Juvenile Xanthogranuloma of the Nose

Abstract

Juvenile xanthogranuloma is a type of non-Langerhans cell histiocytosis that occurs most frequently in infants and children. It is usually asymptomatic and can present as either a cutaneous or extracutaneous lesion. The present case is believed to be the first reported in the English literature of juvenile xanthogranuloma presenting as an ulcerated bleeding lesion on the dorsum of the nose.

SkIndia quiz 35: A girl with multiple papules

“…[3] They stain strongly with histiocytic marker such as CD 68 and are negative for S-100 as was observed in this case [Figure 3]. [2]…”

“…It is a benign regressing or stabilizing disorder, occurring in infancy frequently occurring in the head and neck area. [2] In 5% of patients, JXG involves extracutaneous areas, with a high tendency to affect the ocular region. Other sites reported are the pericardium, liver, lung, kidneys, ovary, and testes.…”

“…Other sites reported are the pericardium, liver, lung, kidneys, ovary, and testes. [2] JXG is associated with other diseases including neurofibromatosis type 1 and juvenile chronic myelogenous leukemia. [3]…”

“…[2] The basic histopathology of the non-LCH shows well-circumscribed nodules with dense infiltrates of histiocytes. Those that involve the skin usually infiltrate the dermis.…”

“…Touton giant cells seen in 85% of cases are characterized by a wreath of nuclei around a homogeneous eosinophilic cytoplasmic center, while the periphery shows prominent xanthomatization. [2]…”