Department of Skin and V.D., PGIMS, Rohtak, Haryana, India.
Indian J Dermatol Venereol Leprol. 2013 Mar-Apr;79(2):235-7. doi: 10.4103/0378-6323.107645.
Epidermolysis bullosa pruriginosa (EBP) is a subtype of dominant dystrophic epidermolysis bullosa (DDEB) and is clinically characterized by pruritic lichenified plaques or prurigo-like lesions with violaceous linear scarring. Pruritus has always been described as one of the most striking features in EBP. Mutations in COL7A gene, especially in the glycine residue, have been shown to cause this form of DDEB. In this report, we describe a north Indian familial clustering of three cases of EBP, spread across two generations, presenting with hypertrophic lichenoid cutaneous lesions, which were completely asymptomatic. Clinical and histopathological analysis favored the diagnosis of EBP in all three cases. They are being reported for their unusual asymptomatic presentation.
瘙痒性大疱性表皮松解症（EBP）是显性营养不良性大疱性表皮松解症（DDEB）的一个亚型，临床上以瘙痒性苔藓样斑块或痒疹样皮损伴紫色线状瘢痕为特征。瘙痒一直被描述为 EBP 最显著的特征之一。COL7A 基因突变，特别是甘氨酸残基突变，可导致这种形式的 DDEB。在本报告中，我们描述了三例 EBP 病例在两代人之间的北印度家族聚集，表现为肥厚性苔藓样皮肤病变，完全无症状。所有三例均通过临床和组织病理学分析支持 EBP 的诊断。我们报告这些病例是因为它们具有不同寻常的无症状表现。
