Epidermolysis Bullosa Acquisita in Childhood

Abstract

Epidermolysis bullosa acquisita (EBA) is a subepidermal autoimmune blistering disease that is rarely reported in childhood. We describe a nine-month-old mulatto boy presenting with multiple, annular, widespread, tense blisters and oral lesions. The diagnosis of EBA was confirmed by histopathology, immunofluorescence, and immunoblotting analysis. The patient was successfully treated with systemic steroids (prednisone) and dapsone. After 20 months of initial treatment, clinical remission was observed, and dapsone remains as the current treatment. This case report emphasizes the rarity of EBA in childhood and the difficulties in reaching the final diagnosis.

In utero development of epidermolysis bullosa acquisita

“…A chronic blistering disease, EBA results from circulating antibodies to the carboxyl terminus of type VII collagen . EBA has rarely been reported in childhood . Reviews of the disease in the pediatric population suggest that mucosal involvement and an inflammatory phenotype, such as in our patient, are common …”

“…EBA has rarely been reported in childhood . Reviews of the disease in the pediatric population suggest that mucosal involvement and an inflammatory phenotype, such as in our patient, are common …”

Childhood Bullous Pemphigoid: Report of Three Cases

“…LABD is the most common subepidermal autoimmune blistering disease in infants, and shows typical vesicobullous eruptions, arranged in an annular and polycyclic pattern, and located on the trunk, neck, face, scalp, flexural and periorificial areas. EBA is also a rare disease in childhood (34), where blisters usually occur at sites of mechanotraumas, leaving scarring and milia formation; however, a clinical BP‐like form may occur, making its differential diagnosis with BP much more difficult.…”

“…(20). Despite being rare in BP, these dermal deposits of IgG at the BMZ using salt‐split technique are a common feature of EBA and BSLE (34). Although EBA and BSLE have a common autoantigen, (collagen VII), BSLE patients often have positive antinuclear antibodies.…”

Epidermolysis bullosa acquisita in a great Dane

“…Epidermolysis bullosa acquisita (EBA) is a rare, autoimmune, blistering skin disease that was first reported in human beings in 1895 (Megahed and Scharffetter‐Kochanek 1994, Hallel‐Halevy and others 2001). Although it typically affects adults, it can occur in children (Trigo‐Guzmán and others 2003). In its classic form, affected patients suffer from a non‐inflammatory mechanobullous disease, resulting in fragile skin and blister formation at the sites of trauma (Kolanko and others 2003).…”