True about histiocytosis X include the following EXCEPT
Ans. d (Associated with angiolipoma of kidney). (Ref. Nelson Textbook of Pediatrics 17^/1728; TABLE 499-1)Eosinophilic Granuloma (EG)# EG usually occurs during the first 3 decades and is most common in boys 5-10 yr.# The skull is most commonly/ affected, but any bone may be involved.# Usually present with local pain and swelling. There is often tenderness and warmth in the area of bone.# Spinal lesions may cause pain, stiffness, and occasional neurologic symptoms.# X-ray: Lytic lesions have well-defined or irregular margins {geographic skull) with expansion of the bone.# Spine involvement may cause uniform compression or flattening of the vertebral body (vertebra plana).# No extraskeletal (visceral) involvement distinguishes EG from the other forms of Langerhans cell histiocytosis (Hand- Schiiller-Christian or Letterer-Siwe variants), which have a less favorable prognosis.# Treatment includes curettage and bone grafting, low-dose radiation therapy, or steroid injection.# Observation for symptomatic lesions is fine because most lesions heal spontaneously and do not recur.
Seborrheic dermatitis is a known feature
Birbeck's granules are characteristic feature
Lytic bony lesions are known
Associated with angiomyolipoma of kidney
Miscellaneous