Milia Over Healing Leprosy Plaque: A Rare Presentation

Milia are benign keratinous cysts that present as small, firm, dome-shaped papules. Leprosy is a chronic granulomatous disease caused by M leprae. It is well known to damage skin adnexa, including eccrine glands, due to infiltration of inflammatory cells or by pressure from granuloma formation followed by their subsequent repair. We report a 12-year-old male with multiple milia over a healing plaque of BT Hansen’s disease to highlight the rare presentation where the healing process and not the acute inflammatory reaction was crucial in milia formation because of the occurrence of milia only over resolving lesions without lepra reactions

Bilateral Groove Sign in Lepromatous Leprosy with Type 2 Lepra Reaction – An Uncommon Presentation

A 35-year-old male patient presented with tingling and numbness over both extremities, skin lesions and buboes in bilateral inguinal region with the classical “sign of groove”. Biopsy from the skin lesion as well as the lymph node aspirate was consistent with lepromatous leprosy with erythema nodosum leprosum (type 2 lepra reaction). This case report highlights the interesting observation of the “groove sign” with leprosy, as a result of lymph node involvement of inguino-femoral region

Hansen's Disease“ Clinically Atypical Presentation with Atypical Histopathology

Type II Lepra reaction is a Th2 mediated hypersensitivity reaction characterized by involvement of skin and nerves. Herein we report an unusual leprosy case that had a rheumatic mode of onset of Type II Lepra reaction. This case also had livedoid plaques, ulcers on the genitalia, extremities as the initial manifestation of Hansen's Disease and atypical histopathology. His symptoms had started 1 year back as polyarthralgia of both knees and small joints of hands and feet for which he was on immunosuppressant by a Rheumatologist who made a diagnosis of rheumatoid arthritis. After 6 months, patient developed red painful lesions; initially on arms, chest and trunk followed by legs. Subsequently, painful ulcerations developed on feet, knees and genitalia and a dermatology referral was made. Histopathology and slit skin smears led to diagnosis of Lepromatous leprosy in Type II Lepra reaction. Hansen's disease is a great imitator. Rheumatic manifestations can be the initial presentation of Type II Lepra reaction. Rheumatic manifestations thus can be the initial presentation of Type II Lepra reaction even before starting Multi drug therapy. High index of suspicion and routine slit skin smears in small peripheral settings can be helpful in diagnosing and treating such cases.

Information on Drugs Used in Management of Lepra Reactions in Commonly used Drug Information Sources in India

Availability of adequate and proper drug information helps in rational prescription of essential drugs. This can be obtained from various sources such as National Formularies; other sources such as Current Index of Medical Specialties, Monthly index of medical specialties and the information available with the regulators and drug package inserts (PI). In this study, we assessed the drug information of the drugs used for treatment of both type of lepra reactions. Five drugs used for treating Lepra reactions were analyzed for any variation (Qualitative) in information on various parameters as mentioned in commonly used drug information sources such as CIMS India, MIMS India, Central Drugs and Standards Control Organization (CDSCO) website and National Formulary of India (NFI). We observed some gross qualitative variation regarding drug information given in different commonly used sources. Variation exists in the quality of information available on indications, contraindications, dosage and completeness of the dosing schedule about drugs available in various sources. As management of Lepra reactions is crucial in achievement desirable outcome of treatment of leprosy, it is important that information regarding drugs used for such indications should be 100% uniform in all commonly used and available sources.

