A child presents with seborrhoeic dermatitis, lytic skull lesions, ear infection and hepatosplenomegaly. The most likely diagnosis is (AIIMS May 2014)
1. HistiocytosisX, constitutes class I and includes the clinical entities of eosinophilic granuloma, Hand Schuller-Christian disease and Letterer-siwe disease.
2. The name Langerhans’ cell histiocytosis (LCH) has been applied to the class I histiocytosis Class I histiocytosis
3. The most common involvement is of the skeleton (80%), the most common sites skull bones, long bones, spinal vertebrae, mastoid and mandible presents with painless or presents with pain and swelling.
4. X-ray shows sharply defined lytic lesions with non-healing borders
5. Clinical manifestation includes, vertebral collapse, and spinal compression, pathological fracture in long bones, chronic draining ears and early eruption of teeth.
Other manifestations includes, seborrheic skin rash in scalp and back (58%) lymphadenopathy (33%) hepatosplenomegaly (20%) and parenchymal infiltration of lungs (15%)