Presentation of Hansen's disease as Lucio phenomenon in an Indian male

Case report

A 35-year old labourer from rural Gujarat, India presented with painless, raw areas over the extremities, face and genitals for 15 days. He gave a history of tingling and numbness on his legs, decrease in tactile sensations on hands & feet, and occasional nasal stuffiness for the previous 3–4 years. He had not undertaken any prior treatment.

On examination, he looked emaciated. Systemic examination revealed no abnormalities. Facial skin was atrophic and wrinkled, with areas of superficial ulceration and necrosis over eyebrows, ear lobes, nose tip and lips. There were multiple areas of necrosis and irregularly shaped ulceration resembling medium-vessel vasculitis, and pitting oedema over hands and feet. Owing to the severe necrosis, the fourth and fifth digits of the right hand had fallen off and necrotising fasciitis was present over right ankle, scrotal and penile skin. There were areas of hypopigmented scarring over the knees (Figure 1). The trunk and proximal limbs were relatively spared. There was sensory neuropathy of the glove-and-stocking type. Bilateral uniform thickening of the ulnar, greater auricular and popliteal nerves was noted on palpation, without any associated tenderness or nodularity.

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Figure 1.

Clinical image showing (a) ciliary and superciliary madarosis, saddle nose with crusting over eyebrows, (b) angulated ischemic ulcer over earlobe, (c) angulated purpuric lesions with haemorrhagic ulceration and slough over dorsum of hands, (d) post- inflammatory scarring and hypochromic scarring over knees, and (e, f) necrotizing fasciitis over scrotum and leg.

A complete blood count revealed a microcytic anaemia. Coagulation profile, liver and renal function tests, urine analysis, and fasting blood sugar were normal. Serology for syphilis, Hepatitis B & C and human immunodeficiency virus was negative. Blood, urine and local swab cultures failed to reveal any microbial growth. The Mantoux test was negative and chest radiography was unremarakble.

On histopathology, ‘foamy macrophages’, and features of ischaemic necrotizing vasculitis in the dermis, with aggregation of acid-fast bacilli predominantly in the peri-adnexal and perivascular areas were present. These bacilli were also present within the endothelial cells. Slit skin smear examination from the earlobes showed growth of Mycobacterium leprae with a bacteriological index of 5+ (Figure 2). Cultures were sterile for bacteria and fungi.

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Figure 2.

(a) Ziehl-Neelsen staining of skin smear showing positively stained clumps of bacilli both intracellularly and extracellularly (b) histopathological image showing ischaemic necrosis of dermis and perivascular inflammatory infiltrate.

Lucio phenomenon was diagnosed. WHO multi-bacillary multi-drug therapy (MDT) and prednisolone 0.5 mg/kg/day was administered. His ulcers were cleansed and dressed with silver-impregnated dressing. Over the next two weeks, significant clinical improvement was seen.

Discussion

The first description by Lucio and Alvardo of the phenomenon dates to 1852. It is observed in treatment-naïve patients with a diffuse, non-nodular form of lepromatous leprosy with shiny infiltrative lesions, i.e., Lucio leprosy, which is usually seen in Mexico. Very few cases have been reported from India. ¹ Its precise aetiopathogenesis has not been elucidated. It is believed that the unhindered multiplication of lepra bacilli in an anergic background leads to their diffuse infiltration. This in turn promotes more antigenic load to be presented to circulating antibodies, resulting in immune-complexes and vasculitis. ² Another hypothesis is that there is some synergy between M. leprae and cocci that cause superadded infection, leading to a hypersensitivity response. ³ Recently, a new species viz., Mycobacterium lepromatosis has been isolated from such patients. ⁴ Regardless of the exact mechanism, there is evidence to implicate immune complexes in its pathogenesis, since lesions of Lucio phenomenon often demonstrate vascular and perivascular deposits of immunoglobulins and complement. ⁵

Its usual clinical course at the outset produces painful purpuric lesions, which eventually develop to form well-defined, angulated, jagged ulcers, having geometric shapes, usually over extremities, trunk or rarely the face. These ulcers tend to heal in 2–8 weeks, with resultant atrophic scarring and a hyperpigmented halo. There is usually no associated fever or constitutional symptoms, and there is absence of any systemic involvement or neuritis, which differentiates it from a Type II lepra reaction. Highly positive bacteriological and morphological indices are characteristic. Treatment with WHO MB-MDT and systemic corticosteroids is generally satisfactory.

A striking Lucio phenomenon often unmasks the innocuous-looking and kind-looking Lucio leprosy! Our case highlights the necessity of maintaining a high index of suspicion even in non-endemic areas especially in patients presenting with medium vessel vasculitis.