Primary lupus vulgaris of the pharynx (a case report).
Primary tuberculous affection of the pharynx is a very rare disease. Tuberculosis of pharynx like tuberculosis of the larynx is always secondary to active pulmonary tuberculosis. Lupus vulgaris, a variant of tuberculous disease occurs secondarily to nasal and facial lesions. Tuberculous lesions in nose, mouth and pharynx even at the peak period in 1930 only average 0.66 per cent. 4 Here a rare case of primary lupus vulgaris of the pharynx has been reviewed.
A 35-year-old female presented with nasal obstruction and occasional mild throat discomfort of two years duration. There was no history of nasal discharge, epistaxis or any other nasal complaints. She had no history of cough, haemoptysis, loss of weight or evening rise of fever. There was no past history of tuberculosis or contact with tuberculosis. She gave history of right ear discharge with decreased hearing since two days. General and systemic examination of the patient was normal.
The examination of nose on anterior rhinoscopy was uncharacteristic. There was absence of air blast on both the sides.
On examination of throat, soft palate and posterior pharyngeal wall showed irregular nodular surface. The uvula was knob like. (See [Figure - 1]). The soft palate was adherent to the posterior pharyngeal wall without leaving any nasopharyngeal airyway. It was woody to feel and bled slightly on touch. The gag reflex was diminished.
Indirect laryngoscopy was normal with normal vocal cord movements. Posterior rhinoscopy, to examine the nasopharynx, could not be done as the palate was fixed to the posterior pharyngeal wall.
The right car showed central perforation with purulent discharge. There were no palpable neck nodes.
Provisional clinical diagnosis was that of rhinosclerorna.
The haemogram was normal except for ESR of 42 min at the end of 1st hour (Westergren). Blood VDRI, was negative. Radiological examination of the chest and cervical spine was normal. Audiogram in view of chronic otitis media showed right severe mixed loss.
Biopsy wag taken from uvula and posterior pharyngeal wall to confirm the diagnosis of rhinoscleroma. Histophathology report revealed multiple granutomas underneath the stratified squamous epithelium. The granulomas consisted of very little caseation, surrounded by epitheloid cells and Langhan's giant cells with lymphocytes indicating tuberculous inflammation (see [Figure - 2]). The patient was put on a combination chemotheray of rifampicin, isoniazid and ethambutol with vitamin D supplement.
Patient came for follow up six months later. She was free of symptoms except for nasal blocking. The irregular nodular appearance of the posterior pharyngeal wall had changed into normal smooth mucus membrane.
She was advised pharyngoplasty in which adhesions between soft palate and posterior pharyngeal wall were incised and silastic tube passed through nose to prevent restenosis. Patient did well post-operatively and at the time of last follow up, six months after surgery had a good nasopharyngeal airway.
Pharyngeal tuberculosis in isolation is extremely rare; associated with co-existing pulmonary tuberculosis it is more common. Lupus of the pharynx is associated with lupus of the skin of face.
Tuberculosis is seen in pharynx in three forms: acute miliary tuberculosis, chronic tuberculous ulceration and lupus vulgaris.
Acute miliary tuberculosis: This is a rare complication and always secondary to pulmonary tuberculosis. It is associated with spread of tubercle bacilli by the blood stream. There is an eruption of minute pearly-grey tubercles or yellowish spots as in lacunar tonsillitis. The process is an acute one and is always accompanied by marked aggravation of the general symptoms of tuberculosis. In addition there is acute pain radiating to the ear, dysphagia, increased salivation, anorexia, rapid wasting and fever. This classical description of acute pharyngeal tuberculosis mentioned by Thomson and Negus certainly does not fit with our patient.
Chronic ulcerating tuberculosis: Chronic ulcerating tuberculosis is always associated with advanced pulmonary tuberculosis and the sputum is laden with tubercle bacilli. There is ulceration in the pharynx and on the tongue. The ulcers are shallow with undermined edges and relatively clear bases, their progress is indolent. The nerve endings are intact, so there is much pain.
Lupus vulgaris: Lupus of the pharynx is almost invariably secondary to lupus of nose and face. Lupus of pharynx and larynx occurs in 10-20% of the patients with lupus of the skin. Lupus occurs twice as frequently in females as in males. It generally begins before puberty. The favourite site in the upper respiratory tract is at the mucocutaneous junction at anterior end of nasal septum and inferior turbinate, whence the disease may spread to the face or the pyarynx. Deposit takes place in the form of minute, discrete pinhead points, generally pinkish with yellowish apple-jelly like centre. As the deposit increases the surface becomes bosselated, the epithelium is destroyed and when the sticky secretion is wiped away the invaded area shows gelatinous, greyish moulds separated by narrow crevices, suggesting worm-eaten appearance. The lesion may be confused with syphilis. In syphilis, however, the disease runs a much rapid course with greater destruction of tissue and serology is positive. Lupus runs a very chronic course, with tendency to heal in part while activity continues elsewhere; consequently there is considerable scarring involving palate. The uvula may be reduced or may vanish. The anterior pillar of fauces and adjoining soft palate may loose elasticity and become insensitive and wooden.
Though the symptoms are generally very slight and often amount to no more than a sense of discomfort in the throat, the danger lies in the disease extending to the larynx and eventually leading to pulmonary tuberculosis.
In view of the above description of lupus, considering the clinical behaviour. of patient's symptoms and signs, it appears that the patient had a rare variety of primary lupus of pharynx.
We thank Dr GB Parulkar, former Dean, Seth GS Medical College and KEM Hospital, Parel, Mumbai, for permitting us to publish this case report.