REVISION DE EPIDERMOLISIS AMPOLLAR ADQUIRIDA

Tomada de la Biblioteca virtual de la Universidad Central de Venezuela del servidor EBSCO y del sistema de informacion Dianmed

Description: Ab-mediated blistering skin disease, Ig deposited in upper dermis

Also called: EBA

Organs Involved: subepidermal; dorsum of hands, elbows, knees

Incidence/Prevalence: rare

Causes: mechanical trauma

Skin: large hemorrhagic bullae , milia (little epidermal cysts found in severe blistering disorders) , inflammatory form resembles bullous pemphigoid

HEENT: oromucosal lesions, no hypertrichosis on face

Rule out: porphyria cutanea tarda, bullous pemphigoid (Ig at roof) epidermolysis Bullosa Simplex (GeneClinics 1998 Oct 6)

Blood tests: 25-50% autoAb

Urine studies: urine tests for PCT negative

Pathology tests: split skin technique – Ig in upper dermis (floor of blister), linear band of IgG or C3 at basement membrane zone

Prognosis:resistant to treatment

Treatment

Medications:

• steroids, dapsone
• discussion of cyclosporine in treatment of EBA can be found in Mayo Clin Proc 1996 Dec;71(12):1182
• no randomized trials identified to guide therapy of epidermolysis bullosa acquisita; systematic review last updated 2000 Jun 13 (Cochrane Library 2003 Issue 1:CD004056)
• use of IV immunoglobulin (IVIG) not recommended (University HealthSystem Consortium guidelines on IVIG preparations which were withdrawn from National Guideline Clearinghouse on 2004 Dec 20 as no longer current)

Surgery:

• early case report of successful treatment of newborn with tissue-engineered skin (Apligraf) can be found in Arch Dermatol 1999 Oct;135(10):1219 (in Pediatric Notes 1999 Nov 4;23(44):173)
• tissue-engineered skin (Apligraf) use reported in 15 children with variois types of epidermolysis bullosa, no control group (Arch Dermatol 2000 Oct;136(10):1225 in Pediatric Notes 2000 Oct 26;24(43):172)

Reviews:

• review of autoimmune bullous (blistering) diseases can be found in Am Fam Physician 1997 Jun;55(8):2709
• picture and discussion can be found in Postgrad Med 1999 Mar;105(3):113