Progressive neuropathy in patients with lepromatous leprosy after multidrug therapy.

BACKGROUND: The lepromatous pole is a stigmatising prototype for patients with leprosy. Generally, these patients have little or no symptoms of peripheral nerve involvement at the time of their diagnosis. However, signs of advanced peripheral neuropathy would be visible during the initial neurological evaluation and could worsen during and after multidrug therapy (MDT). Disabilities caused by peripheral nerve injuries greatly affect these patients' lives, and the pathophysiological mechanisms underlying nerve damage remain unclear. OBJECTIVES: To evaluate the outcome of peripheral neuropathy in patients with lepromatous leprosy (LL) and persistent neuropathic symptoms years after completing MDT. METHODS: We evaluated the medical records of 14 patients with LL who underwent nerve biopsies due to worsening neuropathy at least four years after MDT. FINDINGS: Neuropathic pain developed in 64.3% of the patients, and a neurological examination showed that most patients had alterations in the medium- and large-caliber fibers at the beginning of treatment. Neurological symptoms and signs deteriorated despite complete MDT and prednisone or thalidomide use for years. Nerve conduction studies showed that sensory nerves were the most affected. MAIN CONCLUSIONS: Patients with LL can develop progressive peripheral neuropathy, which continues to develop even when they are on long-term anti-inflammatory and immunosuppressive therapy.

Concurrent pulmonary tuberculosis and lepromatous leprosy in a newly diagnosed HIV positive patient: a case report.

BACKGROUND: The leprosy-tuberculosis (TB) co-infection is rarely reported in recent times. However, this dual comorbidity is associated with high mortality and major morbidity. Unrecognised leprosy-TB co-infection may predispose affected patients to rifampicin monotherapy and subsequent drug resistance. CASE PRESENTATION: A 35 year old migrant, human immunodeficiency virus (HIV) positive male worker presented with 6 month history of symmetric infiltrative nodular plaques of the face and distal, upper extremities. A few days after initial dermatology presentation, a sputum positive pulmonary tuberculosis diagnosis was made at his base hospital. Subsequent dermatology investigations revealed histology confirmed lepromatous leprosy and a weakly reactive rapid plasma reagin test. The presenting clinical features and laboratory results were suggestive of lepromatous leprosy coexisting with pulmonary tuberculosis in an HIV positive patient. CONCLUSIONS: This case illustrates the occurrence of leprosy with pulmonary tuberculosis in an HIV infected patient and the difficulties in interpreting non-treponemal syphilis tests in these patients. This case also highlights the need for a high index of suspicion for co-infection and the need to exclude PTB prior to initiation of rifampicin containing multi-drug therapy (MDT). Interdisciplinary management and social support are crucial in these patients.

Oro-facial manifestations in lepromatous leprosy patients in Central India: clinical findings from a cross-sectional study.

OBJECTIVES: To clinically evaluate the oro-facial manifestations in lepromatous leprosy patients undergoing multidrug therapy in Central India. MATERIALS AND METHODS: Two hundred patients from 2 leprosy treatment centers in Central India who satisfied the diagnostic criteria set by the WHO (2006-2010) committee on leprosy were included in the study. To avoid bias, only patients who started the multi-drug treatment regimen less than 1 year ago were included. All the patients were examined for the presence of oral and facial manifestations. To confirm that the oro-facial manifestations were not due to HIV co-infection, serological diagnostic tests including ELISA, Immunocomb, and Tri-dot were performed. RESULTS: Majority of the patients (n = 189) exhibited oral (n = 145) and/or facial (n = 147) manifestations. The most common oral lesions were found to be fissuring and depapillation of the tongue followed by fibrosis and loss of uvula. Among the facial manifestations, facial skin lesions and loss of eyebrows were most prevalent followed by sagging of facial skin and facies leonine. CONCLUSION: The facial manifestations of leprosy are quite common, readily recognizable, and relatively specific to the disease. Thus, the presence of facial manifestations, especially with co-existing oral lesions must prompt the clinician to mandate further investigations to confirm the diagnosis. CLINICAL RELEVANCE: As evidenced by the present study, facial manifestations and oral lesions are an integral part of leprosy. In addition to being a diagnostic parameter, facial manifestations and oral lesions could potentially be used to monitor the disease progression and treatment outcome.

