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Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid
Published on: June 17, 2025
P11 Prolonged diagnostic journey in infantile bullous pemphigoid
Pratima Poudel1, Celia Moss1, Malobi Ogboli1
1Birmingham Children's Hospital, Birmingham, UK.
Insights
Delayed diagnosis of infantile bullous pemphigoid (IBP) can occur due to its rarity. Prompt referral with photographs to pediatric dermatologists is crucial for accurate diagnosis and effective treatment of this rare blistering disease.
Area of Science:
- Pediatric Dermatology
- Immunodermatology
- Rare Pediatric Diseases
Background:
- Infantile bullous pemphigoid (IBP) typically presents in early infancy (3-5 months) and usually responds well to treatment.
- Misdiagnosis is common due to the rarity of IBP, often leading to delayed diagnosis and treatment, as exemplified by a recent case.
- The condition is frequently mistaken for more common childhood infections like hand, foot, and mouth disease.
Purpose of the Study:
- To highlight the diagnostic challenges and delays associated with infantile bullous pemphigoid (IBP).
- To emphasize the importance of prompt referral to pediatric dermatologists for unusual infantile rashes.
- To underscore the value of clinical photography in facilitating remote specialist assessment.
Main Methods:
- Case report of a 4-month-old female infant presenting with widespread tense vesicles, bullae, and erythema.
- Diagnostic confirmation through clinical examination, histology, and direct immunofluorescence.
- Therapeutic intervention using a combination of erythromycin and oral prednisolone.
Main Results:
- The patient's clinical diagnosis of infantile bullous pemphigoid was confirmed.
- Treatment with erythromycin and prednisolone resulted in rapid improvement within two weeks.
- Complete resolution of lesions was achieved, with steroid discontinuation after eight weeks and no relapse.
Conclusions:
- Infantile bullous pemphigoid, though rare, requires prompt recognition and management by healthcare professionals.
- General practitioners and pediatricians should consider referring infants with unusual bullous eruptions for specialist dermatological evaluation.
- Utilizing clinical photographs can aid pediatric dermatologists in early diagnosis, even in remote consultations.
Abstract:
Infantile bullous pemphigoid (IBP) usually presents at 3-5 months of age and resolves rapidly with appropriate therapy. Unfortunately, diagnosis and effective treatment are often delayed as in the case reported here. The paediatric dermatology team was called to the emergency department to see a 4-month-old girl with widespread tense vesicles, bullae and patchy erythema. The clinical diagnosis of IBP was confirmed by histology and direct immunofluorescence, and she was treated with erythromycin and prednisolone 0.5 mg/kg/day. Within 2 weeks all lesions were drying up and the steroid was discontinued after 8 weeks with no relapse. This child had first presented to the GP more than 3 weeks previously and as well as 3 GP visits had an admission to a paediatric ward with suspected hand, foot and mouth disease. Photographs were sent to the local dermatology team who advised treatment with flucloxacillin and acyclovir. She returned 2 days after discharge with continuing bullous lesions only to be reassured that it would settle in time. Three days later the GP referred her to Urgent Care and thence to our service. Although the diagnosis is straightforward for paediatric dermatologists, the rarity of IBP means that GPs and others may fail to recognize it and favor more common infectious diagnoses. It is likely that a paediatric dermatologist would recognize immune bullous disease even based on photographs. Faced with an unusual infantile rash, GPs and paediatricians should be encouraged to refer promptly, with photographs, to paediatric dermatologists who need to accommodate such referrals.
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