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Epidermolysis bullosa

Epidermolysis bullosa Causes

Many stratified squamous epithelial tissues, such as the skin and oral mucosa, contain a complex BMZ. The BMZ is composed of many specialized components that combine to form anchoring complexes. At the superior aspect of the BMZ, keratin-containing intermediate filaments of the basal cell cytoskeleton insert on basal cell plasma membrane condensations termed hemidesmosomes. Anchoring filaments extend from the basal cell plasma membrane into the extracellular environment and span the lamina lucida, connecting hemidesmosomes with the lamina densa. At the most inferior aspect of the BMZ, type VII collagen-containing anchoring fibrils extend from the lamina densa into the papillary dermis, connecting the lamina densa to anchoring plaques, trapping interstitial collagen fibrils. Thus, the cutaneous BMZ connects the extensive basal cell cytoskeletal network with the abundant network of interstitial collagen fibrils in the dermis.

Epidermolysis bullosa Definition

Epidermolysis Bullosa is a very rare disease inherited genetically. It is often characterized by very fragile skin and blister formation that may come and go. These skin blisters often result from minor friction to other rough surfaces or by trauma.

Epidermolysis bullosa Diagnosis

To diagnose the condition, several clinical tests are run. It includes taking down the medical history of the patients including a review of systems information which can be helpful in associating it with various EB subtypes. A complete physical examination is also performed.

Epidermolysis bullosa Symptoms and Signs

Most common symptoms of patients are skin blisters.

Epidermolysis bullosa Treatment

Treatments for Epidermolysis bullosa includes some medical care which are mostly preventive and supportive. Blisters are punctured with sterile needle to drain the liquid. Open wounds are dressed up to avoid infection. Daily application of topical ointments with polymyxin, silver sulfadiazine and bacitracin. Surgical procedures are done to correct deformities. Frequent consultations to the attending physician is also required to monitor the condition. Diet and activities are also especially modified for the patients.

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