Indian Journal of Paediatric Dermatology

CASE REPORT
Year
: 2016  |  Volume : 17  |  Issue : 2  |  Page : 148--150

A pediatric case of disseminated mutilating lupus vulgaris: A disgrace for society


Suresh Kumar Jain, Prafull Mehta, Savera Gupta, Ramesh Kumar 
 Department of Dermatology, Venereology and Leprology, Government Medical College, Kota, Rajasthan, India

Correspondence Address:
Suresh Kumar Jain
Department of Dermatology, Venereology and Leprology, Government Medical College, Kota, Rajasthan - 324 001
India

Abstract

Lupus vulgaris (LV) is a chronic and progressive form of cutaneous tuberculosis. The lesions may sometimes be associated with extensive destruction of tissue resulting in marked disfigurement and morbidity. A high index of suspicion is crucial for early diagnosis and treatment and thereby prevention of cosmetic deformity. Lupus vulgaris presenting as disseminated mutilating lesions in a child is uncommon, especially in today's era. Herein, we report an unusual case of lupus vulgaris with coexistence of multiple ulcerative mutilating lesions over face and classical plaque over distant site (right thigh) in a 9 year old girl.



How to cite this article:
Jain SK, Mehta P, Gupta S, Kumar R. A pediatric case of disseminated mutilating lupus vulgaris: A disgrace for society.Indian J Paediatr Dermatol 2016;17:148-150


How to cite this URL:
Jain SK, Mehta P, Gupta S, Kumar R. A pediatric case of disseminated mutilating lupus vulgaris: A disgrace for society. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Dec 6 ];17:148-150
Available from: http://www.ijpd.in/text.asp?2016/17/2/148/175661


Full Text

 Introduction



Cutaneous tuberculosis (TB) is the manifestation of extrapulmonary TB that is quite common among the people of low socioeconomic strata in the developing countries. Lupus vulgaris (LV) is the most common form of cutaneous TB, which is acquired either exogenously by direct inoculation of the Bacilli into the skin or endogenously by hematogenous or lymphatic spread from an underlying infected focus in a sensitized host with a moderate to high degree of immunity to Mycobacterium TB. In India, the trunk and lower extremities are most common sites of involvement; whereas face is the most common site in European countries. Classically, lesion of LV is characterized by chronic, progressive, and well-demarcated plaque with apple jelly nodules that extend irregularly leaving behind atrophy and scar formation. Disseminated and mutilative LV, reported here, is a rare presentation, especially in children.

 Case Report



A 9 year old debilitated girl presented to ouroutpatient department with 1½ years history of red colored lesions over face along with destruction of nose. It started as a small nodule on the tip of the nose, which broke down to form an ulcer which gradually extended to adjacent areas with simultaneous destruction of nose. The patient also had cough for last six months. There was no history of fever. Her father had died of pulmonary tuberculosis 2 years back.

On examination, a large ulcerative plaque with atrophy and crusting was present over the upper lip and nose with the destruction of both lateral and septal cartilages. The anterior half of the nose was almost resorbed [Figure 1]. Multiple infiltrating erythematous plaques of variable size and shape were also present on bilateral cheeks and angle of the mouth. Another large atrophic plaque (20 cm × 10 cm 2) with active erythematous border was also present over anteromedial aspect of right thigh [Figure 2]. Submandibular lymph nodes were enlarged and mildly tender.{Figure 1}{Figure 2}

Hematological and biochemical investigations revealed mild anemia (hemoglobin = 10 mg/dl), lymphocytosis, and raised erythrocyte sedimentation rate, which was 60 mm/1st h. X-ray chest revealed air spaces opacities in bilateral lung field with parenchymal calcification suggestive of pulmonary TB. Venereal disease research laboratory test and HIV ELISA test were negative. Ziehl–Neelsen staining of sputum was positive for acid fast Bacilli. Mantoux test was positive with induration of 21 mm read at 48 h. Deep biopsy was taken which revealed epidermal hyperkeratosis and tuberculoid granuloma composed of mononuclear cells and multinucleated Langhans giant cells suggestive of LV [Figure 3].{Figure 3}

 Discussion



The Latin word “lupus” (meaning wolf) signifies the eroded lesion of skin (as if gnawed by a wolf). LV is usually a reinfection TB of the skin, and mucous membrane acquired either exogenously by direct inoculation of the Bacilli or endogenously by hematogenous or lymphatic spread from an underlying infected focus in a sensitized host. In rare situations, it can appear at the site of primary inoculation or at the site of a Bacille de Calmette et Guérin vaccination.

The typical lesion is a well-demarcated erythematous to skin-colored plaque that shows evidence of healing and scarring in one area and activity in another. However, bizarre clinical forms over atypical sites have continued to emerge sporadically, leading to misdiagnosis and delay in treatment. Unusual variants, such as framboesiform, gangrenous, ulcerovegetating, lichen simplex chronicus, and sporotrichoid types,[1],[2],[3] have been added to the list of conventional morphological patterns of papular, nodular, plaque, ulcerative, vegetating, and tumid forms. The ulcerative and the mutilating variants have the highest tendency for scarring together with deep tissue involvement. Nasal lupus attacks the nose and the upper lip–the premaxillary region. In an untreated case, mucosa and cartilage involvements may lead to ulceration and perforation of cartilaginous septum, which is diagnostically important. Nasolacrimal apparatus involvement may lead to dacryocystitis and mucocele formations.

LV is the most common clinical type of cutaneous TB in adults, and the second most common type (incidence varies from 21.3–40%) seen in children.[4],[5],[6],[7] Ramesh et al. found it to be the most common childhood cutaneous TB (63.5%) in their series.[8]

Facial involvement and extensive destruction of the nasal or auricular cartilage causes disfigurement. In Western countries, rich vascularity, porous venous plexus with stasis, cold, hypoxia, and impaired fibrinolysis and host defense at a lower temperature are attributed to the frequent localization of the lesions around the nose and on the cheeks.[9] In India, however, higher temperatures prevailing in most parts of the country may account for the relatively low frequency of facial lesions.[10]

Diagnosis of cutaneous TB is challenging owing to the fact that the clinical appearance of the lesion may not always be characteristic. Hence, clinical diagnosis may be supported by Mantoux test, detection of acid fast Bacilli in sputum and tissue smears and chest radiograph, but diagnosis can be confirmed only by histopathology, which demonstrates granulomatous lesions with diffuse lymphocytic infiltration and multinucleated Langhans giant cells.

We report this case as disseminated ulcerative and mutilating lupus vulgaris along with a classical plaque at a distant site is a rare presentation, even more so in today's era after countrywide implication of Revised National TB Control Programme. Disease progression to such a late stage in a child unveils the sad state of affairs and a dire need of effective surveillance and awareness program in general public along with early diagnosis and treatment to prevent permanent facial disfigurement and social handicap.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

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