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Infantile bullous pemphigoid with "string of pearls sign"
Inês Raposo1, Susana Machado, Rita Sampaio
1Department of Dermatology, Centro Hospitalar do Porto, Portugal. inesraposovs@gmail.com.
Insights
Infantile bullous pemphigoid (BP) is a rare blistering disease in children, presenting with characteristic skin lesions. This case highlights a rare pediatric presentation of bullous pemphigoid and its successful management.
Area of Science:
- Dermatology
- Pediatrics
- Immunology
Background:
- Bullous pemphigoid (BP) is an autoimmune blistering disease typically affecting the elderly.
- Infantile BP is exceptionally rare, posing diagnostic challenges.
- Distinguishing BP from other blistering disorders in infants is crucial.
Observation:
- A 5-month-old infant presented with progressing vesicles and bullae initially on hands and feet.
- Lesions evolved into tense vesicles and bullae in an annular "string of pearls" pattern on the abdomen, face, and neck.
- The clinical presentation mimicked other blistering conditions, necessitating thorough investigation.
Findings:
- Histologic and immunologic examinations confirmed the diagnosis of infantile bullous pemphigoid.
- The patient's presentation mimicked linear IgA bullous dermatosis, emphasizing the importance of differential diagnosis.
- Treatment with oral prednisolone and dapsone achieved disease control.
Implications:
- This case underscores the importance of considering infantile bullous pemphigoid in the differential diagnosis of pediatric blistering diseases.
- Early diagnosis and appropriate management, including corticosteroids and dapsone, can lead to favorable outcomes.
- Further research into the specific triggers and long-term prognosis of infantile BP is warranted.
Abstract:
Bullous pemphigoid (BP) is an immune mediated bullous disease that is manifested by urticarial plaques with superimposed subepidermal blisters and significant pruritus. It is generally found in the elderly, but is rare in the pediatric population. A 5-month-old girl previously diagnosed with hand-foot-mouth disease was examined in our dermatology department owing to vesicles and bullae, initially located to the hands and feet, which progressed with new lesions. Tense vesicles and bullae distributed in an annular string of pearls pattern on the abdomen and facial and cervical regions were noted. Histologic and immunologic findings were consistent with the diagnosis of infantile BP. Disease control was obtained with oral prednisolone and dapsone; the patient was still in clinical remission 6 months after treatment cessation. The differential diagnosis of the clinical presentation of the lesions in our patient is of note, given that this blistering pattern is frequently reported in association with linear IgA bullous dermatosis.