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Benign cephalic histiocytosis: report of four cases
M L de Luna1, I Glikin, J Golberg
1Section of Pediatric Dermatology, Hospital Ramos Mejía, Ceni, Argentina.
Insights
Benign cephalic histiocytosis is a rare, self-healing skin condition in children. This study describes four cases, noting its characteristic presentation and S100 protein-negative histiocytic infiltrate, confirming it requires no treatment.
Area of Science:
- Dermatology
- Pediatric Pathology
- Histiocytosis
Background:
- Benign cephalic histiocytosis is a rare, self-limiting cutaneous disorder affecting children.
- It presents as papules and erythematous macules primarily on the head.
- Distinguishing it from other histiocytic disorders is crucial for appropriate management.
Purpose of the Study:
- To describe the clinical and histopathological features of benign cephalic histiocytosis.
- To confirm its non-X, non-lipid nature.
- To emphasize its benign and self-resolving course.
Main Methods:
- Case study of four pediatric patients diagnosed with benign cephalic histiocytosis.
- Clinical observation of lesion presentation and progression.
- Skin biopsy with histopathological examination, including S100 protein immunoperoxidase staining.
- Electron microscopy in one case.
Main Results:
- Onset between 5 to 9 months of age.
- Lesions predominantly on the head (cheeks), with truncal and limb involvement in some.
- Histopathology revealed S100 protein-negative histiocytic infiltrate in the superficial dermis.
- Electron microscopy showed comma-shaped bodies and desmosomelike junctions, but no Birbeck's granules.
Conclusions:
- Benign cephalic histiocytosis is a distinct entity characterized by specific histopathological findings.
- The condition is self-limiting and requires no specific therapeutic intervention.
- Accurate diagnosis prevents unnecessary treatment and reassures parents.
Abstract:
We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients. Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present. Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.
