ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (12)
Benign cephalic histiocytosis in Infant/Neonate
Print
Other Resources UpToDate PubMed

Benign cephalic histiocytosis in Infant/Neonate

Print Images (12)
Contributors: Chris G. Adigun MD, Jeffrey D. Bernhard MD, Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Benign cephalic histiocytosis (BCH) is a rare non-Langerhans histiocytosis that typically occurs between 2 and 34 months of age and, less commonly, up to the age of 5 years. Approximately 50% of cases begin between the ages of 5 and 12 months. It affects males and females equally. The condition is characterized by asymptomatic, raised, reddish-yellow papules, 2-4 mm in diameter. Papules initially develop on the head in all cases, most often the cheeks, eyelids, forehead, and ears. Lesions may extend to involve the neck and upper trunk. Affected children will always have multiple lesions. Unlike other forms of histiocytoses, mucosa and viscera are not involved.

Involution of lesions occurs spontaneously over the ages of 2-8 years, often with residual postinflammatory hyperpigmentation that persists indefinitely. There have been no reported associated systemic diseases or reports of visceral involvement. Some experts consider BCH to be a childhood variant of generalized eruptive histiocytoma, and some reports suggest that BCH may have overlapping clinical and histologic features with juvenile xanthogranuloma (JXG). There have been reports of BCH transforming later into JXG, further establishing this theoretical overlap. BCH may be difficult to differentiate clinically from JXG, but key differentiating features are that BCH lesions tend to be flatter and are mainly on the head and neck.

Codes

ICD10CM:
D76.3 – Other histiocytosis syndromes

SNOMEDCT:
255192005 – Benign cephalic histiocytosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 11/19/2019
Copyright © 2020 VisualDx®. All rights reserved.
Benign cephalic histiocytosis in Infant/Neonate
Print 12 Images
View all Images (12)
(with subscription)
Benign cephalic histiocytosis : Brown color, Cheek, Face, Orange color, Smooth papule
Clinical image of Benign cephalic histiocytosis
Copyright © 2020 VisualDx®. All rights reserved.