A nodulo-ulcerative lesion on the nose
Published Web Locationhttps://doi.org/10.5070/D33d6958p0
A nodulo-ulcerative lesion on the nose1. Department of Dermatology and Venereology, Faculty of Medicine, University of Porto, Porto, Portugal
Paulo Morais MD1,2, Olga Ferreira MD1,2, Ana Nogueira MD2, Herberto Bettencourt MD3, Filomena Azevedo MD2
Dermatology Online Journal 16 (8): 11
2. Department of Dermatology and Venereology, Hospital S. João EPE, Porto, Portugal
3. Department of Pathology, Hospital S. João EPE, Porto, Portugal
Lupus vulgaris (LV) is a chronic, progressive, and potentially destructive form of cutaneous tuberculosis commonly seen in previously sensitized individuals with moderate to high immunity. We present a case of LV located on the nose of an 84-year-old female patient, discuss the diagnosis and treatment modalities, and emphasize the importance of having a high index of suspicion for this condition.
An 84-year-old female patient with arterial hypertension, dyslipidemia, chronic bronchitis, and a history of pulmonary tuberculosis in adolescence, presented with a 12-month history of a slowly growing erythemato-violaceous, nodulo-ulcerative plaque located on the nasal tip. The plaque was approximately 4 x 2 cm in size and had well-defined borders and a soft consistency (Figure 1). No “apple-jelly” nodules were seen on diascopy. Her general physical and systemic examination was normal. There was no scar suggestive of Bacille Calmette-Guérin (BCG) vaccination. The patient had been previously treated with topical hydrocortisone, fusidic acid, and oral amoxicillin/clavulanic acid with no improvement.
Laboratory investigations including full blood count, biochemical profile, and angiotensin-converting enzyme were normal, except for an elevated erythrocyte sedimentation rate (48 mm/h). A punch biopsy specimen was obtained from the lesion (Figure 2).
Ehrlich-Ziehl-Neelsen staining of the biopsy material was negative for acid-fast bacilli and cultures revealed no growth. However, a polymerase chain reaction (PCR) assay disclosed the presence of Mycobacterium tuberculosis DNA in the biopsy specimen. A Mantoux test was strongly positive with a 25 mm x 25 mm area of induration. Acid-fast bacilli staining, cultures, and PCR for M. tuberculosis DNA were negative in sputum, gastric lavage, feces, and urine samples. Chest X-ray and abdominal ultrasonography were normal.
Therefore, a diagnosis of the lupus vulgaris variant of cutaneous tuberculosis was made and multidrug therapy was instituted (isoniazid 300 mg/day, rifampicin 600 mg/day, ethambutol 1,000 mg/day and pyrazinamide 1,500 mg/day for the first 2 months, followed by rifampicin and isoniazid with the same dosages for the subsequent 6 months). After 6 weeks of treatment the lesion had completely healed, leaving only a slight post-inflammatory hyperpigmentation.
Tuberculosis is still an important health problem in underdeveloped and developing countries. More than 2 billion people, equal to one-third of the world’s population, are infected with Mycobacterium tuberculosis bacilli and 1.8 million people died from tuberculosis in 2008, including 500,000 people with HIV . Cutaneous tuberculosis (CTB) represents 1.5 percent of all cases of extrapulmonary tuberculosis .
Lupus vulgaris (LV), or tuberculosis cutis luposa, is a chronic and progressive form of CTB that occurs in patients with moderate to high immunity against M. tuberculosis, as evidenced by a strongly positive tuberculin test [3, 4, 5]. It is the most frequent variant of CTB in industrialized countries [4, 6, 7, 8] with an average prevalence of 0.37 percent among general dermatology patients . In rare cases, species other than M. tuberculosis can cause this disease [8, 9]. Lesions appear in normal skin as a result of direct extension of underlying tuberculous foci, lymphatic or hematogenous spread, primary inoculation, BCG vaccination, or a previous scrofuloderma [3, 4, 5, 7, 8, 9].
Clinically, the characteristic lesion of LV is an asymptomatic brownish-red solitary plaque, with soft consistency, centrifugal growth and tendency to ulceration. The plaque often shows “apple-jelly” nodules under diascopy [4, 5, 8]. In Europe, more than 80 percent of lesions are on the head and neck, particularly around the nose . This condition is more common in females than in males and all age groups are equally affected. The morphological patterns of LV are categorized into plaque, ulcerative/mutilating, vegetative, tumor-like, and papular/nodular forms. Unusual forms reported include a necklace form, an annular form, and a sporotrichoid form, among others [3, 5, 9, 10].
Because LV is a paucibacillary form of tuberculous infection, bacilli are rarely seen on tissue staining and cultures are frequently negative [2, 4, 5, 7]. However, the Mantoux test can be strongly positive [2, 5, 10]. PCR is a useful and rapid method that has become available in recent years for the diagnosis of LV and other forms of CTB. Although positive in our patient, its sensitivity is usually reduced when used with smear-negative specimens or paucibacillary samples . Sometimes, the diagnosis of CTB is only established retrospectively, after response to a therapeutic trial [2, 5, 10].
Several conditions should be included in the differential diagnosis of LV: sarcoidosis (lupus pernio), tuberculoid leprosy, lupoid leishmaniasis, tertiary syphilis/syphilitic gumma, deep fungal infections, lymphoma, protracted superficial Wegener granulomatosis, lupoid rosacea, granulomatous rosacea, necrobiotic xanthogranuloma, various histiocytoses, port-wine stain, psoriasis, and Bowen disease [3, 5, 10].
According to World Health Organization recommendations the treatment of LV is the same as pulmonary tuberculosis [1, 4, 9]. The basic scheme of tuberculosis treatment consists of an initial phase lasting 2 months, consisting usually of four drugs: isoniazid (5 mg/kg), rifampin (10 mg/kg), ethambutol (15 mg/kg), and pyrazinamide (25 mg/kg). In the continuation phase, lasting 4 to 6 months, two drugs (isoniazid and ethambutol) are necessary [3, 8, 9]. Our patient was treated with this multidrug therapy and showed rapid improvement.
Often, LV runs an extremely chronic course and may, depending on location, lead to disfigurement or mutilation [4, 8, 9]. The ulcerative form is the most destructive and disfiguring form. Fortunately, in our case the skin lesion completely subsided after antituberculous treatment, leaving only slight residual atrophy and hyperpigmentation. Squamous and basal cell epithelioma may arise in long-standing CTB or in the residual scars [5, 11]. The histological picture in our patient ruled out this diagnosis.
Lupus vulgaris is generally under-diagnosed because of a lack of awareness of the disease. This case illustrates the importance of having a high index of suspicion for this condition. In our patient, the diagnosis of LV was made based on the cutaneous symptoms, supported by the histopathological findings, Mantoux test, and PCR assay.
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