Pretibial epidermolysis bullosa: report of a case. 1990

D J Lichtenwald, and W Hanna, and D N Sauder, and H R Jakubovic, and D Rosenthal
Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Pretibial epidermolysis bullosa is a rare variant of hereditary epidermolysis bullosa that is characterized by the delayed onset of a blistering eruption limited to the pretibial area. We present a case in a 48-year-old woman. Immunofluorescence mapping demonstrated blister formation below the basement membrane. This was confirmed by ultrastructural investigations that also revealed rudimentary anchoring fibrils similar to those described in the dominant dystrophic forms of epidermolysis bullosa.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010375 Pedigree The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition. Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D001768 Blister Visible accumulations of fluid within or beneath the epidermis. Bulla,Vesication,Bleb,Bullae,Bullous Lesion,Blebs,Blisters,Bullous Lesions,Lesion, Bullous,Lesions, Bullous,Vesications
D004820 Epidermolysis Bullosa Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties. Acantholysis Bullosa
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001485 Basement Membrane A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers. Basal Lamina,Basement Lamina,Lamina Densa,Lamina Lucida,Lamina Reticularis,Basement Membranes,Densas, Lamina,Lamina, Basal,Lamina, Basement,Lucida, Lamina,Membrane, Basement,Membranes, Basement,Reticularis, Lamina
D001706 Biopsy Removal and pathologic examination of specimens from the living body. Biopsies
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
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

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