与真性红细胞增多症相关的迟发性膀胱大泡性肾小球病

Ait Khabba Chaimae, Mehsas Zoubida, Asermouh Marwa, Meziane Meriem, Ismaili Nadia, B. Laila, Senouci Karima
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引用次数: 0

摘要

膀胱大泡性病变以及迟发性病变是达里尔病(DD)极为罕见的特征,这使得阳性诊断更加困难。它与真性红细胞增多症的关联提示寻找提示恶性血液学转化的迹象。我们报告一个62岁的病人,从58岁开始在内科治疗真性红细胞增多症。她提出了瘙痒性水泡大疱性皮肤病发展了4年。临床检查发现,丘疹-水疱斑块累及所有皱襞:腹股沟、腋窝、臀间、颈部和身体,并伴有掌足底水疱和脓疱。趾甲检查显示红色和白色相间的纵向带。腹部检查显示中度脾肿大。皮肤活检显示局灶性棘层松解伴基底上裂,角化不良伴圆形体和颗粒,与Darier病相符。副肿瘤评估包括胸腹盆腔CT扫描,乳房超声和乳房x光检查,宫颈阴道涂片,腔体CT扫描和胰腺MRI,未显示任何进行性恶性过程。患者口服类维生素a 0.5 mg/kg /天,外用钙三醇伴放血以降低红细胞压积水平。这一进展以临床改善为标志。患者在血液科随访,随访2年后未发现急性白血病、骨髓纤维化或其他肿瘤。我们报告一例晚期DD的妇女真性红细胞增多症,特点是一个罕见的囊泡大泡的表现,其因果关系仍有待确定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Vesiculo-bullous Darier disease late onset in association with polycythemia vera
Vesiculo-bullous lesions as well as the late onset are extremely rare features of Darier disease (DD) making positive diagnosis more difficult. Its association with polycythemia vera prompts to look for signs suggestive of malignant hematological transformation. We report a case of a 62-year-old patient, followed in internal medicine since the age of 58 for polycythemia vera. She presented for pruriginous vesiculo-bullous dermatosis evolving for 4 years. On clinical examination, papulo-vesicular plaques were found involving all the folds: inguinal, axillary, inter-gluteal, neck and body with palmo-plantar blisters and pustules. Examination of the toe nails showed alternating red and white longitudinal bands. Abdominal examination showed moderate splenomegaly. Skin biopsy revealed focal acantholysis with supra-basal clefts, dyskeratosis with the presence of round bodies and grains compatible with Darier's disease. A paraneoplastic assessment was made including a thoraco-abdomino-pelvic CT scan, a breast ultrasound and mammography with cervico-vaginal smear, a CT scan of the cavum and a pancreatic MRI, which did not show any progressive malignant process. The patient was put on oral retinoids at a rate of 0.5 mg/kg per day and topical calcipotriol associated with bloodletting to reduce the hematocrit level. The evolution was marked by clinical improvement. The patient was followed in the hematology department and showed no acute leukemia or myelofibrosis or other neoplasms after a 2-year follow-up. We report a case of late DD in a woman followed for polycythemia vera, characterized by a rare vesiculo-bullous presentation and whose causal link remains to be determined.
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