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Pathology Forum
January 2001

Quiz Case 1

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2001;127(1):78. doi:10.1001/archotol.127.1.78

A PREVIOUSLY healthy 3-year-old boy was referred to the outpatient clinic with a 2-month history of painless otorrhea of his left ear and, according to his parents, decreased hearing. The patient did not suffer from recurrent otitis media, nor was he ill in the period prior to his admission. His medical history was uneventful apart from premature labor and failure to thrive. Physical examination revealed a left aural polyp obliterating the external canal, accompanied by a malodorous purulent discharge. The right tympanic membrane appeared normal, but there was sagging of the right posterior canal wall. Multiple aphthae were noted on the soft palate, conjunctivae, and eyelids. Similar moluscumlike lesions were scattered throughout the trunk and face. The audiogram showed bilateral conductive hearing loss, more prominent in the left ear. High-resolution computed tomographic scans of the temporal bones (Figure 1 and Figure 2) revealed extensive bilateral destructive lesions, and a computed tomographic scan of the chest and abdomen demonstrated disseminated bilateral interstitial lung lesions and hypodense areas in hepatic parenchyma. Tissue for histologic diagnosis, which was obtained from the left mastoid through a lateral temporal bone resection and conjunctival biopsy, demonstrated a proliferation of histiocytes, accompanied by giant cells and eosinophils (Figure 3). Electron microscopy revealed pathognomonic findings (Figure 4).

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