Erythema Nodosum Leprosum as a Rare and Challenging Cause of Pyrexia of Unknown Origin.

A 30-year old male presented with fever for last 1 year. There were associated multiple painful skin eruptions with hyperpigmentation and scaling over whole body which had been progressively increasing. He also had anasarca along with generalized weakness. He presented to us in shock after an acute episode of gastroenteritis. After stabilization, he was evaluated for cause of fever. Routine fever workup (for typhoid, syphilis, malaria, filariasis, HIV, scrub typhus, leishmaniasis) was negative. CECT chest and abdomen revealed hepatosplenomegaly. There was no response to intravenous (IV) antibiotics and anti-fungal medications. Slit skin smears revealed 3+ acid fast bacilli (AFB). Skin biopsy revealed fragmented acid-fast bacilli with dense collection of neutrophils and foamy histiocytes in upper and middle dermis suggestive of Erythema Nodosum Leprosum (ENL). A diagnosis of ENL with lepromatous leprosy was made and patient started on steroids and thalidomide and subsequently on multidrug therapy (MDT). On therapy, patient's symptoms improved, and skin lesions resolved. Though Leprosy itself is a well-known common cause of PUO in India, its first presentation as ENL is rare and needs good index of suspicion and timely management.

Leprosy manifesting with type 2 leprae reaction in a patient presenting with chronic fever: A case report.

Leprosy is a chronic granulomatous disease involving the skin and nerves, leading to a debilitating condition. Leprosy has been controlled in most parts of the world; therefore physicians are not very well versed in the recognition, management and assessment of this disease. The protean manifestations of leprosy often lead to delays in diagnosis and increase the morbidity. We present a case of a 33-year-old male with fever, lymphadenopathy, nodular skin lesions, uveitis and arthritis. Lymphnode, bonemarrow and skin biopsy revealed 3+ AFB smear with negative AFB cultures, leading to the diagnosis of leprosy. The course of illness was complicated by flare of Erythema Nodosum Leprosum (ENL).

Autonomic neuropathy impairing quality of life after completion of MDT: Are we managing enough?

A 26 year old male, treated case of lepromatous leprosy, presented with severe heat intolerance, loss of sensation and sweating over distal parts of both upper and lower limb of 12 years' duration. On examination, there was definite sensory loss over the extremities in glove and stocking pattern and thickening of the bilateral ulnar, common peroneal and radial nerves. There were three trophic ulcers over the plantar aspect of the right foot, atrophy of the small muscles of hand, reabsorption of the distal index, middle and ring fingers along with total clawing of both hands. A slit skin smear for acid fast bacilli revealed fragmented granular bacilli. A starch-iodine test was used to document the pattern of sweating which coincided with the glove and stocking pattern of sensory loss found in lepromatous leprosy. Even though the patient had completed WHO MDT and bacilli were dead, the damage to the autonomic system was extensive in the patient leading to widespread loss of sweating and severe heat intolerance affecting his quality of life. Also the patient had motor deformity in the form of total clawing which added to his disability. Therefore all cases should be followed up even after completion of MDT, counselled regarding the course of the disease, and trained to tackle the consequences of nerve damage in their daily life. We report the case as autonomic dysfunction that has been rarely documented in leprosy patients and also we emphasise the use of the starch-iodine test in such cases along with management of the disease in part by using hydro-oleo therapy.

Nodular melanoma in trophic ulceration of a leprosy patient: a case study.

