Half a Century in Hiding: A Unique Case of Tuberculoid Leprosy with an Unprecedented Incubation Period.

BACKGROUND Leprosy, also known as Hansen's disease, is a neglected tropical disease with low prevalence in the United States. The disease's long incubation period can cause delayed presentation, and most affected individuals have a history of travel or work in leprosy-endemic regions. The immune response to Mycobacterium leprae determines the clinical characteristics of leprosy, with tuberculoid leprosy being characterized by well-defined granulomas and involvement of peripheral nerves. The recommended treatment is a combination of dapsone and rifampin for 12 months. CASE REPORT A 78-year-old man with a history of extensive travel to Africa and Asia 50 years ago, presented with a non-tender, non-pruritic, and hypopigmented skin lesion on his left knee. Biopsy results confirmed granulomatous inflammation and the presence of Mycobacterium leprae, leading to a diagnosis of tuberculoid/paucibacillary leprosy. The patient received dapsone and rifampin treatment, which resulted in symptom improvement. CONCLUSIONS The patient's long incubation period of 50 years between exposure and symptom onset is remarkable and possibly one of the longest reported for tuberculoid leprosy. It emphasizes the importance of considering leprosy in cases with an extensive travel history and long incubation periods. Our patient's case presented contradictory staining results, suggesting potential sampling variation or a rare mixed leprosy form. Based on his clinical findings, he was diagnosed with tuberculoid leprosy. Early diagnosis and treatment are crucial to prevent irreversible nerve damage and improve patient outcomes. Healthcare providers should be vigilant in acquiring a detailed travel history to facilitate early diagnosis and appropriate management of leprosy cases.

Enfermedad de Hansen: comunicación de un caso de lepra tuberculoide en Chile / Hansen's disease: case report of tuberculoid leprosy in Chile

Resumen La enfermedad de Hansen o lepra es una enfermedad infecciosa crónica causada por Mycobacterium leprae, y que afecta principalmente la piel y nervios periféricos. En los últimos años, se ha logrado un mejor control de la enfermedad en forma global. Sin embargo, en Chile la incidencia de la lepra ha ido en aumento. Presentamos el caso clínico de un paciente de 40 años, haitiano, con lesiones e histopatología compatibles con una lepra tuberculoide. Se discute la importancia en la educación sobre enfermedades menos prevalentes en Chile y se hace una revisión sobre la fisiopatología, clínica y clasificación de la enfermedad de Hansen.

Abstract Hansen's disease or leprosy is a chronic infectious disease caused by Mycobacterium leprae, mainly affecting the skin and peripheral nerves. In recent years, a better control of the disease has been achieved globally. However, in Chile the incidence of leprosy has continued to increase. We present a case of a 40-year-old patient Haitian nationality with lesions and histopathology compatible with tuberculoid leprosy. We discuss the importance of education on less prevalent diseases in Chile, in conjunction with a review of the pathophysiology, clinic, and classification of Hansen's disease.

Follow-up assessment of patients with Pure Neural Leprosy in a reference center in Rio de Janeiro-Brazil.

INTRODUCTION: Pure Neural Leprosy (PNL) is a rare clinical form of leprosy in which patients do not present with the classical skin lesions but have a high burden of the disability associated with the disease. Clinical characteristics and follow up of patients in PNL are still poorly described in the literature. OBJECTIVE: This paper aims to describe the clinical, electrophysiological and histopathological characteristics of PNL patients, as well as their evolution after multidrug therapy (MDT). METHODS: Fifty-two PNL patients were selected. Clinical, nerve conduction studies (NCS), histopathological and anti-PGL-1serology were evaluated. Patients were also assessed monthly during the MDT. At the end of the MDT, all of the patients had a new neurological examination and 44 were submitted to another NCS. RESULTS: Paresthesia was the complaint most frequently reported by patients, and in the neurological examination the most common pattern observed was impairment in sensory and motor examination and a mononeuropathy multiplex. Painful nerve enlargement, a classical symptom of leprosy neuropathy, was observed in a minority of patients and in the motor NCS axonal injuries, alone or in combination with demyelinating features, were the most commonly observed. 88% of the patients did not present any leprosy reaction during MDT. There was no statistically significant difference between the neurological examinations, nor the NCS pattern, performed before and after the MDT. DISCUSSION: The classical hallmarks of leprosy neuropathy are not always present in PNL making the diagnosis even more challenging. Nerve biopsy is an important tool for PNL diagnosis as it may guide therapeutic decisions. This paper highlights unique characteristics of PNL in the spectrum of leprosy in an attempt to facilitate the diagnosis and management of these patients.

