Childhood bullous pemphigoid: a case report and 10‐year follow up

Abstract

Bullous pemphigoid (BP) is a very rare acquired immunbullous disease in children and infants. We report the case of a 15-month-old boy referred to our service with multiple tense bullae located predominantly on the trunk, neck and proximal portion of the limbs. Palms, soles and oral mucosa were also affected. Histopathologic and immunopathologic features were characterized with bullous pemphigoid. The patient responded well to systemic steroids, with improvement of his condition. After a 10-year follow up the patient was no longer suffering from the disease.

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

“…Vesicles and blisters arise on erythematous or apparently healthy skin combined with pruriginous, erythematous infiltrated papules and plaques with irregular borders, annular and polycyclic configurations (sometimes similar to erythema multiforme), which may precede or coexist with blisters. 1,2,4,6,11,12,13 Blisters are tight, with variable size, usually symmetrical and ungrouped, with serous and / or hemorrhagic contents. 2 They are located primarily on flexural areas of the limbs, trunk and abdomen.…”

“…Face, head and neck are often affected. 1,2,6,8,11,12,13 Itching is characteristic. Nikolsky sign is negative.…”

“…1 Diagnosis is based on clinical, histopathological and immunofluorescence exams, particularly DIF (direct immunofluorescence) and salt-split skin technique, traditionally used to differentiate BP from other immune subepidermal bullous diseases. 1,2,6,11,14 …”

“…5,7,8 However, some peculiarities are present in childhood BP, such as preferential involvement of palmoplantar regions, oral and genital mucosae and face. 3,4,7,8,11,13,15 Bullous pemphigoid during childhood has a benign, self-limited evolution, with good response to treatment with corticosteroids, lower recurrence rates and better prognosis when compared to the adult form. 8,13 …”

Bullous eruption in a five-month-old girl

“…The response to treatment is rapid and ranges from a few days to several months; relapses are very rare. 12 Secondary cutaneous infections or sepsis may occur, especially in children with widespread involvement. Complications related to the use of systemic or extensive local corticosteroids are also possible.…”