Epidermolysis Bullosa

1:17,000, 50% dystrophic form

Racial distribution equal

Difficult IV access, airway, intraop positioning, reflux, steroid dependence, intraop hemorrhage, sepsis, iatrogenic corneal abrasion, blister formation, and airway obstruction

Problems similar to those found in pts with severe skin burns; severely compromised pts

Difficult intubation (23%) secondary to microstomia

Establishing monitoring and IV access

Dehydration and malnutrition

Anemia, hypoalbuminemia, electrolyte imbalance, and thrombocytosis

Septicemia

Renal and adrenal dysfunction

Characterized by epithelial blistering resulting from minor trauma by lateral shearing forces, not pressure, because of absence of normal intracellular bridges caused by collagen abnormality

Four types: SEB, JEB, DEB, and Kindler syndrome

Associated conditions: Growth retardation, pyloric stenosis, esophageal stricture, pseudosyndactyly, enamel hypoplasia, muscular dystrophy, squamous cell carcinoma, and malignant melanoma

SEB: Most common form; intraepidermal blisters on the soles and palms only in Weber-Cockayne form, generalized in Kobner form, generalized herpetiform in Dowling-Meara form, and generalized in association with muscular dystrophy in the MD form

JEB: Blisters formed in the intralamina lucida and in intertriginous areas in the inversa form, which are generalized with growth retardation in the Herlitz form, generalized without growth retardation in the non-Herlitz form, and generalized with pyloric atresia

DEB: Blisters formed in the sublamina densa and in intertriginous areas in the inversa form, on ankles in the pretibial form, on arms and legs in the pruriginous form; generalized blisters in the non–Hallopeau-Siemens form and with growth retardation and severe extracutaneous involvement in the Hallopeau-Siemens form, and aggressive squamous cell carcinomas (very commonly)

Kindler syndrome: Blisters formed at multiple levels, intralamina lucida and sublamina densa; Kindler syndrome (previously considered as poikilodermatous photosensitivity disease); skin findings including atrophic scarring and nail dystrophy; possibly associated with severe colitis, esophagitis, urethral strictures, and ectropions; squamous cell carcinoma (can develop)