[Association of borderline tuberculoid leprosy and tuberculosis: A case report and review of the literature]. / Association d'une lèpre borderline tuberculoïde et d'une tuberculose : un cas et revue de la littérature.

INTRODUCTION: In metropolitan France, nearly 20 new cases of leprosy are diagnosed each year. The incidence of tuberculosis in France is 8/100,000 inhabitants and there are very few accounts of association of these two mycobacteria. Herein we report a case of co-infection with borderline tuberculoid (BT) leprosy and disseminated tuberculosis diagnosed in metropolitan France. PATIENTS AND METHODS: A male subject presented with diffuse painless infiltrated erythematous plaques. The biopsy revealed perisudoral and perineural lymphohistiocytic epithelioid cell granuloma as well as acid-alcohol-fast bacilli on Ziehl staining. PCR was positive for Mycobacterium leprae, confirming the diagnosis of leprosy in the BT form. The staging examination revealed predominantly lymphocytic left pleural effusion, right-central necrotic adenopathy without histological granuloma, negative screening for BK, a positive QuantiFERON-TB™ test, and a positive intradermal tuberculin reaction. The clinical and radiological results militated in favour of disseminated tuberculosis. Combined therapy (rifampicin, isoniazid, ethambutol and pyrazinamide) together with clofazimine resulted in regression of both cutaneous and extra-cutaneous lesions. This rare co-infection combines leprosy, often present for several years, and tuberculosis (usually pulmonary) of subsequent onset. The pathophysiological hypothesis is that of cross-immunity (with anti-TB immunity protecting against subsequent leprosy and vice versa), supported by the inverse correlation of the two levels of prevalence and by the protection afforded by tuberculosis vaccination. In most cases, treatment for TB and leprosy improves both diseases. Patients presenting leprosy should be screened for latent tuberculosis in order to avoid reactivation, particularly in cases where corticosteroid treatment is being given.

[Atypical Localization of a Case of Leprosy in Lomé (Togo)]. / Localisation atypique d'un cas de lèpre à Lomé (Togo).

This was a 50-year-old woman with a selling activity living in Lomé who came for a consultation in March 2016 for a facial flushing that had been going on for 2 months without pain or pruritus. On examination, there was a single, erythemato-squamous closet of the right hemiface. There was no infiltration of the right ear. There was moderate cutaneous heat compared to the left hemiface which was without any lesion. Examination of nails, hair, palms and plants was normal. There was no hypertrophy of the peripheral nerves (superficial cervical plexus, ulnar, median). The face was not fixed. Complementary examinations noted a normal blood count and negative HIV status. Histology performed on a biopsy fragment concluded tuberculoid leprosy. The patient was first put on WHO multidrug therapy during 6 months. But one month after stopping this treatment, the lesions resumed. She was referred to a multibacillary leprosy protocol during one year. She had been seen 4 months after stopping treatment, without recurrence. It is important not to ignore leprosy in case of atypical erythema of the face even in the absence of other evocative signs and to perform a biopsy to the slightest doubt.