UNLABELLED: Non-healing chronic trophic ulceration is very common in leprosy patients. Marjolin's ulcer consists of the malignant transformation of a chronic ulcerative lesion. Nodular melanoma developing from Marjolin's ulcer, caused by a trophic ulceration of a leprosy patient, is very rare with only a few cases reported in the literature. Due to the disguised presentation of these malignancies within trophic ulceration lesions in leprosy, neoplastic transformation is frequently overlooked, leading to misdiagnosed and delayed treatment. This paper reports a case of an 83-year-old man with lepromatous leprosy and chronic ulceration on the foot for 22 years. Over a period of 2 months, the ulcer enlarged, turned black, and became more painful. The patient underwent regional excision and immunotherapy after the diagnosis of malignant nodular melanoma. After 9 months follow-up, no metastasis was found. DECLARATION OF INTEREST: There were no external sources of funding for this study. The authors have no conflicts of interest to declare.

Elastophagocytosis and Elastolysis in Leprosy.

Elastophagocytosis is the engulfment of the elastic fibres by the histiocytes, multinucleated giant cells, or both. The cutaneous lesions showing elastophagocytosis are annular elastolytic giant cell granuloma, actinic keratoses, persistent insect-bite reactions, elastosis perforans serpiginosa, foreign body granuloma. Occasionally, it may occur in infectious diseases like leprosy, granulomatous syphilis, North-American blastomycosis, bacterial folliculitis, and cutaneous leishmaniasis. We report a case of lepromatous leprosy with necrotic erythema nodosum leprosum with secondary anetoderma. Histopathology from the atrophic macule of anetoderma revealed periappendageal, perineural infiltration, elastophagocytosis and reduction in elastic fibres.

Disseminated cutaneous BCG infection following BCG immunotherapy in patients with lepromatous leprosy.

Cutaneous complications of Bacillus Calmette-Guerin (BCG) vaccine, especially in the form of generalised disease, are uncommon and mostly occur in immunocompromised individuals. There is a paucity of data on the cutaneous adverse reactions secondary to BCG immunotherapy in leprosy. We report two unique cases of disseminated cutaneous BCG infection following immunotherapy in patients with lepromatous leprosy. To our knowledge, cutaneous BCG infection presenting as widespread lesions after immunotherapy and confirmed by isolation of Mycobacterium bovis by polymerase chain reaction (PCR) has not been described. A high index of suspicion is required when leprosy patients who receive BCG immunotherapy develop new lesions that cannot be classified as either reaction or relapse, and diagnosis may be confirmed on histopathology and PCR.

A case of lepromatous leprosy with co-existing tuberculosis verrucosa cutis (TVC).

The association of leprosy and cutaneous tuberculosis has been reported rarely. Though cross-immunity may exist between these two mycobacterial infections, tuberculosis can occur throughout the spectrum of leprosy. We report a case of lepromatous leprosy with Type II reaction, with tuberculosis verrucosa cutis (TVC). The patient presented with multiple skin-coloured tender nodules of variable size, involving the upper and lower extremities, nodular enlargement of both ear lobes, erythematous plaques on the face and icthyotic patches over both lower legs and feet, for 6 months. There was also a slow-growing asymptomatic warty plaque over the right lateral malleolus, present for 1 year. Slit skin smear examination from ear lobes, forehead and nodules, and histopathology of the warty lesion, respectively confirmed the diagnoses of leprosy and tuberculosis.

Resident rounds part III: case report: a papulo- nodular eruption with systemic signs and symptoms.

This is a typical presentation of erythema nodosum leprosum in a patient with lepromatous leprosy who recently migrated from Micronesia. The clinical presentation, pathology findings, pathogenesis, and therapeutic options are reviewed here.

Unusual Presentation of Necrotic Erythema Nodosum Leprosum on Scalp: A Case Report.

