Presentation of pleural effusion and dilated cardiomyopathy in a case of dystrophic epidermolysis bullosa

被引：1

作者：

Arshad, Muhammad Asharib ^{[1
]}

Ayub, Muhammad Rashid ^{[2
]}

Zil-E-Ali, Ahsan ^{[4
]}

Rashid, Sumara ^{[3
]}

Kazi, Muhammad Yaqub ^{[2
]}

Pervaiz, Muhammad Arshad ^{[5
]}

机构：

[1] FMH Coll Med & Dent, Lahore, Pakistan

[2] FMH Coll Med & Dent, Dept Pediat Med, Lahore, Pakistan

[3] FMH Coll Med & Dent, Dept Dermatol, Lahore, Pakistan

[4] Punjab Rangers Teaching Hosp, Dept Surg, Lahore, Pakistan

[5] Hamad Med Corp, Hamad Gen Hosp, Dept Anesthesia, Doha, Qatar

来源：

JOURNAL OF THE PAKISTAN MEDICAL ASSOCIATION | 2020年 / 70卷 / 06期

关键词：

Epidermolysis Bullosa Dystrophica; Pleural Effusion; Dilated Cardiomyopathy;

D O I：

10.5455/JPMA.49858

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Epidermolysis Bullosa (EB), a genetic disorder of the skin that presents with eruptive lesions accompanied by blistering has multiple types. We present a case of dystrophic and epidermolysis bullosa (DEB), a rare variant of the disease with the underlying pathophysiology involving a mutation of type VII collagen that serves as an anchoring protein for basement membrane to the dermis. The patient presented with palmoplantar hyperkeratosis with blistering extending on multiple sites of the body, bilateral pleural effusion and an ejection fraction of 23% with moderate mitral regurgitation. The patient was treated symptomatically with diuretics and inotropic medication for the dilated heart, along with draining of pleural spaces. No case of DEB with pleural effusion has been reported prior to this one. We believe this is the first case that presented with both pleural effusion and dilated cardiomyopathy.

引用

页码：1106 / 1109

页数：4

共 10 条

[1] Evaluating the Hemodynamical Response of a Cardiovascular System under Support of a Continuous Flow Left Ventricular Assist Device via Numerical Modeling and Simulations [J].