Il s'agit d'une femme de 50 ans, revendeuse, résidant à Lomé qui a consulté en mars 2016 pour une rougeur du visage évoluant depuis 2 mois sans douleur, ni prurit. À l'examen, on notait un placard unique érythémato-squameux de l'hémiface droit avec une bordure infiltrée. Il n'y avait pas d'infiltration du pavillon de l'oreille droite. Il y avait une chaleur cutanée modérée par rapport à l'hémiface gauche qui était sans aucune lésion. L'examen des ongles, des cheveux, des paumes et plantes était normal. On notait une absence d'hypertrophie des nerfs périphériques (plexus cervical superficiel, cubital, médiane). Le visage n'était pas figé. Les examens complémentaires notaient un hémogramme normal et une sérologie VIH négative. L'histologie réalisée sur un fragment biopsique a conclu à une lèpre tuberculoïde. La patiente a d'abord été mise sous le protocole de polychimiothérapie de l'OMS pendant 6 mois. Mais un mois après l'arrêt de ce traitement, les lésions ont repris. Elle a été remise sous un protocole de lèpre multibacillaire pour une durée d'un an. Elle a été revue 4 mois après l'arrêt du traitement, sans récidive. Il importe de ne pas méconnaître une lèpre devant un érythème atypique du visage même en l'absence d'autres signes évocateurs et de réaliser une biopsie au moindre doute.

Neuritic Leprosy; An Intriguing Re-visit to a Forbidden Ailment.

Leprosy is a chronic infectious disease that presents with varied manifestations. Pure neuritic leprosy is one of the rarest forms of the disease which is characterized by nerve involvement without the characteristic cutaneous stigmata. Eleven year old, healthy male presented with progressively increasing painful swelling at the medial aspect of the arm near to the right elbow joint with difficulty in extending right ring and little fingers at interphalangeal joint and numbness in the same region for last 1 year with no cutaneous abnormalities. Physical examination revealed 6x3 cm firm, tender lesion 3 cm proximal to the right elbow joint with positive tinel's sign, without signs of inflammation, along with characteristic claw hand deformity of right hand and atrophy of hypothenar and interossei muscle. Electro-diagnostic testing revealed findings consistent with a right ulnar axonal neuropathy above the elbow. Magnetic resonance imaging revealed well defined heterogeneously hyper intense linear lesion along the course of thickened ulnar nerve in the distal arm extending posterior to the medial condyle. It also showed an oval shaped lesion (2.1x1.0 cm) arising from the same segment of the nerve, without any bony or muscular involvement of that area. The patient underwent surgical exploration and ulnar nerve decompression with biopsy. Pathology revealed necrotizing granulomatous inflammatory acid fast bacilli stain negative lesion, which was histologically consistent with caseous abscess caused by tuberculoid leprosy, pathognomonic for Hansen's disease. He has been started on antibiotic therapy and is referred to leprosy center for further course of management. Pure neuritic leprosy, though rare, should be considered as differential diagnosis in cases presenting with peripheral neuropathy at leprosy-endemic areas. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Nerve abscess in pure neural leprosy mistaken for peripheral nerve sheath tumour with disastrous consequence: what can we learn?

A 34-year-old Indian man presented to an orthopaedician with gradually progressive hypoesthesia affecting his right lower limb and an ipsilateral common peroneal nerve swelling around the knee. The nerve swelling was diagnosed as a peripheral nerve sheath tumour based on MRI findings and was excised, only to be revealed as leprous nerve abscess on histopathology later. The patient developed right foot drop as a result of common peroneal nerve biopsy. This case presents several learning points in the diagnosis of pure neural leprosy.

Tuberculoid leprosy masquerading as systemic lupus erythematosus: an interesting observation.

Leprosy is a chronic granulomatous infectious multisystem disease that may present with protean manifestations. It mimics many systemic and dermatological disorders. Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus. It is important to be aware of such manifestations of leprosy and improve awareness of it in clinicians to avoid misdiagnosis and delay in treatment.

Leprosy with ulnar nerve abscess: ultrasound findings in a child.

We report the ultrasound findings of a typical case of nerve abscess due to leprosy in an 11-year-old boy. The patient had previously undergone pediatric multibacillary leprosy multidrug therapy (MDT) in accordance with World Health Organization guidelines. He presented to our service with bilateral ulnar neuritis with no response to prednisone (1 mg/kg). Right ulnar nerve ultrasound revealed nerve hypoechogenicity, fascicular pattern disorganization, marked fusiform thickening, and a round anechoic area suggestive of intraneural abscess. Intense intraneural power Doppler signal was detected, indicating active neuritis. Intravenous methylprednisolone had a poor response and the patient was submitted to ulnar nerve decompression, which confirmed nerve abscess with purulent discharge during surgery. As the patient weighed more than 40 kg, treatment with a pediatric dose was considered insufficient and adult-dose MDT was prescribed, with improvement of nerve pain and function. Although leprosy is rare in developed countries, it still exists in the USA and it is endemic in many developing countries. Leprosy neuropathy is responsible for the most serious complications of the disease, which can lead to irreversible impairments and deformities. Nerve abscess is an uncommon complication of leprosy and ultrasound can efficiently demonstrate this condition, allowing for prompt treatment. There is scant literature about the imaging findings of nerve abscess in leprosy patients. Radiologists should suspect leprosy in patients with no other known causes of neuropathy when detecting asymmetric nerve enlargement and nerve abscess on ultrasound.

