Rare Cause of CSOM: Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a proliferation of dendritic mononuclear cells with infiltration into organs locally or diffusely. Most cases occur in children. LCH can also present as chronic otitis media and otitis externa due to involvement of the mastoid and petrous portions of the temporal bone with partial obstruction of the auditory canal. A 4 year old male child presented with complaints of bilateral ear discharge for 4 months and inability to walk and giddiness for 2 days. On otoscopic examination, in right ear, polypoidal tissue was present in the external auditory canal which bled on touch. CECT head and MRI brain was done which showed large altered intensities in the region of bilateral external and middle ears involving the temporal bone. It also showed a well defined ovoid expansile lesion involving the skull vault in left high parietal region. Biopsy was taken from the polypoidal tissue in the right EAC which on HPE showed features suspicious for LCH. On IHC, the tissue was found out to be immunoreactive for CD 68, CD 1a and S-100 with score of 4+ for all three of the IHC markers. Patient was later put on chemotherapy and steroids which resulted in disease remission.

Keywords: Bone; Histiocytosis; Media; Otitis; Temporal.

Fig. 1

X-ray skull showing lytic lesion in skull bone

Fig. 2

HRCT Bilateral Temporal Boneshowing multiple lytic lesions with destruction of squamous and mastoid part of both temporal bones

Fig. 3

Multiple Large Polygonal cells (Langerhan’s cells)with ample cytoplasm, nuclei are round, oval or bean shaped with prominent longitudinal grooves.Immunohistochemistry revealed. (a) Immunoreactivity for CD 68. (b) S-100 protein. (c) Presence of CD1a Antigen