Epidermolysis Bullosa Follow-up

  • Author: M Peter Marinkovich, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 22, 2010
 

Further Inpatient Care

When a patient with epidermolysis bullosa (EB) is hospitalized for severe blistering, treat the blisters aggressively with wound and nutritional management (see Wound healing in Medical Care; Nutritional management in Diet). Regular whirlpool therapy can help with gentle cleansing and debridement of wounds. Whirlpool therapy is a helpful adjunct available in most hospitals and assists in the care of inpatients with epidermolysis bullosa.

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Further Outpatient Care

Implementation of proper wound and nutritional care is critical to the outpatient care of epidermolysis bullosa. Home health care providers familiar with skin care, nutrition, and physical therapy can be helpful. Education of patient and family members is essential.

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Inpatient & Outpatient Medications

Patients with severe epidermolysis bullosa require significant amounts of wound-care supplies, such as plain petroleum gauze, nonadhering gauze such as Adaptic or Telfa, petroleum jelly, antibiotic ointment, and self-adhering gauze. Be sure to prescribe sufficient quantities of these materials. Insurance companies and health maintenance organizations may neglect to cover these essential therapies. Physicians and social workers working together may need to advocate for their patients in this regard.

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Transfer

Take great care to avoid trauma to the skin during transfers or additional blistering will occur, especially in patients with severe epidermolysis bullosa. Never apply tape to the skin of patients with epidermolysis bullosa.

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Deterrence/Prevention

Prevention of trauma to the skin reduces blistering. Padding of limbs helps reduce unnecessary trauma. A soft mechanical diet helps reduce oral and esophageal erosions.

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Complications

Complications can include the following:

  • SCC: Arising in chronic wounds or scars of recessively inherited epidermolysis bullosa, this form of SCC is invasive and has high metastatic potential. Other epidermolysis bullosa subtypes do not show a tendency to develop SCC.
  • Pseudosyndactyly (mitten-hand deformity): This is a frequent complication in patients with recessively inherited epidermolysis bullosa but is rare in other subtypes. In this disorder, skin grows around the digits because of repeated blistering and dystrophic healing. Over time, the digits are encased in a mitten of skin. Therapeutic surgical approaches are available, but the rate of recurrence is high (see Surgical Care).
  • Mucosal complications: Patients with recessively inherited epidermolysis bullosa often have esophageal manifestations. Esophageal scarring secondary to repeated blistering and healing results in dysphagia from webbing, strictures, or stenosis. These complications are rare in patients with epidermolysis bullosa simplex but occur in patients with Herlitz and other nonlethal forms of junctional epidermolysis bullosa and dominantly inherited dystrophic epidermolysis bullosa. No cases of esophageal involvement have been reported in the generalized benign atrophic form of junctional epidermolysis bullosa (see Surgical Care). While patients with the Herlitz form of junctional epidermolysis bullosa have the greatest tendency for tracheolaryngeal involvement, recessively inherited epidermolysis bullosa may involve the tracheolaryngeal mucosa as well.
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Prognosis

Epidermolysis bullosa is a lifelong disease. Some subtypes, especially the milder epidermolysis bullosa forms, improve with age.

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Patient Education

Education in proper nutrition and wound care is essential for the patient and family.

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Contributor Information and Disclosures
Author

M Peter Marinkovich, MD  Associate Professor, Department of Dermatology and Program in Epithelial Biology, Stanford University Medical Center; Dermatology Service, VA Palo Alto Health Care System

M Peter Marinkovich, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Jean Paul Ortonne, MD  Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France

Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Epidermolysis bullosa simplex, Weber-Cockayne subtype. This mild bullous disease is characterized by localized blistering at sites of trauma such as the feet.
Epidermolysis bullosa simplex, Koebner subtype. Palmoplantar blistering and hyperkeratosis are noted.
Epidermolysis bullosa simplex, Koebner subtype. Close-up image shows hyperkeratotic papules and plaques on the palm.
Junctional epidermolysis bullosa, Herlitz subtype. This severe disease is characterized by generalized intralamina lucida blistering at birth, significant internal involvement, and a poor prognosis.
Dominantly inherited dystrophic epidermolysis bullosa. The blistering in this disease often is localized and is characterized by scarring and milia in healed blister sites.
Dominantly inherited dystrophic epidermolysis bullosa. This subtype, similar to other dystrophic and junctional epidermolysis bullosa subtypes, can result in nail dystrophy and loss.
Recessively inherited dystrophic epidermolysis bullosa pseudosyndactyly (mitten-hand deformity) of the hands and feet
Recessively inherited dystrophic epidermolysis bullosa, oral cavity blistering and scarring
Recessively inherited dystrophic epidermolysis bullosa, squamous cell carcinoma
Diagram illustrating the organization of the dermal epidermal basement membrane and level of disruption in epidermolysis bullosa subtypes
 
 
 
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