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Novel Clinical Observations on Benign Cephalic Histiocytosis in a Large Series
Algün Polat Ekinci1, Nesimi Buyukbabani2, Can Baykal1
1Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Insights
Benign cephalic histiocytosis (BCH) is an underrecognized infant skin condition. This study found BCH often presents beyond the head and neck and typically resolves with time.
Area of Science:
- Pediatric Dermatology
- Histiocytosis
- Rare Diseases
Background:
- Benign cephalic histiocytosis (BCH) is a rare, self-healing non-Langerhans cell histiocytosis.
- It primarily affects infants and young children, with lesions typically on the head and neck.
- Fewer than 60 cases have been reported in English literature.
Purpose of the Study:
- To evaluate the clinical features and follow-up data of 11 patients diagnosed with BCH.
- To assess the presentation and natural course of this rare condition.
- To reevaluate the nomenclature of BCH based on observed extracephalic involvement.
Main Methods:
- Retrospective study of 11 BCH patients.
- Data collected between 2004 and 2016.
- Evaluation of clinical presentation and treatment outcomes.
Main Results:
- Median age at onset was 8 months; lesions often appeared on the face (10/11 patients).
- Extracephalic involvement (trunk, extremities) was noted in 81.8% of patients.
- Two distinct clinical presentations observed: dome-shaped papules (n=5) and flat papules (n=6).
- Seven patients with a 5-year follow-up showed significant resolution or regression without treatment.
Conclusions:
- BCH is likely underrecognized, with this study adding 11 new cases.
- Clinical presentation is variable, and extracephalic involvement is common.
- The term "cephalic" may be inaccurate given the frequent involvement beyond the head and neck.
Background/Objectives:
Benign cephalic histiocytosis (BCH) is a rare form of non-Langerhans cell histiocytosis in infants and young children characterized by self-healing macules and papules occurring primarily in the head and neck region. So far there have been nearly 60 reported cases in the English-language literature.
Methods:
In this retrospective study, we evaluated clinical features and follow-up data of 11 patients diagnosed with BCH between 2004 and 2016 in the Department of Dermatovenereology, Istanbul Medical Faculty.
Results:
There were 5 girls and 6 boys (median age 24 months, range 9-72 months). The median age at the onset of lesions was 8 months (range 3-36 months). The lesions first appeared on the face in 10 patients and on the trunk in 1. Proximal parts of the extremities and trunk were also involved in nine patients (81.8%). Patients were categorized into two groups based on their clinical features; five had 20 to 30 predominantly red-brown dome-shaped papules and six had 50 to hundreds of yellow-brown or predominantly pinkish brown flat papules. Four patients were lost to follow-up. In seven patients with a mean follow-up of 5 years, four had nearly complete resolution and three showed remarkable regression without treatment.
Conclusion:
With 11 additional cases from a single center, BCH seems to be an underrecognized disease. Its clinical presentation is not uniform. Considering that most of the patients in this series and those previously reported had extracephalic involvement, the term "cephalic" needs to be reevaluated.
