Title: Epidermolysis bullosa
Authors: Heddie Sedano DDS and Robert J. Gorlin DDS MS
Journal: Oral Surgery Oral Medicine and Oral Pathology
Dates: May 1989
Topic: review of types and symptoms of EB
Summary:
Epidermolysis Bullosa Simplex: most frequently involved in sites of friction or trauma; altered nails in ~20% of cases; no scarring or pigmentation following healing. Often affects the hands, feet and neck. Most often found neonatally or in infants. Intraoral blisters are less severe than other forms of EB. Teeth are not affected.

EB Atrophicans Generalisata Gravis (Herlitz type): present within the first few hours of life and hemorrhagic vesicles are seen at the base of the fingernails. The face and scalp are also affected. Palms and soles are never affected. Fragile and hemorrhagic vesicles are found at the junction of hard and soft palates. Hypoplastic and pitted enamel leading to extensive caries principally in the molar teeth. Many die in the first few months, those who survive have syndactyly, anemia, growth retardation and nail dystrophy. Perioral and perinasal crusted and granular hemorrhagic lesions are pathognomonic in adult patients.

Dominant Dystrophic Form: Flat, pink scar producing bullae of the ankles, knees and hands. 20% of patients manifest oral bullae.

Scarring EB with Dermolytic Blisters: 4 different types with bullae usually manifesting at or shortly after birth, caused by pressure ad trauma. In infants, the occiput, the scapulas, elbows, fingers buttocks and feet are most affected. The bullae leave painful ulcers upon rupture. Keloidal scars often follow healing. Formation of clawhand and enclosure of the hand in a glovelike epidermal sac have been noted frequently. Dwarfism can be secondary to scarring in growth areas.Shrinkage of the conjunctiv aand keratitis with corneal opacity has also been noted. Hoarseness, aphonea and dysphagia may result due to bulla in the larynx. Hypoplastic enamel rapidly affected by dental caries, delayed eruption and frequent retention. Oral mucosal vesicles form soon after birth from the negative pressure from sucking. The lingual mucosa appears thick, gray and smooth. Repeated blistering may lead to limited opening and akyloglossia. Other related conditions are severe periodontal disease with alveolar bone resorption, atrophy of the maxilla with resultant relative mandibular prognathism, increased mandibular angle and oral carcinoma.

EB acquisita: blisters in areas of trauma; associated with amyloidosis, multiple myeloma, diabetes meelitus and IBD (may represent a GI cutaneous syndrome with an immune pathogenesis). Extensive dental caries, leading to complete loss of teeth has been noted in every patient with this condition.

EB non scarring atrophicans generalisata mitis: oral mucosal bulla,nodular excrescences on the palate and gingiva, some cases present with pitted enamel.

Assessment: Interesting but a lot of 'sometimes' and 'may lead to' implying.