Serum cytokines in erythema nodosum leprosum versus non-reactional leprosy: A systematic review and meta-analysis

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Erythema nodosum leprosum is an immune-mediated complication of leprosy whose underlying mechanism has not yet been fully elucidated, making management difficult.</p><p style="text-align: justify;"><strong>OBJECTIVES:</strong> To determine the serum cytokine profile of ENL compared to non-reactional leprosy states.</p><p style="text-align: justify;"><strong>METHODS:</strong> An open literature search was performed using MEDLINE, Cochrane Library, TRIP and HERDIN electronic databases using the keywords ("cytokines" or "inflammatory mediators") and ("erythema nodosum leprosum" or "ENL") and ("leprosy" or "lepra"). Studies were selected by two independent review authors. Risk of bias was assessed using the Newcastle-Ottawa Scale and statistical analysis was performed using RevMan 5.3 software.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Eight cross-sectional studies with 197 participants were included. Meta-analysis showed that both serum IL-17 and serum IFN-? were significantly decreased (Z 2.39, p = 0.02 and Z 2.74, p = 0.01, respectively) in ENL compared to non-reactional states. However, for IL-1?, IL-6, IL-10, IL-22, TNF-? and TGF-?, no significant differences were found between the two groups.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> ENL appears to be an exacerbation of the Th2 cytokine response seen in the lepromatous pole of leprosy. However, despite pooling of data, sample sizes remain small resulting in significant heterogeneity. Future studies involving large sample sizes and investigating a wider range of cytokines are encouraged.</p>

Reacción lepromatosa tipo I, a propósito de un caso / Type I lepra reaction: a case report

La lepra es una infección crónica, granulomatosa, producida por Mycobacterium leprae, que afecta piel y nervios periféricos. Se describen dos tipos de reacciones leprosas: tipo I y tipo II, las que corresponden a cuadros agudos que exacerban la enfermedad. Estas leproreacciones pueden ocurrir antes, durante o después del tratamiento. Se presenta el caso de un paciente masculino que acude a consultar con lesiones cutáneas y resultado de biopsia de piel con diagnóstico de lepra. Se inicia tratamiento multidroga OMS-MB1. Posteriormente presenta una leproreacción tipo I, por lo que se le realiza tratamiento con prednisona.

Leprosy is a chronic granulomatous infection of the skin and peripheral nervous system produced by Mycobacterium leprae. Two types of acute leprosy reactions have been described: type I and type II. These reactions can occur before, during or after treatment. We present the case of an adult male patient presenting with skin lesions and skin biopsy diagnostic for leprosy. A multidrug WHO-MB 1 treatment was initiated, after which he presents with type I lepra reaction requiring corticosteroids.

A Patient With Coarse Facies And Skin Changes — A Case Report Of Undiagnosed Hansen's Disease Presenting To Primary Care. When Should We Suspect It, And What Should We Do?

A 44-year-old Malay gentleman was seen at the polyclinic on the advice of a medical social worker. He was noted to have coarse facies, extensive skin peeling, and nail deformities in all 4 limbs. He was suspected to have leprosy and referred to the National Skin Centre (NSC) which confirmed the diagnosis of Lepromatous Leprosy by histology. He has shown good progress since starting on a course of treatment. A brief review is presented here as a reminder of the challenges faced in the care of a condition largely forgotten in our highly urbanised setting: Hansen’s disease.

Reacciones lepromatosas en pacientes adultos y pediátricos en un centro de referencia. Programa Nacional de Lepra, Paraguay / Lepromatous reactions in adults and pediatric outpatients in a reference center. National Lepra Program, Paraguay

Introducción la lepra, llamada también enfermedad de Hansen, es una afección de la piel y de los nervios periféricos, infectocontagiosa, causada por Mycobacterium leprae. Las reacciones lepromatosas se presentan aún con tratamiento y son expresiones de respuesta inmunitaria. Conocerlas es importante a fin de facilitar el abordaje. Objetivo determinar la frecuencia y tipo de reacciones lepromatosas en pacientes con diagnóstico de lepra que acuden al centro de referencia de Enfermedad de Hansen en el Hospital Distrital de San Lorenzo, de enero 2013 a diciembre 2015. Metodología observacional, descriptivo, retrospectivo de corte transverso. Resultados se incluyeron 217 pacientes, 72% presentaban lepra MB y 63% era de sexo masculino. La prevalencia de reacción lepromatosa fue 44%, siendo más frecuentes las de tipo 2 (65%). Se presentó reacción lepromatosa como debut de la enfermedad en 27 %. Treinta y tres pacientes presentaron de tres a doce episodios de reacción lepromatosa. El tratamiento fue talidomida y corticoides. Conclusiones la prevalencia de leprorreacciones fue cercana al 50%, predominando las de tipo 2. El tratamiento utilizado fue talidomida y/o corticoides dependiendo del tipo de reacción lepromatosa.

