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Association of juvenile xanthogranuloma with café-au-lait macules
G P Thami1, S Kaur, A J Kanwar
1Department of Dermatology and Venereology, Government Medical College and Hospital, Sector 32, Chandigarh-160047, India.
Insights
This case study details a 2-year-old boy with juvenile xanthogranuloma, presenting with yellowish-brown facial lesions and café-au-lait spots. Biopsy confirmed the benign, self-limiting nature of this rare pediatric skin condition.
Area of Science:
- Dermatology
- Pediatrics
- Histopathology
Background:
- Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic disorder typically affecting infants and young children.
- JXG commonly presents with cutaneous lesions, but can rarely involve extracutaneous sites.
- Early diagnosis and management are crucial for appropriate patient care and parental counseling.
Observation:
- A 2-year-old boy presented with yellowish-brown papules on the face and asymptomatic café-au-lait macules on the trunk.
- Physical and developmental milestones were normal, with no history of seizures or family history of similar lesions.
- Skin biopsy revealed a dermal infiltrate of lymphocytes, eosinophils, foamy histiocytes, Touton giant cells, and fibroblasts, confirming JXG.
Findings:
- Histopathological examination of the skin biopsy confirmed the diagnosis of juvenile xanthogranuloma.
- Laboratory investigations including complete blood counts and liver/renal function tests were within normal limits.
- The patient's cutaneous lesions were characteristic of JXG, with no signs of neurofibromatosis.
Implications:
- Juvenile xanthogranuloma is a benign and self-limiting condition, requiring regular follow-up for monitoring.
- Accurate histopathological diagnosis is essential to differentiate JXG from other pediatric dermatoses.
- Understanding the benign nature of JXG reassures parents and guides clinical management strategies.
Abstract:
A 2-year-old boy was referred to the dermatology services for the evaluation of yellowish-brown raised lesions over the face of 3 months' duration. In addition, he had multiple asymptomatic hyperpigmented spots over the trunk, which his parents had noticed at the time of birth. His physical and mental milestones of development were normal. Apart from the cutaneous lesions, the child was otherwise well and there was no history of seizures. He was the only child born to nonconsanguineous parents. No family history of similar lesions was available. The boy weighed 14 kg, had a height of 88 cm, and a head circumference of 48 cm. General physical and systemic examination was normal. Cutaneous examination revealed multiple, yellowish-brown papules over the forehead, sides of the face, and shoulders. The papules measured 0.5-0.8 cm in diameter, were discrete, well defined, oval and flat topped without any surface changes (Fig. 1). Multiple café-au-lait macules varying from 0.5 to 3.0 cm in diameter were present over the trunk (Fig. 2). There was no freckling in the axillary or the inguinal regions and no neurofibromas were present. A diagnosis of juvenile xanthogranuloma was considered. Complete blood counts, urine analysis, hepatic and renal function tests, and serum lipid levels were normal. A skin biopsy from a papule over the shoulder revealed a dense infiltrate in the dermis underlying a normal epidermis. The infiltrate was well demarcated and comprised lymphocytes, eosinophils, and foamy histiocytes along with Touton giant cells and proliferating fibroblasts. This confirmed the diagnosis of juvenile xanthogranuloma. The parents were informed about the benign and self-limiting nature of the disorder and were advised to bring the child for regular follow-up.