Indian Journal of Paediatric Dermatology

: 2016  |  Volume : 17  |  Issue : 2  |  Page : 161--163

Extensive lupus vulgaris: A variant of extrapulmonary tuberculosis

Savita Arya1, Ramesh Kumar Kushwaha1, Moti Lal Bunkar2, Suresh Jain1,  
1 Department of Dermatology, Venereology and Leprosy, Government Medical College, Kota, Rajasthan, India
2 Department of Respiratory Medicine, Government Medical College, Kota, Rajasthan, India

Correspondence Address:
Savita Arya
Department of Dermatology, Venereology and Leprosy, Government Medical College, Kota - 324 010, Rajasthan

How to cite this article:
Arya S, Kushwaha RK, Bunkar ML, Jain S. Extensive lupus vulgaris: A variant of extrapulmonary tuberculosis.Indian J Paediatr Dermatol 2016;17:161-163

How to cite this URL:
Arya S, Kushwaha RK, Bunkar ML, Jain S. Extensive lupus vulgaris: A variant of extrapulmonary tuberculosis. Indian J Paediatr Dermatol [serial online] 2016 [cited 2020 Nov 28 ];17:161-163
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Cutaneous tuberculosis (TB) is an important variant of extrapulmonary TB, with an incidence of around 5.9 cases per 1000 population. Lupus vulgaris (LV) is the most common variant of cutaneous TB (approximate 74%). LV has a progressive nature, which occurs due to dissemination from an endogenous focus or exogenously by the direct inoculation of the bacilli into the skin or lymphatic spread from an underlying infected focus in a sensitized host with a high degree of immunity to mycobacterium TB.[1] Cutaneous TB is often seen with malnutrition, low socioeconomic environments, and in crowded societies. Clinically, it has various morphological variants, including plaque, ulcerative, vegetative, papular, tumor, and nodular varieties. Apple jelly nodule with scar formation and tissue destruction is a characteristic feature of LV plaque. Usually, LV is localized to a single site with limited involvement and extensive disease involving multiple dermatomes is not commonly reported. Here, we are presenting such a case of extensive LV involving multiple sites.

A 10-year-old girl came to the Dermatology Outpatient Department with skin lesions of 2 years duration over both sides of the neck and upper part of the anterior chest wall. Examination revealed well-demarcated, irregularly bordered, nontender, slightly erythematous to scaly, infiltrated plaques extending from auricular region to the neck, trunk, and axillary area [Figure 1]a and [Figure 1]b. Initially, the lesion was localized to the auricular region, later it progressed slowly and involved multiple sites. Systemic examinations, including respiratory system, were within normal limits with no regional or distant lymphadenopathy. She was treated with antibiotics in the past with no significant improvement. Parents denied for any surgical intervention in the recent past. There was no personal or family history of pulmonary TB.{Figure 1}

Routine hematological and biochemical investigations revealed no abnormalities. Serological test results for Leishmania, syphilis, and human immunodeficiency virus were negative. Skiagram chest posteroanterior view showed no abnormality and purified protein derivative test (Mantoux test) was normal with 8–10 mm induration after 48–72 h. Histopathological examination of the punch biopsy specimen showed normal epidermis with focal parakeratosis and noncaseating granulomas consisting of epithelioid histiocytes, plasmocytes, and Langhans giant cells in the dermis suggestive of LV [Figure 2]a. The acid-fast bacillus was not found in the tissue sections by Ziehl–Neelsen (Z-N) stain and a culture of the biopsy material was also negative.{Figure 2}

The patient was treated with short-course chemotherapy regimen involving four drug regimen for initial 2 months (isoniazid 10 mg/kg, rifampicin 10 mg/kg, pyrazinamide 25–30 mg/kg, and ethambutol 15–20 mg/kg) followed by 4 months of two drugs (isoniazid and rifampicin). She responded well, improved, and got a complete resolution of the lesion with marked residual atrophic scarring; causing cosmetic defacement and mutilation along with fibrotic contractures [Figure 2]b.

Cutaneous TB may be acquired by direct inoculation, local invasion, or hematogenous dissemination and these infections are classified as multibacillary and paucibacillary.[2] In rare situations, it can appear at the site of primary inoculation or at bacillus Calmette-Guérin vaccination. Classification of cutaneous TB depends on the morphology, mode of infection, or the immunologic state of the host. LV is one of the most common progressive variants of cutaneous TB. It has a wide variety of presentations ranging from plaque formation to nodular or tumor-like lesion with “apple jelly nodule on diascopy.” It is more common in males than females with no age-specific predilection. Childhood TB represents 5–15% of all the cases of TB. In India, the prevalence of childhood skin TB has been reported to be 18.7% of all the cases of skin TB in Chandigarh, 20.4% in Varanasi, 24.41% in Chennai, and higher prevalence ranging from 31.7% to 53.9% in a recent case series from Delhi.[3] The course of this disease is marked by ulceration and scarring, thus, its clinical manifestation is diverse and a number of complications may ensure. The involution in one area with atrophy and scarring while expansion in another one is the characteristic features of LV lesions.

LV is extremely chronic and without therapy, its course usually extends over many years. Sometimes, it may show self-healing. Although there are periods of relative inactivity, it is progressive and leads to the considerable impairment of function and disfiguration. The most serious complication of long-standing LV is the development of carcinoma.

Most cases of primary TB, particularly in children, are contracted on the extremities and on the face following scratches, bruising, lacerations, pin-pricks, impetigo, boils, piercing, tattoos, and circumcision. In India, the most common affected site is buttocks and extremities, while in the western countries lesions favor head and neck.[4]

The diagnosis of cutaneous TB is based on the clinical features, demonstration of acid-fast bacilli on smear, tissue culture, skin biopsy, and PCR in the recent years. However, tissue culture is the gold standard for diagnosis and also for monitoring the emergence of drug-resistant strains. Culture is often negative as LV is a paucibacillary form of TB infection. Mantoux test is also helpful. It indicates the previous contact with mycobacterium TB infection, and can lead to false-negative results in the case of anergy. Standard short-course chemotherapy for the treatment of cutaneous TB involves the administration of four anti-TB drugs for the first 2 months (isoniazid, rifampicin, and pyrazinamide), followed by isoniazid and rifampicin for 4 months. The differential diagnosis of LV includes sarcoidosis, deep fungal infections, lymphocytoma cutis, tertiary syphilis, leprosy, lupoid leishmaniasis, and discoid lupus erythematous.[5]

Involvement of multiple dermatomes in cutaneous TB is very rare and a high degree of clinical suspicion is required for early diagnosis, especially in a country endemic for TB such as India. The histopathological examination increases the diagnostic accuracy in correlation with the clinical appearance when direct analysis (acid-fast bacilli by Z-N stain) or culture is negative. Sometimes, diagnosis of cutaneous TB is very difficult, in this situation confirmation established by judging the response of therapeutic drug trial for at least three weeks. This case emphasizes the importance of biopsy and histopathology and also claims the early diagnosis of LV to overcome the morbidity associated with scarring and mutilation, which may result in gross cosmetic disfigurement.

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