Granulomatous ulcers of the nose and oropharynx: Lupus vulgaris revisited.
Head and Neck Clinic
A 48-year-old farmer was referred to the otolaryngology clinic for consultation regarding the ulcers over his nose and oropharynx. The nasal ulcer had first been noticed 4 years earlier, and the oropharyngeal ulcer 3 months earlier. Eight years earlier, the patient had been noncompliant in following an antituberculosis drug regimen, and he had active pulmonary tuberculosis.
The nasal lesion had first manifested as a few coalescent reddish patches that softened and eroded over time. The erosion left a small ulcer, which gradually increased in size until it caused a significant deformity (figure 1, A). Apple-jelly-like nodules were absent on diascopy. Examination of the oropharynx revealed that the right tonsillar fossa had a deep ulcer with prominent edges, and that it was covered with necrotic debris (figure 1, B). The patient had no palpable cervical nodes, and he was nonreactive to testing for human immunodeficiency virus (HIV).
Biopsies of both ulcers identified noncaseating granulomatous lesions without any features of vasculitis, necrosis, or dysplasia (figure 2). No acid-fast bacilli were found. Chest x-ray detected noncavitating patchy opacities bilaterally in the middle and upper zones that were suggestive of tuberculous pneumonia (figure 3); no circumscribed lesion or significant hilar lymphadenopathy was noted. Findings on examination of the eyes (cornea and conjunctiva), urinalysis (for hypercalciuria, proteinuria, and hematuria/red cell casts), and ultrasonography of the renal system were unremarkable. The serum angiotensin-converting enzyme level was not elevated, and the urea and creatinine levels were normal.
The patient was treated with antituberculosis drugs ("previously treated" category), which resulted in satisfactory healing of the nasal and tonsillar lesions (figure 4), as well as the pulmonary lesions.
The clinicohistologic spectrum of our patient's ulcer is a classic illustration of the ulcerative form of mucocutaneous lupus vulgaris (LV) owing to a reactivation of pulmonary tuberculosis. LV manifests as a postprimary chronic granulomatous ulcer in patients with moderate to good immunity. As demonstrated in our case, there could be clinically evident latent or reactivated tubercular foci in the lymph nodes, mucosa, lungs, and joints that might serve as the primary or source point for hematogenous spread resulting in LV. (1)
In a less common scenario, lupus might also be attributable to direct inoculation from an exogenous source in abraded skin or over a vaccination scar (bacillus Calmette-Guerin). (1) However, lupus associated with florid noncavitating pulmonary lesions, as seen in our patient, might also be encountered during periods of a transient waning in host immunity. Moreover, coexistence of cutaneous and mucosal lupus is seldom seen, which demonstrates the dual pathology of hematogenous spread and inoculation.
Mucosal lupus is rare, (1) and hence our patient's oropharyngeal lesion might actually have been tuberculosis cutis orificialis. Tuberculosis cutis orificialis is a more common form of secondary cutaneous tuberculosis; it is contracted by reinoculation from an exogenous source, but the resulting lesion is essentially multibacillary, and it classically occurs in patients with a persistently poor immune status. (1)
The variability in the etiopathogenesis of LV calls for a change in attitude from clinician-otolaryngologists in terms of diagnosing patients with destructive ulcers in the face and pharyngeal mucosa. Notwithstanding the need to exclude other chronic granulomatous ulcers such as Wegener granulomatosis, sarcoidosis, and Hansen disease that might cause damage to the septum and gross mutilation of the nasal and/or facial architecture, a high index of suspicion should be reserved for LV. This is in context with the current resurgence of extrapulmonary tuberculosis secondary to the HIV/acquired immunodeficiency syndrome pandemic, (2) the emergence of multidrug resistance, and the health consequences of immigration.
These factors might also explain the increased incidence of LV in the tropical East, when it has been mostly prevalent in the temperate West (i.e., northern Europe, including the U.K.). (3) However, involvement of the nose and pharyngeal mucosa in our patient seems atypical. Unlike lupus in the temperate West, which primarily affects the head and face (nose, perinasal area, cheeks, and ear), lupus in the tropical East primarily involves the lower extremities and buttocks. (1) Also, there is not always a definitive history of a patient's defaulting on his or her medication regimen or of having an active tubercular source, thus making the diagnosis challenging.
