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Urticating Hashimoto-Pritzker Langerhans cell histiocytosis
D F Butler1, B D Ranatunge, R P Rapini
1Department of Dermatology, Texas Tech University, Health Sciences Center, Lubbock, Texas 79430, USA.derdfb@ttuhsc.edu
Pediatric Dermatology
|February 24, 2001
Summary
Neonatal red-brown papules can be confusing. This case highlights that a positive Darier sign in infants may indicate Hashimoto-Pritzker Langerhans cell histiocytosis (LCH), not just mastocytosis.
Area of Science:
- Dermatology
- Pediatrics
- Histiocytosis
Background:
- Neonatal red-brown papules pose diagnostic challenges.
- The Darier sign is typically associated with mastocytosis.
- Hashimoto-Pritzker Langerhans cell histiocytosis (LCH) can mimic mastocytosis due to mast cell infiltration.
Observation:
- A case report of an infant presenting with neonatal red-brown papules.
- The infant exhibited a positive Darier sign and acute urticaria.
- The clinical presentation suggested a diagnostic dilemma between mastocytosis and LCH.
Findings:
- The infant was diagnosed with Hashimoto-Pritzker Langerhans cell histiocytosis (LCH).
- The positive Darier sign in this LCH case caused initial diagnostic confusion.
- Histological examination confirmed the presence of mast cells within the LCH infiltrate.
Implications:
- This case broadens the differential diagnosis for neonatal papules with a positive Darier sign.
- Recognizing LCH in infants with a positive Darier sign is crucial for appropriate management.
- Early and accurate diagnosis of neonatal LCH can prevent potential complications.