Lepra reactions are acute episodes occurring during the disease process of leprosy and are of 2 types: type 1 or reversal reaction and type 2 reaction or erythema odosumleprosum (ENL). In the episodes of lepra reaction several parts are affected including face and extremities like oral cavity. In the present case report we reported a rare case of lepromatous leprosy with necrotic ENL involving scalp apart from the usual sites. A 58 year old married male presented to us with complaints of spontaneous onset, recurrent eruption of multiple reddish raised painful lesions. Biopsy from the infiltrated skin over the back showed atrophic epidermis, free Grenz zone, diffuse and periadnexal macrophage granulomas with predominant mononuclear infiltrate, appandageal atrophy, fibrosis around the neural structures and leukocytoclastic vasculitis. Fites stain showed strong positivity for M. leprae. His routine blood investigations showed anemia (Hb = 7.8 gm%), neutrophil leukocytosis (TLC = 17,600, DLC = P66L28M4E2) and raised ESR (80 mm in the first hour). These bullous and necrotic lesions in leprosy may be a manifestation of severe type II reactions in patients with very high bacillary load.

Fulminant hepatic failure in a 15 year old boy with borderline lepromatous leprosy and type 2 reaction.

The liver is the most frequently affected visceral organ in leprosy, particularly in the multibacillary group. Administration of hepatotoxic drugs may also affect liver function. We report the case of a male patient, diagnosed as borderline lepromatous leprosy with Type 2 reaction, who was managed with multibacillary multidrug therapy and steroids, and who then developed acute hepatitis and succumbed to sudden cardiac death. Although erythema nodosum leprosum has been described as a rare cause of death in the literature, such an occurrence in the present era when leprosy has been eliminated needs a special mention.

Case Report: Recurrent Type 2 Leprosy Reaction Associated with Multiple Nerve Function Impairments Refractory to Steroids: A Therapeutic Challenge.

Nerve function impairment (NFI) in leprosy results in serious deformities of the face, hands, and feet and contributes significantly to the stigma associated with the disease. Most literature on NFI focuses on either type 1 reaction-associated NFI, or the silent neuropathy, whereas NFI associated with type 2 reaction (T2R) is less well researched. The latter, however, can be more refractory to conventional treatment, not solely owing to its recurrent nature. We present a therapeutically challenging case of a 31-year-old male with borderline lepromatous leprosy with recurrent T2R associated with recurrent and progressive sensorimotor NFI, largely unresponsive to oral steroids and multibacillary, multidrug therapy.

Chronic type 2 reaction in lepromatous leprosy with underlying Plasmodium falciparum infection: A case report.

Tropical diseases prevalent in leprosy-endemic areas may alter the immunological patient response and also complicate the presentation of leprosy reactional episodes. The introduction of anti-malarial drugs in our case produced a subsidence of reaction. With dwindling manpower skilled in leprosy, the reactional episodes are very often treated with non-steroidal anti-inflammatory drugs, steroids and thalidomide, neglecting the possibility of other co-existing infections, tropical or other. Our case emphasises the importance of history, examination and balanced investigation in the context of tropical diseases in endemic areas before injudicious intervention.

Near fatal case of Lucio leprosy with Lucio phenomenon with pleomorphic features and its successful management from a non-endemic area.

Lucio leprosy is a diffuse non-nodular form of lepromatous leprosy. Lucio phenomenon is a type of reactional state which occurs in untreated cases due to the bacillary invasion of endothelial cells. We hereby describe a histopathologically confirmed case of Lucio leprosy with Lucio phenomenon. The patient presented with pleomorphic clinical features and started taking antileprosy treatment and systemic steroids. After few days of admission, she developed deep ulcers exposing the fascia. She also developed cardiogenic shock secondary to septicaemia. She was managed with inotropes and broad-spectrum antibiotics. The patient was given appropriate wound care and the ulcers healed within a period of 3 months and antileprosy drugs were continued. Our patient is a de novo case of Lucio leprosy with Lucio phenomenon and pleomorphic clinical features who developed near fatal septic shock. She was managed successfully. Despite the extensive disease manifestation, all the wounds healed completely.