Rare Cases
Kimura disease
● Introduction:
➣ Originally identified as "eosinophilic hyperplastic lymphogranuloma" in 1937, Kimura disease (KD) was formally recognised as Kimura's disease in 1948. With a male-to-female ratio of 3:1, it is uncommon outside of Asia and most commonly observed in adolesc
Pneumolabyrinth
● Introduction:
• Definition: Air in the vestibule, semicircular canals, or cochlea.
• Taken into account radiographic evidence of a perilymphatic fistula (PLF).
•PLF = improper connection between the inner ear and the air-filled middle ear → perilymph leakage.
•
Laryngocele
●Introduction:
• Unlike other neck cysts, this one communicates with the laryngeal lumen and is uncommon, benign, and filled with air or fluid.
• Larrey (1829) described it, and Virchow (1867) gave it a name.
• The annual incidence is about 1 in 2.5 million.
• The M
Lacrimal sac rhinosporidiosis
INTRODUCTION
The Rhinosporidium seeberi epidemic in South India is the cause of the chronic mucocutaneous granulomatous illness known as rhinosporidiosis.
Originally categorised as a fungus, it is currently included within the Mesomycetozoa clade, which includes aquatic proti
Tolosa-Hunt Syndrome
Introduction
An uncommon idiopathic granulomatous inflammatory condition that might impact the orbit, superior orbital fissure, or cavernous sinus.
Important clinical characteristic: Ophthalmoplegia with severe unilateral orbital discomfort (CN III, IV, VI).
Eduardo Tolosa
Behçet’s Disease
● Introduction:
➣ The traditional triad—oral aphthae + genital ulcers + uveitis—was initially defined in 1937 by Turkish dermatologist Hulusi Behçet. Historical note: ➣ Hippocrates' works from the fifth century BC contained comparable descriptions.
➣ Epidemiolog
Relapsing Polychondritis
● Overview:
➣ Relapsing polychondritis (RP) is an autoimmune disease that affects cartilaginous and proteoglycan-rich tissues. It is uncommon, severe, episodic, and progressive.
Ears, nose, larynx, trachea, bronchi, blood vessels, heart, eyes, kidneys, and joints are fre
Congenital Nasal Pyriform Aperture Stenosis (CNPAS):
● Overview:
• CNPAS, a very rare cause of nasal obstruction in infants, is caused by bony overgrowth of the maxillary nasal processes.
• Because babies must breathe via their nostrils, clinical signs include apnoea, respiratory discomfort, recurrent cyanosis, and feeding
Cogan’s syndrome
● Definition: Cogan's syndrome (CS) is a rare, suspected autoimmune condition that is typified by vestibuloauditory symptoms (tinnitus, vertigo, and hearing loss) and inflammatory ocular illness (traditionally non-syphilitic interstitial keratitis, IK).
●Epidemiology:
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langerhans cell histiocytosis of the mandible
● Case Example:
The left cheek of a 4-year-old child is uncomfortably swollen.
● Clinical examination:
• Diffuse left mandibular oedema;
• Pliable and painful submandibular lymph nodes;
• Limited mouth opening (trismus, ~20 mm);
• No oral abnormalities