Lupus vulgaris uncovered by FNAC after a decade of clinical misdiagnosis as hemangioma

A 49-year-old female presented to the surgery outpatient department (OPD) complaining of swelling on right cheek for 10 years. It was gradually increasing in size causing progressive cosmetic disfigurement. She had no history of fever/weight loss/cough, piercing/tattooing, trauma, or surgical procedure done at site of lesion. Personal and family history of tuberculosis (TB) or contact with TB patient was absent. The patient had received multiple antibiotics but with no relief. Bacillus Calmette Guerin (BCG) scar was present. The lesion was well demarcated, edematous, reddish-brown, papulonodular tumor-like with irregular edges and measured 20 × 18 cm over right cheek [Figure 1]. The surface was rough, scaly, and showed mild scarring. Baseline investigations including complete blood counts and chest X-ray were unremarkable except for an elevated ESR value of 30 mm/1st hour.Figure 1: Papulonodular tumor-like lesion on right cheek, reddish-brown in color with rough, scaly surfaceBased on long history and clinical appearance of lesion, a provisional diagnosis of a vascular lesion (hemangioma) was considered. The patient was then referred to department of Pathology for fine needle aspiration cytology (FNAC) of the lesion. A detailed examination of head and neck region prior to FNAC revealed enlarged right upper cervical and submental group of lymph nodes (LN). Subsequently, aspiration was carried out from both the cheek lesion as well as enlarged lymph nodes. Air-dried, Giemsa stained smears from both sites showed numerous epithelioid cell granulomas in background of chronic inflammatory cells [Figure 2]. Ziehl Neelsen staining of LN smears showed presence of acid fast bacilli (AFB) [Figure 2: Inset]. However, none of the smears showed features of vascular lesion despite careful examination. Thus, a final diagnosis of lupus vulgaris (LV) with tuberculous lymphadenitis was rendered. The patient was prescribed antitubercular treatment (ATT). After 9 months of ATT, the lesion over the cheek showed significant reduction in size along with atrophic scarring [Figure 3] and the lymphadenopathy subsided.Figure 2: Photomicrograph of the aspirate showing epithelioid cell granuloma (Giemsa, 400x). Inset: Ziehl Neelsen (ZN) stain showing acid fast bacilli (ZN, 400x)Figure 3: Post treatment image of the lesion shows healing with atrophic scarring of the surfaceTB is a common infection of tropical countries including India and is caused by mycobacterium tuberculosis and mycobacterium bovis.[1] Lungs are the most commonly affected site. However, the disease is notorious for its capability to affect any organ including skin.[2,3] Cutaneous tuberculosis (CTB) is a rare form of extrapulmonary TB and comprises 1–2% of total cases.[1] LV is a common form of CTB in adults. Its most common presentation is plaque form followed by vegetative, tumor-like, and papulonodular lesion. Ulcerative lesions are the least common (14.2%).[1] Our patient presented with hypertrophic, papulonodular tumor-like lesion. There may also be associated regional lymphadenitis as in our case. LV may develop secondary to cervical tubercular lymphadenitis or pulmonary TB.[2] Our patient also had associated cervical tubercular lymphadenitis. CTB can be acquired endogeneously or exogenously. Rarely, direct inoculation and BCG vaccination can cause TB in a sensitized individual with high degree of immunity toward bacteria.[2] Buttocks and extremities are more frequently affected in Asians while facial lesions are rare.[2] Our patient had lesion on cheek which is rare site, thus causing delay in diagnosis. At times, the clinical presentation may also overlap with other cutaneous conditions further adding to the diagnostic confusion, especially in absence of constitutional symptoms of TB. Early lesions of LV may resemble hemangioma as in our case.[2] A search for similar cases in literature revealed the only other case of long standing LV mimicking hemangioma where the real diagnosis was achieved only after 42 years.[3] LV is a benign but chronic condition with duration of illness varying between 3 months and 50 years before medical help is sought.[3] Although there are periods of relative inactivity, the condition is progressive and leads to considerable functional and cosmetic impairment causing psychological trauma to the patient. FNAC is not routinely employed for diagnosing LV. Cohesive epithelioid cell granulomas with/without necrosis and chronic inflammatory infiltrate are seen but AFB is rarely demonstrable.[4,5] Since positive ZN staining clinched the final diagnosis in our case, other investigations like culture and PCR were not done. Also, patient improved following ATT intake. Thus, a vast spectrum of clinical manifestations and involvement of uncommon sites can cause diagnostic dilemma leading to delay in diagnosis of LV. FNAC is not a primary modality of investigation in CTB but it helped in achieving the correct diagnosis in our case, thus overcoming the morbidity associated with gross cosmetic disfigurement. Therefore, it can serve as an effective diagnostic adjuvant in these lesions. Response to therapeutic trial of ATT further confirms the diagnosis. Also, it is noteworthy to consider CTB as differential diagnosis in chronic atypical lesions taking long time to heal, especially in endemic areas. Our case also highlights how the clinical skill of the cytopathologist was instrumental in uncovering a clinically misdiagnosed condition. Hence, a thorough clinical examination by the performing cytopathologist coupled with proper evaluation of cytomorphologic features and appropriate use of special stains are crucial for accurate diagnosis. 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.