Leprae reaction resembling rheumatologic disease as presenting feature of leprosy.

Leprosy is a chronic granulomatous infection caused by Mycobacterium leprae with predominant involvement of skin and nerves. We present a 70-year-old man with leprosy whose initial presentation resembled rheumatologic disease, due to leprae reaction. He presented with an 8-week history of worsening neuropathic pain in the right forearm, associated with necrotic skin lesions on his fingers that had ulcerated. Physical examination revealed two tender necrotic ulcers at the tip of the right middle finger and the dorsal aspect of the left middle finger. The patient had right wrist tenosynovitis and right elbow bursitis. Apart from raised inflammatory markers, the investigations for infection, connective tissue disease, vasculitis, thromboembolic disease and malignancy were negative. During the fourth week of hospitalization, we noticed a 2-cm hypoesthetic indurated plaque on the right inner arm. Further examination revealed thickened bilateral ulnar, radial and popliteal nerves. A slit skin smear was negative. Two skin biopsies and a biopsy of the olecranon bursa revealed granulomatous inflammation. He was diagnosed with paucibacillary leprosy with neuritis. He responded well to multidrug therapy and prednisolone; his symptoms resolved over a few weeks. This case illustrates the challenges in diagnosing a case of leprosy with atypical presentation in a non-endemic country.

Autochthonous borderline tuberculoid leprosy in a man from Florida.

Leprosy (Hansen's disease) is a chronic contagious granulomatous disease principally affecting the skin and peripheral nervous system, caused by Mycobacterium leprae. In this report, we present a case of autochthonous leprosy in a man from Florida as the first human case reported from this region. Authors believe dermatologists need to be aware of the possibility of autochthonous transmission of leprosy in the Eastern-Southern United States, and should consider leprosy in any patient with atypical skin lesions, even when a history of contact with armadillo is missing.

Tuberculosis and Leprosy: Classical Granulomatous Diseases in the Twenty-First Century.

Leprosy and tuberculosis are chronic mycobacterial infections that elicit granulomatous inflammation. Both infections are curable, but granulomatous injury to cutaneous structures, including cutaneous nerves in leprosy, may cause permanent damage. Both diseases are major global concerns: tuberculosis for its high prevalence and mortality, and leprosy for its persistent global presence and high rate of neuropathic disability. Cutaneous manifestations of both leprosy and tuberculosis are frequently subtle and challenging in dermatologic practice and often require a careful travel and social history and a high index of suspicion.

Atypical lesions in relapsed leprosy

Relapsed cases of leprosy are not common in patients treated regularly with the recommended polychemotherapeutical schemes. Relapses must be readily identified and distinguished from reactions. This article reports a clinical case of relapse in leprosy, with atypical and exuberant skin changes in patient treated for eleven years with a paucibacillary scheme.

Brazilian clinical trial of uniform multidrug therapy for leprosy patients: the correlation between clinical disease types and adverse effects

This study sought to verify the correlation between leprosy types and the adverse effects of treatment drugs. This quantitative, prospective, nested study was developed at the Dona Libânia Dermatology Centre in Fortaleza, Brazil. Data were collected from November 2007-November 2008. During this period, 818 leprosy patients were diagnosed and began treatment. Forty patients with tuberculoid leprosy (TT) were selected. Twenty patients followed a standard therapy of dapsone and rifampicin and 20 were administered dapsone, rifampicin and clofazimine (U-MDT). Twenty patients with borderline lepromatous (BL) and lepromatous leprosy (LL) were also selected and treated with U-MDT. All of the subjects received six doses. With the exception of haemolytic anaemia, there was a low incidence of adverse effects in all the groups. We did not observe any differences in the incidence of haemolytic anaemia or other side effects across groups of patients with TT, BL or LL treated with U-MDT.

