Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood

Abstract

Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.

Retrospective Study on Autoimmune Blistering Disease in Paediatric Patients

“…BP, which is considered a disease of elderly adults, is rare in children, with fewer than 100 cases reported in the literature . Similarly, BSLE, an infrequent skin manifestation appearing in less than 5% of patients with SLE, has been reported in fewer than 15 children to the best of our knowledge .…”

Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

“… 6 Diagnosis of BP is based on a combination of clinical, histopathological, and immunofluorescence exams, which can also be observed in epidermolysis bullosa acquisita (EBA), another subepidermal bullous disease. 3 However, the IF-salt split technique can be used to differentiate them. In BP, IgG deposits can be found on either the epidermal or the dermal side, rarely on both sides, while EBA deposits only occur on the dermal side.…”

“…In BP, IgG deposits can be found on either the epidermal or the dermal side, rarely on both sides, while EBA deposits only occur on the dermal side. 3 , 7…”

“…The diagnosis of autoimmune blistering diseases is frequently confirmed by histological and immunopathological studies. 3 Here, we report a young patient diagnosed with BP by clinical, histopathological, and immunofluorescence examinations, in which only the anti-BP180 levels were high. Interestingly, autoantibodies proved to be positive for anti-Dsg 1 and 3, and numerous milia appeared during recovery.…”

Acral bullae in an infant

“…Diagnosis is confirmed by immunofluorescence demonstrating linear IgG and/or C3 deposition along the basement membrane. Corticosteroids have shown to be extremely effective with few treatment failures and lesions healing within weeks or months 2 3. Steroid-sparing agents such as dapsone, mycophenolate mofetil and intravenous immunoglobulin have been suggested as second-line therapy for more challenging cases; however, only limited data is available 4.…”