An ulceration that tore into the flesh like the ravages of a wolf probably fitted the clinical description and explains the word ‘lupus’ which means wolf. The commonness of this condition in earlier times accounts for the adjective ‘vulgaris’ in lupus vulgaris.[6
] The earliest description of lupus vulgaris was by Erasmus Wilson, in 1865. The other synonyms for this condition are: tuberculosis luposa and tuberculosis luposa cutis.[7
Tuberculous gumma results from hematogenous dissemination from a primary site of tuberculosis, especially in malnourished children with impaired immunity.[8
] Tuberculous gumma differs from scrofuloderma, in arising independently, of an underlying lymph node.[5
] In our patient, the sinuses were present in the gluteal region. Our patient had a positive Mantoux test, indicating moderate immunity.
The clinical variants of lupus vulgaris are many and include hypertrophic, ulcerative, vegetative, papular, nodular and mucosal lesions. Hypertrophic forms may present as tumorous growths of soft consistency or may show epithelial hyperplasia with the production of hyperkeratotic masses, as was observed in our patient. Edema, lymphatic stasis and recurrent erysipelas, elephantiasic thickening and vascular dilatation accompany these tumor like forms and may cause gross deformity,[9
] leading to a clinical picture, as in our patient, on the medial aspect of the thighs.
Lupus vulgaris (LV) originates from a tuberculous condition or a clinically inapparent focus elsewhere in the body, by hematogenous, lymphatic or contiguous spread.[9
] In our patient the axillary lymphadenopathy, which started when she was 13 years of age, could be presumed to be the primary focus, as her X-ray of the chest was normal. In about 30% of the cases, LV could be preceded by scrofuloderma,[9
] as was observed in our patient too, though the LV lesions were far removed from the scrofuloderma lesions. Uncontrolled diabetes might have contributed to the hematogenous dissemination of bacilli.
The face is the most common site of the involvement of LV among patients from the Western countries.[9
] In India, the lower extremities, especially the buttocks, seem to be affected more commonly, as noted in our patient. The probable hypothesis is that the bacilli lying dormant for years are reactivated by trauma and non-specific inflammation.[3
The histological changes in the epidermis depend on the morphology of the lesion - either atrophy or hyperplasia is prominent.[10
] In our patient, there was marked hyperkeratosis, papillomatosis and acanthosis, corresponding to the clinical picture on the medial aspect of the thigh. The patient refused a second biopsy from the discharging sinuses on the gluteal region, which probably could have been helpful in establishing a definitive diagnosis of gumma. However, it would have been an academic exercise, since the tuberculous pathology was revealed by a previous biopsy from an adjacent site.
Elephantiasis is applied to many dermatological conditions that ultimately result in severe lymphatic obstruction and stasis. The skin becomes discolored and patches of warty growths occur in the affected area,[7
] as was observed in our patient.
Elephantiasis of the vulva due to extensive destruction of the lymph nodes in the inguinal and femoral region by tuberculous process, though uncommon, was reported.[1
] However, in our patient, due to the gross thickening and verrucosity of the lesions on the medial aspect of thigh, the inguinal and femoral lymph nodes could not be palpated.
In view of the varied morphology of the lesions, with hyperkeratotic verrucous plaques on the medial aspect of thighs and the discharging sinuses on the perineum and gluteal region, in addition to the long standing healed lesions of scrofuloderma in the axillae, this case could not be classified exactly and, hence, was labeled as cutaneous tuberculosis, leading to elephantiasis of the external genitalia.
With the initiation of anti tuberculous treatment, the perineal lesions began to regress. The discharging sinuses healed. The pedunculated polypoid growth from the labium minus was excised. There was no significant alteration in the swelling of the axillae, for which she was referred to a surgeon. Her candidal vulvovaginitis was treated with topical and systemic antifungal medications.
The present case is being highlighted for the rarity of elephantiasis of the genitalia, as a sequel to cutaneous tuberculosis.