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Rare Presentation of Mucous Membrane Pemphigoid With Pemphigoid of the Pulmonary System in 10-Month-Old Female
Hannah Kovar1,2, Ira Shukla1,3, India Hill1,2
1Children's Hospital New Orleans, New Orleans, Louisiana, USA.
Insights
A rare case of mucous membrane pemphigoid (MMP) and pulmonary system pemphigoid (POPS) in an infant showed initial improvement with immunosuppressants but ultimately succumbed to respiratory failure.
Area of Science:
- Pediatric Dermatology
- Pulmonology
- Immunodermatology
Background:
- Mucous membrane pemphigoid (MMP) and pemphigoid of the pulmonary system (POPS) are rare autoimmune blistering diseases.
- These conditions can affect multiple organ systems, including the skin, mucous membranes, and lungs.
Abstract:
We present a 10-month-old girl with diffuse mucous membrane pemphigoid (MMP) and pemphigoid of the pulmonary system (POPS). Systemic corticosteroids, intravenous immunoglobulin, cyclophosphamide, rituximab, and mycophenolate mofetil led to improvement in cutaneous and mucosal lesions. However, she had progressively worsening airway involvement refractory to immunosuppression and ultimately died from respiratory failure.
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