Das Jayanta Kumar, Sengupta Sujata, Gangopadhyay Asok Kumar
Department of Dermatology, Ramakrishna Mission Seva Pratisthan Hospital, Kolkata, India.
Indian J Dermatol Venereol Leprol. 2005 Mar-Apr;71(2):109-11. doi: 10.4103/0378-6323.13996.
Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, is characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions, appearing at birth or later. Scarring and prurigo are most prominent on the shins. Treatment is unsatisfactory. We report three such cases: two of them first cousins, are described with history of blisters since childhood, followed by intensely pruritic lesions predominantly on the shins, and dystrophy of toenails, but no albopapuloid lesions or milia. Intact blisters were present in one case, and excoriations were seen in the other two. All of them showed encouraging response to cryotherapy.
痒疹性大疱性表皮松解症是一种遗传性机械性大疱病，其特征为瘙痒、苔藓化或结节状痒疹样皮损、偶尔因外伤诱发水疱、抓痕、粟丘疹、甲营养不良和白色丘疹样皮损，于出生时或出生后出现。瘢痕和痒疹在小腿最为明显。治疗效果不理想。我们报告3例此类病例：其中2例为近亲，自幼有水泡病史，随后主要在小腿出现剧烈瘙痒性皮损以及趾甲营养不良，但无白色丘疹样皮损或粟丘疹。1例有完整水疱，另外2例可见抓痕。所有病例冷冻治疗后均有令人满意的反应。
