21岁女性大疱性表皮松解症合并佝偻病1例。

IF 0.9 Q4 DERMATOLOGY Case Reports in Dermatology Pub Date : 2022-09-01 DOI:10.1159/000525068
Renni Yuniati, Rakhma Yanti Hellmi, Gema Citra Dwijayanti, Meira Dewi Kusuma Astuti, Gerard Pals, Dimitra Micha, Sultana Mh Faradz
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摘要

大疱性表皮松解症(EB)是一组罕见的遗传性疾病,表现为皮肤的机械脆性。当皮肤受到创伤时,这种情况会导致皮肤出现水泡、皮肤糜烂和皮肤溃疡。在这个病例报告中,我们提出了一个21岁女性EB和多发性骨骼畸形的病例。她从4岁开始就出现反复的皮肤脱落和水泡,从9岁开始出现多处骨骼弯曲。在体检中,我们发现全身性色素沉着斑与皮肤红斑。有许多大疱和结痂病变,病变上有糜烂和擦伤。实验室检查发现维生素D 25-OH含量低(8.6 ng/mL)。骨密度测量发现骨密度低,x线检查发现骨质减少和骨弯曲。使用全外显子组测序,未检测到与孟德尔遗传疾病相关的基因的致病序列或拷贝数变异。除了遗传疾病外,维生素D 25-OH水平低可能是该患者佝偻病发生的主要原因。
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Epidermolysis Bullosa and Rickets in a 21-Year-Old Female: A Case Report.

Epidermolysis bullosa (EB) is a group of rare genetic diseases that exhibit mechanical fragility of the skin. This condition will result in the occurrence of skin blisters, skin erosions, and skin ulcerations when the skin is subjected to trauma. In this case report, we present a case of EB and multiple skeletal deformities in a 21-year-old female. She came to our clinic with recurrent skin exfoliations and blisters that occurred since she was 4 years old and multiple bones bowing since she was 9 years old. On physical examinations, we found generalized hypopigmentation macule with erythematous skin. There were numerous bullae and crusted lesions, with erosion and excoriations on the lesions. Laboratory examinations identified low vitamin D 25-OH (8.6 ng/mL). Bone densitometry measurement found low bone density, and X-ray examination found osteopenia and bone bowing. Using whole-exome sequencing, no causative pathogenic sequence or copy number variants in the genes associated with Mendelian inherited disorders were detected. The low levels of vitamin D 25-OH may most likely be the main reason for the occurrence of rickets in this patient aside from the genetic disorder.

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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
57
审稿时长
9 weeks
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