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Lupus vulgaris causing nasal perforation: Not a thing of the past
2 Dept. of Otorhinology, Govt. Medical College Hospital, Sector 32 B, Chandigarh - 160 030, India
G P Thami
Dept. of Dermatology, Govt. Medical College Hospital, Sector 32 B, Chandigarh - 160 030
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Kaur S, Thami G P, Singhal S K. Lupus vulgaris causing nasal perforation: Not a thing of the past. Indian J Dermatol Venereol Leprol 2003;69:182-183
Granulomatous diseases, both infective and non-infective in origin frequently affect the head and neck region, particularly the nose and the nasal sinuses. A high index of clinical suspicion and a correlation of clinical features, histology and other laboratory investigations helps in arriving at a correct diagnosis. We report a case of lupus vulgaris of the nose, presenting with nasal perforation and discuss its differential diagnoses.
A 30-year-old Indian woman presented with a one year history of a reddish lesion over her nose, followed by ulceration at the same site. The ulcer was increasing in size despite previous treatment with several courses of broad spectrum systemic antibiotics. There was no pastor a family history suggestive of tuberculosis. She had received BCG vaccination in childhood.
Clinical examination revealed a 1 x 1 cm sized, erythematous, ulcerated plaque covered with brownish crusts over the left aloe nasi [Figure - 1]. Removal of crust revealed a 0.5 x 0.5 cm sized perforation of the underlying cartilage into the left nasal cavity. The nasal septum, lips and oral mucosa were normal. There was no regional or distant lymphadenopothy and general physical and systemic examination was normal.
Biopsy from the ulcerated plaque showed non-caseating epithelioid cell granulomas along with Langerhan′s type cells in the dermis, surrounded by lymphocytes. Involvement of nerve fibres by granulomas or features of vasculitis were not observed. Special stains for acid-fast bacilli and fungi and bacterial, mycobacterial and fungal cultures were negative. Routine laboratory investigations including complete blood counts, biochemical profile, liver function tests, urinalysis and chest x-ray were normal. Serology for syphilis, ELISA for human immunodeficiency virus (HIV), sputum for acid fast bacilli and anti-nuclear antibodies were negative. An intradermal tuberculin test (Mantoux test) was highly positive with an induration of 18x20 mm.
The various differential diagnoses considered were lupus vulgaris, leishmaniasis, Hansen′s disease, Wegener′s granulomatosis and midline granuloma. However, based on clinical features, histology and highly positive tuberculin test, lupus vulgaris was regarded as the most likely diagnosis. Anti-tuberculous therapy (6 - month regimen, 2HRZE + 4HR) was started. Clinical improvement was observed within one month and at the end of
six months of therapy, the ulcerated plaque had completely resolved. A clean-looking perforation with healthy margin was present, which was surgically repaired and closed to achieve an acceptable cosmetic appearance.
Lupus vulgaris is a chronic, progressive and the commonest form of secondary cutaneous tuberculosis. It develops in a previously sensitized host having a high degree of tuberculin sensitivity. The condition is more common in males than in females and lupus vulgaris usually occurs through hematogenous or lymphatic spread from an underlying infective focus. Rarely it may develop following direct inoculation of the bacilli into skin or at the site of bacille calmette-Guerin (BCG) vaccination.,
Clinically it is characterized by soft reddish-brown plaques with apple jelly nodules on diascopy. The lesions pursue a chronic course over several years and grow by peripheral extension and central scarring. The diverse clinical forms of lupus vulgaris include popular, nodular, plaque, ulcerative, vegetative and tumour-like lesions. In India, trunk, buttocks and extremities are the predominant sites affected, in the West the lesions favour head and neck.,
The disease commonly affects the nose and nasal cartilages. The nasal bones are however spared. Ulceration, necrosis and scarring occur with destruction of deeper tissues and cartilage leading to gross deformities and contractures. Direct extension or lymphatic spread from the nasal focus may also involve the soft and hard palate, gingiva, larynx and pharynx.
The clinical differential diagnosis of lupus vulgaris includes various infective and non-infective disorders showing a predilection for nose and adjoining areas of face. Atypical mycobacterial infections due to M. avium intracellulare complex and M. fortuitum-chelonae complex that may present with erythematous nodules, plaques, abscesses and non-healing ulcers, have been misdiagnosed as lupus vulglaris. However, the culture characteristics help to reach a correct diagnosis. Leishmaniasis, characterized by cutaneous ulceration and involvement of oro-nasal mucosa, may progress to destruction of the central structures of face including nose. Histopathology show a granulomatous infiltrate and Leishman-Donovan bodies representing the parasites, which can be grown on culture. Rhinoscleroma is another chronic infective granulomatous disorder caused by Klebsiella rhinoscleromatis, that causes destruction of nose and other structures of upper respiratory tract. Diagnosis is established on histology showing characteristic Miculikz cells in the deep dermis.
The cartilage destruction of nose in lupus vulgaris is similarto that seen in Hansen′s disease, however, loss of lateral eyebrows and diffuse infiltration of face and ear lobes are unique to leprosy. Syphilis, another chronic condition, should be ruled by serology and special histological stains.
Amongst non- infective disorders, nasal septal defects can be observed in Wegener′s granulomatosis (WG), midline granuloma and sarcoidosis. WG, a systemic vasculitis, primarily involves the upper and lower respiratory tracts and kidneys, mucosal abnormalities of nose and paranasal sinuses range from diffuse mucosal thickening, antineutrophil cytoplasmic antibody test and presence of necrotizing granulomatous vasculitis on histopathology. Lupus pernio, a variant of cutaeous sarcoidosis, showing chronic persistent violaceous nodules under tip of nose to exuberant plaques spreading across nose to both cheeks may also be mistaken for lupus vulgaris. Other sites of predilection for lupus pernio include lips, eyelids, ear and fingers. There may be intrathoracic involvement along with bone and eye lesions and histology and radiological features can help in differentiating this variant of sarcoidosis from lupus vulgaris. The diagnosis of lupus vulgaris is often missed initially due to lack of awareness of the disease and chronic atypical clinical presentation. Thus a high index of clinical suspicion-helped by morphology and histology and appropriate investigations to rule out closely simulating disorders, enables an early diagnosis of lupus vulgaris of nose. Timely commencement of proper anti- tuberculous treatment can help to achieve an early cure and can prevent the irreversible destructive lesions of face of this chronic infection.
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