Epidermolysis bullosa acquisita



Epidermolysis bullosa acquisita


Lawrence S. Chan


Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports


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Epidermolysis bullosa acquisita is an uncommon, chronic, autoimmune blistering disease of the skin and mucous membranes. The disease primarily affects elderly individuals and occurs predominantly at trauma-prone skin areas (the non-inflammatory mechanobullous scarring subset) or widespread skin areas (the generalized inflammatory non-scarring subset). IgG (or rarely IgA) autoantibodies targeting the skin basement membrane component type VII collagen (anchoring fibrils) and minor physical trauma are the major contributing factors to the blistering process.



Management strategy


Epidermolysis bullosa acquisita, especially the non-inflammatory mechanobullous subset, is characteristically very resistant to conventional medical therapies. For an immune-mediated blistering disease associated with autoantibodies that target skin components, the logical approach is to modify the immune response to reduce the production and effect of the autoantibodies to their target skin component type VII collagen. However, no target-specific treatment is currently available. Thus the presently available non-target-specific immunosuppressants not only reduce the immune responses against self protein, but also suppress the patient’s immune defense to pathogens, resulting in a relative immunodeficiency. Therefore, when treating patients with this disease, every effort should be made to use anti-inflammatory rather than immunosuppressant agents, to use the lowest possible doses of immunosuppressant for the shortest duration, and to replace immunosuppressants with other anti-inflammatory medications whenever possible. A commonly used initial regimen is systemic corticosteroid with either mycophenolate mofetil or dapsone or both as a corticosteroid-sparing agent. For adult patients without significant medical problems, a combination of oral prednisone (1 mg/kg daily), mycophenolate mofetil (1–2 g daily), and dapsone (100–200 mg daily) can be started. Because of its rarity, no well-controlled clinical trial has been performed for epidermolysis bullosa acquisita. The following therapeutic guidelines are derived mainly from case reports of small groups or single patients.


Other therapeutic agents have been reported to be beneficial for this disease. Colchicine (1–2 mg daily) has been reported to significantly improve the disease. Cyclosporine (5–9 mg/kg daily) has been shown to be beneficial in reducing blister formation and speeding up healing. Intravenous immunoglobulin (IVIG) treatment (400 mg/kg daily) has also been demonstrated to reduce new blister formation and facilitate healing. In addition, extracorporeal photochemotherapy has been used successfully in some patients. Most recently, a monoclonal antibody against B-cell-specific target CD20 medically termed rituximab (usual dose 375 mg/m2 body surface area, multiple doses) has been shown to be quite effective in several cases of epidermolysis bullosa acquisita, considering the medication-resistant nature of the disease. At present the high cost and difficulty of obtaining insurance company approval are the major hindrances to the use of rituximab.


In addition to medical treatments, patients with this disease should be instructed to avoid physical trauma as much as possible. Vigorous rubbing of their skin and the use of harsh soaps and hot water should also be avoided. Patients should be instructed to care for open wounds promptly and to recognize local skin infection and seek medical attention when infection occurs.



Specific investigations

















Congenital epidermolysis bullosa acquisita: vertical transfer of maternal autoantibody from mother to infant.

Abrams ML, Smidt A, Benjamin L, Chen M, Woodley D, Mancini AJ. Arch Dermatol 2011; 147: 337–41.


While epidermolysis bullosa acquisita rarely occurs in children, it has never been reported in an infant until now. This case, which occurred in an infant, should raise the awareness of maternally transferred epidermolysis bullosa acquisita by physicians when they encounter blistering disease in a neonate.


Physicians need to recognize the possibility of maternal transfer of autoantibodies and the transient nature of the blisters (with no need for systemic treatment). This case of naturally passive transfer disease further demonstrates the pathogenic role of the autoantibodies as was illustrated in animal model of epidermolysis bullosa acquisita.

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Aug 7, 2016 | Posted by in Dermatology | Comments Off on Epidermolysis bullosa acquisita

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