Clinical presentation of tuberculoid leprosy in an epidermodysplasia verruciformis patient.

Epidermodysplasia verruciformis (EV) is triggered by a variety of mechanisms that at least partly include genetic background. We present a Brazilian man with a 30-year history of flat, wart-like lesions with clinical, histopathological, and evolutive aspects consistent with papillomavirus (HPV)-associated EV. Histological analysis of the wart lesions showed epidermis with hyperkeratosis, regular acanthosis, hypergranulosis, and cells with abundant basophilic cytoplasm. Moreover, a perivascular lymphocytic infiltrate was found in the superficial dermis, consistent with a viral wart. Type-2-HPV DNA was detected in various fragments of skin-wart lesions using the polymerase chain reaction (PCR). Two years after the EV diagnosis, the patient presented with an anesthetic well-demarcated, erythematous and mildly scaly plaque on his right forearm. A histopathological analysis of this lesion demonstrated the presence of a compact tuberculoid granuloma. Ziehl-Neelsen staining demonstrated the presence of rare acid-fast bacilli and confirmed the tuberculoid leprosy diagnosis. The patient's Mitsuda Intradermal Reaction was positive. To elucidate the possible mechanism involved in this case of EV, we genotyped the HLA genes of this patient. DQB genotyping showed the polymorphic HLA alleles DQB1*0301 and 0501. The patient was treated with a paucibacillary multi-drug therapy scheme, and the disease was cured in six months. This report describes an EV patient with an M. leprae infection, confirming that tuberculoid leprosy patients possess a relatively specific and efficient cell-mediated immunity against the bacillus and, therefore, localized forms of the disease. Moreover, we show the possible involvement of the polymorphic HLA alleles DQB1*0301 and 0501 in EV induction mechanisms.

Trigeminal trophic syndrome complicating a case of borderline tuberculoid leprosy.

An example of trigeminal trophic syndrome presenting as ulceration of ala nasi in a case of borderline tuberculoid leprosy is reported. To the best of our knowledge, this is only the second case report of this manifestation in leprosy to be documented.

Development of leprosy in a patient with rheumatoid arthritis during treatment with etanercept: a case report.

BACKGROUND: Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disorder. There is a clear association between some disease-modifying drugs used to treat RA and infection. The introduction of the anti-tumor necrosis factor (TNF) therapies has improved the outcome of severe RA. The TNF-antagonism may increase susceptibility to granulomatous pathogens such as Mycobacterium tuberculosis, Listeria monocytogenes, and Histoplasma capsulatum. METHODS: We report the case of a 37-year-old woman with RA receiving an anti-TNF agent, who developed a rash on her back and both legs, which was finally diagnosed as tuberculoid leprosy. RESULTS: This is the first case of leprosy due to anti-TNF therapy reported in Europe. CONCLUSIONS: Clinicians should be aware of this and other types of atypical and serious infections that patients may suffer from when treated with anti-TNF agents.

Oral lesion in leprosy: borderline tuberculoid diagnosis based on detection of Mycobacterium leprae DNA by qPCR.

Oral lesions are rarely reported in paucibacillary forms of leprosy. We report here a case with an erythematous hyposensitive lesion in the palate and no skin lesions. In addition to routine tests, biopsies of the lesion in the palate and of clinically normal surrounding areas were performed and subjected to real-time PCR for detection of Mycobacterium leprae DNA. The biopsy of the oral lesion was positive for bacilli DNA, followed by positive serum anti-PGL-1 and Mitsuda test, but with negative histopathology. The patient was diagnosed with a borderline tuberculoid form. After multidrug therapy the lesion had significantly regressed and the bacilli DNA detection in the former lesion was negative. The bacilli DNA detection in an oral lesion by real-time PCR not only improved leprosy diagnosis, but also helped in the classification of clinical form, and in the establishment of the appropriate therapeutic regime.