INTRODUCTION

Leprosy is a granulomatous disease caused by infection with Mycobacterium leprae. It is a chronic multisystem disease with the predominant involvement of the skin and the peripheral nerves [1]. It may also affect the mucous membranes, eye, nose, joints, lymph nodes, internal organs, and bone marrow [2]. This disease has become rare in many countries around the world [3]. Herein, we report two cases of delayed diagnosis of leprosy with multisystemic involvement.

CASE REPORT

Case 1

A 48-year-old male patient presented with generalized infiltrated plaques associated with paresthesias of the limbs evolving for three years. A physical examination revealed leonine facies, depilation of the eyebrows, and erythematous infiltrated plaques on the trunk and limbs (Fig. 1), associated with amyotrophy of the dorsal muscles of the hands and hypoesthesia of the lower limbs. Bacteriological sampling of the ear lobe was positive, with a bacteriological index of Hansen’s bacillus of 4 crosses. A skin biopsy revealed epithelioid granulomatous dermatitis without caseous necrosis. The electromyogram (EMG) showed sensitive polyneuropathy in all four limbs. A facial CT scan revealed a thinning of the nasal septum with bone lysis. Ophthalmological examination showed sequelae of uveitis. Hand and foot X-rays showed diffuse osteoporosis with bone erosions. As the patient had multibacillary leprosy, the WHO-recommended treatment regimen based on dapsone, clofazimine, and rifampicin was administered.

Figure 1: Leonine facies and infiltrated plaques of the trunk.

Case 2

A 63-year-old male patient presented with infiltrated plaques of the face, hypoesthesia of the feet, and ulceration of the left foot associated with paresthesias of the limbs, all of an undetermined chronic course. A physical examination revealed infiltration of the face (Fig. 2a) and ears with papules and scales, achromic macules on the trunk, and an ulcer of the dorsal surface of the left foot with acro-osteolysis of the fingers and toes (Fig. 2b). Bacteriological sampling of the ear lobe was positive, with a bacteriological index of Hansen’s bacillus of 4 crosses. A skin biopsy of the ulcer showed polymorphic granulation tissue. EMG revealed multiple axonal mononeuropathies. A facial CT showed a defect in the nasal septum, and X-rays of the hands and feet revealed osteolytic lesions. A diagnosis of multibacillary leprosy was established, and the patient received polychemotherapy combining dapsone, clofazimine, and rifampicin.

Figure 2: (a) Infiltrated and scaly plaques of the face. (b) Acro-osteolysis and ulceration of the dorsum of the foot.

DISCUSSION

Leprosy, or Hansen’s disease, is a chronic infectious disease caused by the acid-fast rod Mycobacterium leprae [3]. According to the WHO, individuals with one of the following three features have leprosy: (i) definite loss of sensation in a pale or reddish skin patch; (ii) a thickened or enlarged peripheral nerve with loss of sensation; and (iii) the presence of acid-fast bacilli in a slit-skin smear [4].

In 2000, the WHO declared leprosy to be completely eradicated. The disease shows polar clinical forms depending on the patient’s immune response to M. leprae, as well as other intermediate forms. The WHO recommended the use of dapsone and rifampin for tuberculoid leprosy and in combination with clofazimine for managing the lepromatous disease. The combination and duration of treatment vary depending on the type of leprosy [5,6].

Leprosy is a chronic multisystem disease with the predominant involvement of the skin and the peripheral nerves [1]. Bone disease is one of the principal prognostic factors in leprosy and occurs in 15–29% of patients. Higher percentages (40–95%) have been reported in the literature, in part because such data comes from subjects in leper colonies and are, therefore, selected cases [7]. Leprous bone lesions mainly affect the hands and feet and, in more advanced cases, the bones of the cranium or axial skeleton. They may be divided into specific, non-specific, and osteoporotic. The specific changes are the direct result of bone invasion by M. lepra and present mainly as geodes or bone cysts. Non-specific changes are much more common. They are divided into two large groups that may overlap: 1) neurotrophic lesions due to the characteristic neurological damage favoring the appearance of trophic ulcers and recurrent trauma; 2) lesions due to superinfection; these usually present as acro-osteolysis or bone reabsorption in the distal phalanges of the fingers and toes. Finally, osteoporosis is the second most common manifestation after the non-specific changes [8].

Ocular involvement in leprosy is estimated to be 70–75%. About 10–50% of leprous patients suffer from severe ocular symptoms, and blindness occurs in about 5%. Ocular manifestations include madarosis, lagophthalmos, corneal exposure, keratitis, corneal ulceration and scarring, episcleritis and scleritis, conjunctival and scleral lepromas, uveitis, uveal effusion, and retinal pearls and detachment [9].

Nasal clinical manifestations (NCM) of leprosy have long been described, particularly, in lepromatous disease. The symptoms included anosmia, nasal cartilage pain/hypersensitivity, epistaxis, septal erosion/perforation, saddle nose, skin changes (rash/edema), and persistent congestion or rhinitis [10].

CONCLUSION

The peculiarities of our observations were the long duration of disease before correct clinical diagnosis, the multi-system involvement, and the severe deformity of the hands and feet, which is evidence of the long duration of the disease. Physicians should be vigilant in screening and treating new active cases of leprosy even in the post-leprosy elimination era.

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