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Eosinophilic granuloma, Langerhans cell histiocytosis (LCH), Hennebert's symptom

Eosinophilic granuloma

Eosinophilic granuloma – is a phase of granulomatous Hand–Schüller–Christian disease. Some authors consider that eosinophilic granuloma is a tumor. Eosinophilic granuloma is a relatively rare disease. Eosinophilic granuloma occurs at any age, but predominantly in children and young persons.

Langerhans cell histiocytosis (LCH)) changed several names until it was renamed in 1985 by the Histiocyte Society: Hand–Schüller–Christian disease, Abt-Letterer-Siwe disease and histiocytosis X.

Skull CT reveals bone lesions with classical for eosinophilic graunuloma bevelled edges.

Eosinophilic granuloma occurs as a focal ossifluent (dissolution of bone) in the process of bone with preferred localization in the cranial vault (usually frontal bone), ribs, long bones, pelvis. Temporal bone alone or with other centers - a favorite location eosinophilic granuloma.

With a single lesion of eosinophilic granuloma good prognosis (possible fibrosis with spontaneous cure), but the observed shift in malignant histiocytosis. When multiple lesions eosinophilic granuloma forecast bad.

Course of the disease eosinophilic granuloma – a case of chronic purulent otitis media (suppuration of the ear, granulation or polyp in the external ear canal, sometimes pain in the ear, often paresis of the facial nerve). There is a swelling behind the ear, or (more rarely) anterior and upward, rarely – fistula.

When infiltrative lesions of the capsule, endoostium maze - the patient has symptoms of inner ear lesions. X-ray examination revealed bone defect without reactive sclerosis in the mastoid or more of the temporal bone. Sometimes the bone defect is palpated through the swollen tissue. With the spread of eosinophilic granuloma possible degradation (destruction) of the entire petrous with symptoms of cranial nerves.

T1 weighted post-contrast coronal MRI slice demonstrate eosinophilic granuloma as an enhancing mass in the superotemporal aspect of the left orbit.

Eosinophilic granuloma diagnosis

The diagnosis of eosinophilic granuloma pose with a biopsy (from the mastoid process). Histological examination of eosinophilic granuloma is stated reticular granulation tissue with marked infiltration of eosinophilic granulocytes, and in some places – Sternberg giant cells, foci of necrosis.

In the diagnosis of eosinophilic granuloma from the modern imaging techniques in addition to the a biopsy of the affected tissue and radiography are used MRI or CT scan of the skull bones. Tomography allows to specify the amount of eosinophilic granulomas and the spatial arrangement of the bone lesion with adjacent soft tissues (blood vessels, nerves, etc.).

Atypical nuclei are indicated by arrows on biopsied tissue - an infiltrate of neutrophils, histiocytes with somewhat atypical nuclear features, multinucleated giant cells, and occasional eosinophils (deep red nuclei) with focal areas of necrosis, consistent with eosinophilic granuloma.

 

Eosinophilic granuloma treatment

Treatment of eosinophilic granuloma is performing by surgery, during which usually shows a yellow-brown mass with an exposure of the dura mater, sigmoid sinus, facial nerve. After surgery, radiotherapy (a total dose of 40 Gy (1 rad-0, 01 Gy)) and treatment with corticosteroids.

 

Hennebert's symptom

For the first time Hennebert's symptom described in an intact eardrum in patients with congenital syphilis. Hennebert's fistular symptom detected during the pressor test (pressure on the tragus).

Hennebert's symptom occurs in patients with episodic vertigo is not syphilitic etiology. The proposed formation mechanism of Hennebert's fistular symptom – it vestibulofibrosis or adhesions between the inner surface of the plate of stapes and membranous labyrinth.