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[Pemphigus vulgaris in pediatrics: A case report]
Ana Laura Casuriaga Lamboglia1, Agustina Minut Gubitosi1, Claudia Guebenlián Bakerdjian1
1Asociación Española, Montevideo, Uruguay.
Insights
Pemphigus vulgaris is a rare autoimmune blistering disease in children. Early diagnosis and treatment, including biopsy and immunofluorescence, are crucial for managing this condition.
Area of Science:
- Pediatric Dermatology
- Autoimmune Blistering Diseases
- Rare Pediatric Conditions
Background:
- Pemphigus vulgaris is a severe autoimmune blistering disease that rarely affects children.
- Timely diagnosis and treatment are essential for improving patient prognosis.
- Understanding its clinical presentation and management in pediatric patients is critical.
Observation:
- A 2-year-old boy presented with generalized blistering dermatosis initially misdiagnosed as bullous impetigo.
- The patient showed no improvement with antibiotics and developed mucosal involvement.
- Histological and direct immunofluorescence studies confirmed pemphigus vulgaris.
Findings:
- Pemphigus vulgaris in children can mimic more common infectious or inflammatory conditions.
- Diagnostic delays can occur due to the disease's rarity and initial presentation.
- Histopathology and direct immunofluorescence are key for accurate diagnosis.
Implications:
- A high index of suspicion is necessary for early pemphigus vulgaris diagnosis in children.
- Dermatological consultation and skin biopsy are vital for confirming the diagnosis.
- Prompt treatment with corticosteroids and immunomodulators can lead to a good clinical response.
Introduction:
pemphigus vulgaris is a serious and infrequent disease in children. Its timely diagnosis and treatment allows modifying its prognosis. The objective is to describe its clinical characteristics, and the diagnostic and therapeutic approach of this uncommon autoimmune blistering disease in children.
Clinical Case:
2-year-old male patient, previously healthy. He initially presented generalized dermatosis with maximum lesion areas at the umbilical region, limbs and genitals; characterized by blisters, some denuded, and of easy bleeding, without mucosal involvement nor fever. Bullous impetigo was diagnosed and topical and systemic antibiotic treatment was started, showing no clinical improvement. He developed extension of the lesions with oral and anal mucosal involvement. The histologic and direct immunofluorescent study of lesions and perilesional skin confirm the diagnosis of pemphigus vulgaris. The patient started treatment with corticosteroids and immunomodulatory agents with good clinical response.
Conclusions:
Due to the similarity with other more prevalent infectious and inflammatory diseases, a high index of suspicion is required in order to avoid delays in the diagnosis and the start of treatment. In patients with blisters with an unexpected clinical evolu tion, it is necessary to conduct a joint evaluation with a dermatologist and to assess the opportunity of performing a biopsy of the lesion and perilesional skin for histological study and direct immunofluo rescence, which will allow diagnostic confirmation.
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