LV is a paucibacillary lesion, and it seldom yields Mycobacterium spp on histology, culture, (3) guinea pig inoculation, and polymerase chain reaction (50 to 72% sensitivity (4) compared with ~95% in multibacillary forms (5)). This underlines the fact that suggestive clinical findings, granulomas, and giant cells on histology, a strongly positive Mantoux test, and a response to antituberculosis therapy remain the cornerstones for diagnosing mucocutaneous lupus. (6)
For otolaryngologists who deal with ulcerative lesions in the head and neck, LV should be strongly considered in the differential diagnosis. Timely intervention with antituberculosis drugs should ensure a dramatic recovery, while a delay might result in malignant transformation (primarily squamous cell carcinoma), since LV-being a chronic, indolent, cutaneous lesion-may behave as facultatively precancerous. (7)
The authors thank Prof. Debabrata Bandyopadhyay, MD, head of the Department of Dermatology and Venereal Diseases at the Medical College and Hospital, Kolkata, for his invaluable help and suggestions in the preparation of the manuscript.
(1.) Steger JW, Barrett TL. Cutaneous tuberculosis. In: James WD, ed. Military Dermatology. Washington, D.C.:TMM Publications; 2009:355-89.
(2.) Fitzgerald DW, Sterling TR, Haas DW. Mycobacterium tuberculosis.In: Mandell GL, Bennett JE, Dolin R, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 7th ed. Philadelphia:Churchill Livingstone; 2010:3129-63.
(3.) Freitag DS, Chin R. Facial granulomas with nasal destruction. Chest 1988; 93 (2): 422-3.
(4.) Hsiao PF, Tzen CY, Chen HC, Su HY. Polymerase chain reaction based detection of Mycobacterium tuberculosis in tissues showing granulomatous inflammation without demonstrable acid-fast bacilli. Int J Dermatol 2003; 42 (4): 281-6.
(5.) No authors listed. Diagnostic Standards and Classification of Tuberculosis in Adults and Children. This official statement of the American Thoracic Society and the Centers for Disease Control and Prevention was adopted by the ATS Board of Directors, July 1999. This statement was endorsed by the Council of the Infectious Disease Society of America, September 1999. Am J Respir Crit Care Med 2000; 161 (4 Pt 1): 1376-95.
(6.) Alavi SM, Nashibi R. Nasal tuberculosis in a 56 year old woman. Caspian J Intern Med 2014; 5 (1): 49-51.
(7.) Haller D, Reisser C. Lupus vulgaris manifestation as a destructive nose and facial tumor [in German]. HNO 2009; 57 (4): 364-7.
From the Department of Otorhinolaryngology-Head and Neck Surgery, Medical College and Hospital, Kolkata, West Bengal, India (Dr. Dutta and Dr. Sinha); and the Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine and Sagore Dutta Hospital, Kolkata (Dr. Ghatak).
February 24, 2016 by Mainak Dutta, MS; Soumya Ghatak, MS; Ramanuj Sinha, MS, DNB
Caption: Figure 1. Preintervention photographs show the ulcers on the nose (A) and the right tonsillar fossa (B). The nasal ulcer involves the tip, columella, alae, and the anterior part of the cartilaginous septum, with an adjacent cuff of induration and crusting. The tonsillar ulcer is deep, with prominent edges, and is covered with necrotic debris.
Caption: Figure 2. Pathologic analysis of the biopsy specimen obtained from the nasal ulcer shows several noncaseating granulomas with typical Langhans giant cells (arrows) surrounded by a rim of lymphocytes, but without any features of vasculitis, necrosis, or dysplasia. No acid-fast bacilli are seen (hematoxylin and eosin, original magnification x100).
Caption: Figure 3. Chest x-ray shows the noncavitating patchy opacities bilaterally in the middle and upper zones that suggest tuberculous pneumonia.
Caption: Figure 4. After antituberculosis drug therapy, photos show the healing of the nasal ulcer at 4 (A) and 10 (B) months and the tonsillar fossa ulcer at 4 months (C).
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|Author:||Dutta, Mainak; Ghatak, Soumya; Sinha, Ramanuj|
|Publication:||Ear, Nose and Throat Journal|
|Date:||Feb 1, 2016|
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