Rhinoscleroderma Flashcards
RHINOSCLEROMA causative agent
chronic granulomatous disease caused by Gram- negative bacillus called Klebsiella rhinoscleromatis
Klebsiella rhinoscleromatis is also called as
Frisch bacillus
PATHOLOGY
The disease starts in the nose and extends to nasopharynx, oropharynx, larynx
Mode of infection
unknown
Age and Sex predilection
Both sexes of any age may be affected.
CLINICAL FEATURES
Atrophic stage.
Granulomatous stage.
Cicatricial stage.
Atrophic stage resembles
atrophic rhinitis and is char- acterized by foul-smelling purulent nasal discharge and crusting.
Granulomatous stage
nodules form in nasal mucosa. There is also subdermal infiltration of lower part of external nose and upper lip giving a “woody” feel
Nodules are painless and nonulcerative.
Cicatricial stage
This causes stenosis of nares, distortion of upper lip, adhesions in the nose, nasopharynx and oropharynx. There may be subglottic stenosis with respi- ratory distress.
Biopsy shows
infiltration of submucosa with plasma cells, lymphocytes, eosinophils, Mikulicz cells and Russell bod- ies.
diagnostic features of the disease
Mikulicz cells and Russell bodies.
Mikulicz cells
large foam cells with a central nucleus and vacuolated cytoplasm containing caus- ative bacilli
Russell bodies
homogenous eosinophilic inclusion bodies found in the plasma cells
They occur due to accumulation of immunoglobulins secreted by the plasma cells.
TREATMENT
streptomycin (1 g/day) and tetracycline (2 g/day) are given together for a minimum period of 4–6 weeks and repeated, if necessary, after 1 month.
Steroids can be combined to reduce fibrosis
Surgical treatment may be required to establish the airway and correct nasal deformity
Rx is stopped only when
two consecutive cultures from the biopsy material are negative
