HAEM5:Juvenile xanthogranuloma: Difference between revisions

Juvenile Xanthogranuloma is a rare histiocytic neoplasm comprising about 0.5% of all pediatric tumors, seldom seen in in adults. 20-35% cases are congenital, shows male predilection and mostly (>70% cases) arise during the first year of life.

JXG lesions are generally asymptomatic; their appearance is typically different in adult and paediatric settings. Infants may present with ≥1 cutaneous, pale yellow-tan, dome-shaped papulonodular lesions. Approximately 5% of patients present with multiple lesions. Typically lesions begin as raised, pink to dark-brown lesions that may become less elevated over time. Spontaneous resolution of some lesions, leaving residual scarring or wrinkling, may occur after months or years. A clinical subtype of JXG called benign cephalic histiocytosis occurs in head and neck of young children, asymptomatic, self-healing papular lesions. The lesions are often large, solitary and persistent in adults; in this context Erdheim–Chester disease is an important differential diagnosis. JXG may occur in patients with neurofibromatosis type 1 and is also reported in Wiskott–Aldrich syndrome.   

|Asymptomatic in the beginning

|Abnormal blood count, liver enzymes, metabolic tests

Cytopenia if bone marrow involved