Epidermolysis Bullosa Acquisita in an 8‐year‐old Girl

Borok, Michael; Heng, Madeline C. Y.; Ahmed, A. Razzaque

doi:10.1111/j.1525-1470.1986.tb00532.x

Cited by 28 publications

(20 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Indirect immunofluorescence on salt‐split skin showed IgG deposits at the dermal side of the cleavage favoring the diagnosis of EBA and a common finding in all 30 EBA cases already reported (4–20).…”

Section: Discussionsupporting

confidence: 57%

See 1 more Smart Citation

Epidermolysis Bullosa Acquisita in Childhood

Trigo-Guzmán

Conti

Aoki

et al. 2003

The Journal of Dermatology

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 57%

“…Mucosal involvement, especially of the oral cavity, is observed in childhood EBA (4–6, 8, 10, 12–16). Sixteen out of the 30 cases reported in children have presented with oral lesions; the genital area was affected in seven, and ocular involvement leading to blindness was present in one case (13).…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis Bullosa Acquisita in Childhood

Trigo-Guzmán

Conti

Aoki

et al. 2003

The Journal of Dermatology

View full text Add to dashboard Cite

show abstract

“…The classical type presents with marked skin fragility, blisters and erosions at sites of trauma, and heals with scarring and milia. The inflammatory type can mimic almost all other chronic bullous diseases, and its clinical differentiation from BP, cicatricial pemphigoid and linear IgA bullous dermatosis may be difficult 4–6,9–26 . In some patients, characteristics of both classical and inflammatory phenotypes of EBA have been observed, 9 but most patients with EBA in adulthood appear to have the classical type of EBA.…”

Section: Discussionmentioning

confidence: 99%

Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type VII collagen: case report and review of the literature

Mayuzumi

Akiyama

Nishie

et al. 2006

British Journal of Dermatology

View full text Add to dashboard Cite

SummaryEpidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies to type VII collagen, a major component of anchoring fibrils. Most patients with EBA are adult and develop autoantibodies to the non-collagenous 1 (NC-1) domain of type VII collagen．In this report, we describe a 4-year-old Japanese boy presenting pruritic vesicles and tense blisters over his whole body.Immunofluorescence studies revealed linear IgG/C3 deposits along the dermal-epidermal junction of the patient's skin, and circulating IgG autoantibodies mapping to the dermal side of 1M NaCl-split skin. By immunoblotting analysis using dermal extracts as a substrate, the patient's IgG antibodies labeled a 290 kD protein corresponding to type VII collagen.Immunoblotting studies using recombinant proteins demonstrated that the patient's circulating autoantibodies recognized not only the NC-1 but also the non-collagenous 2 (NC-2) domain of type VII procollagen.Review of the previously reported cases and the present case suggested that EBA cases with autoantibodies to regions other than the NC-1 domain are all children younger than 10 years of age with clinical features of an inflammatory phenotype.2

show abstract

“…[3][4][5][6][7][8] Epidermolysis bullosa acquisita in childhood 39 The age of onset ranges from 3 months to 12 years. Of 13 cases, there were nine girls and four boys.…”

Section: Discussionmentioning

confidence: 99%

“…Of 13 cases, there were nine girls and four boys. [3][4][5][6][7][8][9] Delay in diagnosis and treatment has been a problem because of incorrect assessment. Clinical subdivisions of epidermolysis bullosa acquisita have been based on adult patterns, although children seem to have more inflammatory disease.…”

Section: Discussionmentioning

confidence: 99%

Epidermolysis bullosa acquisita in childhood

Varigos

Dowling

1998

Aust J Dermatology

View full text Add to dashboard Cite

This case report of an 11-year-old girl describes a juvenile form of epidermolysis bullosa acquisita, an autoimmune disease of IgG antibodies to basement membrane type 7 collagen. Our case illustrates an unusually severe, acute inflammatory presentation of this condition with prominent mucosal and constitutional features requiring admission to a paediatric burns unit. The treatment consisted of supportive topical and systemic agents, prednisolone and dapsone. She responded to dapsone alone and the course of the illness was uneventful.

show abstract

Epidermolysis Bullosa Acquisita in an 8‐year‐old Girl

Cited by 28 publications

References 25 publications

Epidermolysis Bullosa Acquisita in Childhood

Epidermolysis Bullosa Acquisita in Childhood

Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type VII collagen: case report and review of the literature

Epidermolysis bullosa acquisita in childhood

Contact Info

Product

Resources

About