[Pretibial epidermolysis bullosa. A rare form of epidermolysis bullosa simplex]. 1998

P Tsianakas, and S Fraitag, and A Archimbaud, and O Verola, and C Paul, and C Prost
Polyclinique de Dermatologie, Hôpital Saint-Louis, Paris.

BACKGROUND Pretibial epidermolysis bullosa had been classified as a rare localized form of autosomal dominant dystrophic epidermolysis bullosa. METHODS We report a sporadic case of a patient suffering from bullous lesions induced by minor trauma on pretibial skin. The lesions healed with atrophic scars. No milia formation was observed. The mapping of dermoepidermal junction by LH 7:2 and GB3 monoclonal antibodies was normal. By electron microscopy, numerous perinuclear vacuoles were observed and the cleavage occurred within the basal keratinocytes. CONCLUSIONS This patient had clinical features in accordance with a diagnosis of pretibial epidermolysis bullosa. However, in contrast to previous case reports, the ultrastructural pattern was this of an epidermolysis bullosa of simplex type.

UI MeSH Term Description Entries
D007868 Leg Dermatoses A nonspecific term used to denote any cutaneous lesion or group of lesions, or eruptions of any type on the leg. (From Stedman, 25th ed) Leg Dermatosis,Dermatoses, Leg,Dermatosis, Leg
D007869 Leg Injuries General or unspecified injuries involving the leg. Injuries, Leg,Injury, Leg,Leg Injury
D008297 Male Males
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013977 Tibia The second longest bone of the skeleton. It is located on the medial side of the lower leg, articulating with the FIBULA laterally, the TALUS distally, and the FEMUR proximally. Tibias
D015603 Keratinocytes Epidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell. Keratinocyte
D016110 Epidermolysis Bullosa Simplex A form of epidermolysis bullosa characterized by serous bullae that heal without scarring. Mutations in the genes that encode KERATIN-5 and KERATIN-14 have been associated with several subtypes of epidermolysis bullosa simplex. Epidermolysis Bullosa Herpetiformis Dowling-Meara,Epidermolysis Bullosa Simplex Kobner,Weber-Cockayne Syndrome,EBS, Acral Form,EBS, Generalized,EBS-DM,Epidermolysis Bullosa Herpetiformis Dowling Meara,Epidermolysis Bullosa Herpetiformis, Dowling-Meara,Epidermolysis Bullosa Herpetiformis, Dowling-Meara Type,Epidermolysis Bullosa Simplex, Cockayne-Touraine Type,Epidermolysis Bullosa Simplex, Dowling-Meara Type,Epidermolysis Bullosa Simplex, Generalized,Epidermolysis Bullosa Simplex, Koebner Type,Epidermolysis Bullosa Simplex, Localized,Epidermolysis Bullosa Simplex, Weber-Cockayne Type,Epidermolysis Bullosa of Hands and Feet,Weber-Cockayne Type Epidermolysis Bullosa Simplex,Acral Form EBS,Acral Form EBSs,Bullosa Simplices, Epidermolysis,EBSs, Acral Form,EBSs, Generalized,Epidermolysis Bullosa Herpetiformis, Dowling Meara,Epidermolysis Bullosa Herpetiformis, Dowling Meara Type,Epidermolysis Bullosa Simplex, Cockayne Touraine Type,Epidermolysis Bullosa Simplex, Dowling Meara Type,Epidermolysis Bullosa Simplex, Weber Cockayne Type,Epidermolysis Bullosa Simplices,Generalized EBS,Generalized EBSs,Weber Cockayne Syndrome,Weber Cockayne Type Epidermolysis Bullosa Simplex

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