SkIndia Quiz 36: A Phantasmagoric Lesion: A Face for Thought.

A 22-year-old immunocompetent female presented to the dermatology outpatient department with a hyperkeratotic erythematous plaque with irregular border over the malar area of face and bridge of nose since one year. Initially, the lesion started as papule on the right cheek, which eventually extended onto the bridge of the nose and further over the malar regions of the left cheek to attain the present size, developing pus discharge at places and crusting and scarring at other places [Figure 1]. It was tender on examination. There was no history of fever, photosensitivity, visit outside the native place, or any chronic illness. Family history was nonsignificant. The patient had taken treatment in the form of oral antibiotics, oral steroids, and intralesional steroid injection but with no relief. Hematological and biochemical parameters were within normal limits. Histopathological examination from the left malar area revealed area of ulceration with dense inflammatory infiltrate, acute and chronic, rich in plasma cells. On step sectioning, a solitary epithelioid cell granuloma with Langhans giant cells was seen. However, there was no caseation necrosis [Figures 2 and 3, H and E, ×40].

Figure 1

Hyperkeratotic erythematous plaque with irregular borders over the malar area of face with scarring at places

Figure 2

Dense inflammatory infiltrate, acute and chronic, rich in plasma cells (H and E, ×40)

Figure 3

Langhans giant cells seen on step sectioning without caseation necrosis (H and E, ×40)

Special stain: Ziehl–Neelsen stain for acid fast bacilli, Grocott-Gomori's (or Gömöri) Methenamine Silver stain and Periodic Acid Shiff stain for fungus, and Giemsa stain for Leishman Donovan bodies were negative.

Answer

Lupus Vulgaris (plaque variety).

Discussion

Lupus vulgaris is a chronic, progressive, postprimary, paucibacillary form of cutaneous tuberculosis (TB), occurring in a person with moderate or high-degree of immunity. It is the most common form of cutaneous TB in adults in the Indian subcontinent and South Africa.[1] All age groups are equally affected, with females two to three times more commonly than males.[1] The most prominent histopathologic feature is the formation of typical tubercles with or without caseation, surrounded by epithelioid histiocytes and multinucleate giant cells in the superficial epidermis with prominent peripheral lymphocytes. Secondary changes such as epidermal thinning and atrophy or acanthosis with excessive hyperkeratosis or pseudoepitheliomatous hyperplasia can also be noted. Acid-fast bacilli are usually not found.[1]

It can be caused by hematogenous, lymphatic, or contiguous spread from elsewhere in the body. Frequent localization of the lesions around the nose and on cheeks is attributed to the rich and porous venous plexuses with stasis, cold and hypoxia, impaired fibrinolysis and host defense at a lower temperature, as prevailing in western countries. Thus face is the most commonly affected site in European countries.[2] Higher temperatures prevailing in most parts of India may be responsible for the relatively low frequency of the facial lesions in comparison to the Western world. Extensive facial involvement as seen in this could easily be misdiagnosed owing to its dubious presentation.

Characterized by clinical expression, there are five main types[3] of lupus vulgaris:

Plaque: Flat plaques with irregular or serpiginous edge. The surface may be smooth or psoriasiform. Large plaques may show irregular areas of scarring with islands of active lupus tissue.

Ulcerative and mutilating forms: Scarring and ulceration predominate with crusting. The deep tissues and cartilage are invaded and contractures and deformities occur.

Vegetating form: This is characterized by marked mucous membrane invasion and cartilage destruction. When the nasal or auricular cartilage is involved, extensive destruction and disfigurement ensue.

Tumor-like forms: The hypertrophic form presents either as soft tumor-like nodules or as epithelial hyperplasia with the production of hyperkeratotic masses.

Papular and nodular forms: Multiple lesions occur simultaneously in disseminated lupus: True “miliary lupus.” This usually occurs after temporary immunosuppression as in postexanthematous forms, such as after measles.

The differential diagnoses to be considered at early stages are leprosy, sarcoidosis, lymphocytoma, and leishmaniasis. The histopathology and culture reports help to differentiate lupus vulgaris from the deep mycoses, which closely resemble the vegetating and crusted types.[3]

Standard four drug antituberculosis therapy must be given in histopathologically confirmed cases for six months. A therapeutic trial of triple antituberculosis therapy: Isoniazid, rifampicin, and pyrazinamide may be considered in cases where the diagnosis is difficult. A clinical response would be expected within 4–6 weeks.[1] Our patient was referred to the DOTS center where she was started on the Category 1 regime.

Thus lupus vulgaris, although the most common morphological variant of cutaneous tuberculosis, can sometimes occur with bizarre clinical presentations over atypical sites, often leading to misdiagnosis and inappropriate treatment. Timely diagnosis and treatment can actually be a face saver, literally.

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Conflicts of interest

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