detected during a 10-year clinical course with benign diffuse papillomatosis extending from the mucosa of the larynx to the esophagogastric junction. The case was complicated with chronic thyroiditis and interstitial pneumonia, which were not treated with any medication. AN skin lesions and mucosal papillomatosis regressed with oral cyclosporine A, accompanied by the lowering of autoantibody titers. This is the first report of generalized AN involving an area from the mucosa of the larynx to the esophagogastric junction accompanied by autoimmune manifestations which responded to systemic immunosuppressive therapy.
黑棘皮病（AN）是一种色素沉着性角化性皮肤病变，已知与恶性疾病和内分泌病相关。我们报告了一例非常罕见的全身性AN病例，具有干燥综合征和系统性红斑狼疮样特征，但无B型胰岛素抵抗。在长达10年的临床病程中，未检测到内部恶性肿瘤或其他内分泌紊乱，包括糖耐量异常，病变表现为从喉黏膜延伸至食管胃交界处的良性弥漫性乳头状瘤病。该病例合并慢性甲状腺炎和间质性肺炎，未接受任何药物治疗。口服环孢素A后，AN皮肤病变和黏膜乳头状瘤病消退，自身抗体滴度降低。这是首例累及从喉黏膜到食管胃交界处区域的全身性AN伴有自身免疫表现且对全身免疫抑制治疗有反应的报告。
