Genetically-determined epidermolysis bullosa

Definition

Epidermolysis bullosa (EB) is the name given to a heterogeneous group of blistering disorders which in the majority of cases are due to genetically-determined defects in structural proteins of the epidermis and dermo-epidermal junction. The genetic forms are to be distinguished from the immunobullous disorder, epidermolysis bullosa acquisita (qv).

Exclusions

• Epidermolysis bullosa acquisita (EB43)

5 subordinate codes

• EC30Epidermolysis bullosa simplex
• EC31Junctional epidermolysis bullosa
• EC32Dystrophic epidermolysis bullosa
• EC33Syndromic epidermolysis bullosa
• 4B4ZDiseases of the immune system, unspecified

Nearby in Genetic or developmental disorders affecting the skin

• EC10-EC1YGenetic syndromes affecting the skin
• EC20Genetic disorders of keratinisation
• EC21Genetic defects of hair or hair growth
• EC22Genetic defects of nails or nail growth
• EC23Genetic disorders of skin pigmentation
• EC40-EC4YGenetic disorders affecting dermal collagen, elastin or other matrix proteins
• EC50-EC5YSpecified developmental anomalies affecting the skin
• Chromosomal disorders affecting the skin