Swollen hands and feet syndrome: An uncommon presentation of a common disease

Introduction

Leprosy is an infectious disease caused by M. leprae with predominant skin and neurological involvement. Although musculoskeletal involvement can occur in leprosy during the disease course, the first presentation of leprosy with musculoskeletal manifestation is relatively rare. We present such a case.

Case report

A 46-year-old male presented with painful swelling of both hands and feet with ulcerations of the fingers and toes and painful swelling of both ears for the previous six months (Figure 1(a)–1(c)). He also complained of multiple erythematous lesions over his back and chest for the previous two months (Figure 1(d)), and gave a history of nasal stuffiness and epistaxis, tingling sensation over the hands and feet, but without any suggestion of motor weakness. There was no history of fever, systemic symptoms, or any similar illness in the past. He was taking analgesics with which he had only marginal relief of pain, though his skin rash and swelling of his extremities did not improve.

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Figure 1.

(a). Figure showing swollen hands and fingers. (b). Figure showing swollen feet with healing ulcers over the medial aspect of great toe. (c). Figure showing erythematous swelling involving the whole of the ear including both ear cartilage and lobule. (d). Figure showing multiple erythematous plaques over the trunk.

Examination revealed multiple erythematous plaques over the back and chest, diffuse swelling of hands and feet, healing ulcers over the toes and diffuse erythema and swelling involving both ears. There were no other skin changes such as hair loss, madarosis, or any neuropathic changes. There was no sensory impairment overlying the skin plaques.

However, close examination did reveal impaired pain sensation distally over hands and feet, although other sensory modalities were intact. There was no nerve thickening, all deep tendon reflexes were intact with normal muscle power of both proximal and distal groups of muscles including small muscles of hands and feet. There was diffuse swelling and tenderness involving hands and feet though. No other significant findings were made.

Considering the presence of multiple skin lesions, sinus symptoms, hand and foot swelling and sensory deficit over the extremities, the differential diagnosis included primary systemic vasculitis, rheumatoid arthritis and leprosy.

Investigations revealed mild anaemia (Hb: 107 g/L) with normal leucocyte and platelet count, elevated erythrocyte sedimentation rate (44 mm/1^st h) and normal liver and renal function tests. Autoimmune workup showed negative rheumatoid factor, anti-CCP, ANA and ANCA. Nerve conduction studies showed sensory neuropathy affecting both upper limbs and distal sensorimotor neuropathy affecting both lower limbs. Slit skin smear from the ear lobules and skin lesions over the chest and back showed the presence of acid-fast bacilli with a bacteriological index (BI) 6 + over ear lobules and 4 + over the chest wall. Considering the presence of a large number of skin lesions with high BI, the presence of neurological symptoms and absence of systemic symptoms, a diagnosis of lepromatous leprosy with a type I lepra reaction was made. Multi-bacillary multi-drug therapy (MB-MDT) for leprosy (rifampicin, clofazimine, dapsone) and prednisolone (1 mg/kg/day) was initiated.

Symptomatic improvement in the skin lesions and partial improvement of neurological symptoms were found at follow-up after four weeks and tapering of prednisolone was started but with continuation of MDT. It was intended to continue MB-MDT for 12 months and continue his prednisolone with gradual tapering till the recovery of neurological symptoms.

Discussion

Rheumatological manifestations may be seen in 64–77% of cases of leprosy. ¹ Although arthritis is the commonest, swollen hands and feet due to tenosynovitis may also be a presenting manifestation, especially in the lepromatous form of leprosy and during lepra reactions. ² These may mimic inflammatory rheumatological disorders such as rheumatoid arthritis. Although these features are more common in the type 2 lepra reaction, they are relatively rare in type 1 reactions. A detailed history and careful clinical examination is essential to reach a correct diagnosis.