Epidermolysis bullosa acquisita in Child
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Synopsis
There are several clinical forms of EBA, as defined by the International Bullous Diseases Group in 2018:
- Classical / mechanobullous form – A noninflammatory form that affects trauma-prone sites and extensor skin surfaces.
- Nonclassical / nonmechanobullous bullous pemphigoid (BP)-like form – An inflammatory form with features characteristic of BP mixed with atypical lesions for BP, such as skin fragility and milia. It is characterized by a generalized eruption of vesicles and bullae.
- Mucous membrane form – A mucous membrane pemphigoid-like form, which predominantly affects mucous membranes (mouth, pharynx, conjunctiva, genitalia).
- Brunsting-Perry form – A chronic recurrent blistering dermatosis of the head and neck that presents with scarring alopecia.
- IgA form – A presentation that may resemble linear IgA bullous dermatosis and presents with linear IgA deposits in the basement membrane.
EBA can also occur in different clinical settings; there are reports of EBA occurring as a paraneoplastic phenomenon, in association with psoriasis vulgaris, and in the setting of inflammatory bowel disease or other autoimmune diseases, particularly systemic lupus erythematosus (SLE). A meta-analysis estimated the prevalence of associated comorbidities at close to 10%. The same study found incidence of inflammatory bowel disease in 1.5% of patients with EBA. It is postulated that the damaged gut epithelium exposes previously sequestered collagen VII to the immune system, resulting in the generation of auto-antibodies, which escape and go on to attack the skin.
Associations with endocrinopathies and other systemic complications do not seem to follow the same rate in pediatric cases. The few pediatric cases that have associated autoinflammation were also found to have HLA-DR2 (most frequently), HLA-DR4, HLA-DR5, or HLA-DR7 aberrancy.
An increased incidence of EBA in patients with darker skin phototypes has previously been noted. This has now been attributed to enriched representation of HLA-DRB1*15:03 allele found in African Americans as well as HLADRB1*16.
EBA typically has a slow onset and a chronic clinical course. Scarring may produce secondary dysfunction, particularly of the hands and fingers, in the mechanobullous variant. Other sequelae include hair and nail loss, esophageal stenosis, periodontal disease, malnutrition, and blindness.
Related topic: epidermolysis bullosa simplex
Codes
ICD10CM:L12.30 – Acquired epidermolysis bullosa, unspecified
SNOMEDCT:
2772003 – Epidermolysis bullosa acquisita
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Bullous pemphigoid / bullous pemphigoid of childhood
- Mucous membrane pemphigoid
- Linear IgA bullous dermatosis / linear IgA bullous dermatosis of childhood
- Porphyria cutanea tarda
- Dermatitis herpetiformis
- Bullous systemic lupus erythematosus – To distinguish SLE-associated EBA from bullous SLE, EBA tends to be resistant to treatment, whereas marked improvement of lesions after initiation of dapsone or azathioprine is more characteristic of bullous SLE.
- Bullous drug eruption
- Contact dermatitis / contact dermatitis (pediatric)
- Bullous tinea pedis
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Last Reviewed:07/20/2020
Last Updated:07/28/2020
Last Updated:07/28/2020
Epidermolysis bullosa acquisita in Child
See also in: External and Internal Eye