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Leopard skin.
The Lancet Diabetes & Endocrinology ( IF 44.0 ) Pub Date : 2020-03-17 , DOI: 10.1016/s2213-8587(20)30076-0
Holger Henneicke 1 , Wulf Tonnus 2 , Lorenz C Hofbauer 1
Affiliation  

A 58-year-old woman presented with fever and fatigue for the past 4 weeks. She reported hyperpigmented lesions on her arms and face on a background of mostly hypopigmented skin, creating a leopard-like appearance (). The lesions first appeared 9 months earlier, and the dark pigment intensified over time. Her history included vitiligo and primary adrenal insufficiency, for which she received glucocorticoid replacement therapy with delayed-release hydrocortisone (10 mg orally per day; 0·11 mg per kg body weight [87 kg]) and fludrocortisone (0·1 mg orally per day). Serum electrolytes were normal; C-reactive protein was raised to 40 mg/L (reference: <5 mg/L); and erythrocyte sedimentation rate was elevated at 46 mm/h (reference: <30 mm/h after 1 h), while complete blood count was within the normal range. Serum cortisol was 17 nmol/L (reference: 124–662 nmol/L) and adrenocorticotropic hormone (ACTH) was 274 pmol/L (reference: 2·6–10·1 pmol/L). Based on her presentation, we suspected inadequate glucocorticoid replacement because of enhanced glucocorticoid demand. Hydrocortisone was increased to 40 mg per day. A CT scan showed splenomegaly and diffuse lymphadenopathy (). Surgical extirpation and histopathological analysis of an axillary lymph node revealed follicular lymphoma (stage 3B). The insidious development of lymphoma might have enhanced the patient's glucocorticoid requirement, as reflected by her symptoms and elevated concentrations of ACTH. Cosecretion of α-melanocyte-stimulating hormone with ACTH, both from the common precursor POMC, enhanced the contrast of hyperpigmented islands on vitiligous skin. After treatment with bendamustine and rituximab (R-Bendamustine protocol: 3 cycles, cumulative dose of bendamustine at 1080 mg [IV], cumulative dose of rituximab at 2250 mg [IV]), lymphadenopathy and splenomegaly had disappeared and the patient was free of symptoms when on hydrocortisone (20 mg per day).

中文翻译:

豹皮。

一名58岁的女性在过去4周内出现发烧和疲劳。她报告说,她的胳膊和脸上的色素沉着病变多为色素沉着的皮肤,形成了豹子般的外观()。病变首先在9个月前出现,并且深色色素随时间推移而加剧。她的病史包括白癜风和原发性肾上腺功能不全,为此接受了糖皮质激素替代治疗,包括延迟释放的氢化可的松(每天口服10 mg;每千克体重[87 kg] 0·11 mg)和氟可的松(每次口服0·1 mg)天)。血清电解质正常;C反应蛋白升高至40 mg / L(参考值:<5 mg / L);红细胞沉降速率提高到46 mm / h(参考值:1 h后<30 mm / h),而全血细胞计数在正常范围内。血清皮质醇为17 nmol / L(参考:124–662 nmol / L)和促肾上腺皮质激素(ACTH)为274 pmol / L(参考:2·6-10·1 pmol / L)。根据她的介绍,我们怀疑由于糖皮质激素需求增加,糖皮质激素替代不足。氢化可的松增加到每天40毫克。CT扫描显示脾肿大和弥漫性淋巴结病()。腋窝淋巴结的手术切除和组织病理学分析显示滤泡性淋巴瘤(3B期)。淋巴瘤的隐匿发展可能增加了患者对糖皮质激素的需求,这从她的症状和ACTH浓度升高反映出来。共同的前体POMC中的α-黑素细胞刺激激素与ACTH的共分泌增强了玻璃质皮肤上色素沉着的岛的对比。用苯达莫司汀和利妥昔单抗治疗后(R-苯达莫司汀方案:
更新日期:2020-03-19
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