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Related Experiment Video

Updated: Jun 10, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

A congenital, erythematous eruption.

Jason S Ballin¹, Brian P Green, Robin Gehris

¹Naval Branch Health Clinic, Department of Aviation Medicine, Kingsville, Texas, USA. jason.ballin@med.navy.mil

Dermatology Online Journal

|August 3, 2010

Summary

Congenital self-healing Langerhans cell histiocytosis is a rare condition presenting at birth. This case highlights the diagnosis in a newborn with characteristic skin lesions and confirmed Langerhans cell infiltration.

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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

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Published on: December 15, 2011

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Published on: October 12, 2012

Area of Science:

Dermatology
Pediatrics
Pathology

Background:

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare, benign neonatal disorder.
It typically presents with characteristic skin lesions at birth, often resolving spontaneously.

Observation:

A 2-day-old male infant presented with multiple erythematous, scaly papules present since birth.
The lesions were distributed across the scalp, face, trunk, and extremities.
No extracutaneous involvement was noted.

Findings:

Histopathological examination of a skin biopsy revealed numerous Langerhans cells in the papillary dermis.
Immunohistochemical stains for CD1a and Langerin were positive, confirming Langerhans cell presence.
The diagnosis of congenital self-healing Langerhans cell histiocytosis was established.

Implications:

This case underscores the importance of recognizing CSHLCH based on clinical presentation and histopathology.
Early diagnosis facilitates appropriate management and reassurance for families, given the typically self-limiting nature of the condition.
Further research into the pathogenesis and long-term outcomes of CSHLCH is warranted.