Introduction leprosy, wich is cause by Mycobacterium leprae, also known as Hansen's Disease, affects skin and peripheral nerves. Lepromatous reactions (LRs) are expressions of an immune reaction and remain as a major persistent problem. LRs are present even with appropriated treatment. Emphasis must be made in early diagnosis and prevention of the catastrophic consequences of LRs. Objective to determine the frequency and type of lepromatous reactions in leprosy patients with leprosy attending to reference center of Hansen´s Disease in the District Center Hospital in San Lorenzo, from January 2013 to December 2015. Methodology observational, retrospective cross sectional study. Results 217 patients were included, 72% with multibacillary leprosy. 63% were male. Lepromatous reactions were found in 44%, been more frequent Type II reaction, in 65% of cases. LRs as oset disease occurred in 27%. 33 patients presented from 3 to 12 episodes of lepromatous reaction. The number of LRs episodes per patient were 3 to 12. Thalidomide was used as treatment in Erithema Nodosum Leprosum (ENL) and corticosteroids for the other types de LRs. Conclusions prevalence of PRs were 50%, been more frecuent the type II. Reaction the treatment used was Thalidomide and/or corticosteroids depending on the type of lepromatosus reaction.

Rare atypical feature of type II lepra reaction with ulcerative skin lesion in a lepromatous leprosy patient / 나학회지

Lepra reaction is an acute exacervational status occurring in the chronic course of leprosy. Type 2 lepra reaction occur from multibacillus leprosy (such as BL or LL), which mediated by antigen-antibody immune complex deposited vasculitis, involving multiple organs, and usually associated with constitutional symptoms such as fever, malaise, joint pains. The skin lesions is characterized by painful and tender red papules or nodules on the skin, which also refers to erythema nodosum leprosum (ENL). Different rare atypical skin lesions of ENL have been reported such as bullous, ulcerative, necrotic, pustular, sweet syndrome like lesions in literature. The 74 years old male patient with lepromatous leprosy presented as multiple crusted ulcers on the trunk and red edematous nodule on extremities and face. The histopathologic feature was typical for type 2 lepra lesion. Fite stain revealed large histiocytes containing well defined dense granular bacillus. This case was very rare and interesting because of unusual crusted ulcerative skin lesions combined with conventional ENL lesions and leprosy itself is rare in Korea these days.

Evaluation of key histologic variables in skin biopsies of patients of borderline leprosy with type 1 lepra reaction.

Background: Leprosy remains an important health problem mainly in the African and South-East Asia regions. Type 1 reaction is an immune-mediated phenomenon known to complicate at least 30% of patients of leprosy. Diagnosing type 1 reaction correctly is important for timely institution of therapy to prevent and treat neuropathy-associated disability and morbidity. There is paucity of literature on definitive criteria for histologic diagnosis of type 1 reaction. This study was conducted to determine the key histologic variables for diagnosing type 1 reaction. Methods: This was a prospective study recruiting 104 patients with borderline leprosy. Three pathologists blinded to the clinical diagnosis independently assessed the cases. The agreement between each histological variable and clinical diagnosis was then calculated by using Cohen's kappa (Κ) coefficient. Results: Histological diagnosis of type 1 reaction was given to 27 (67.5%) of 40 clinically diagnosed cases of type 1reaction cases. Histological variables chosen as key variables for histological diagnosis of type 1 reaction were presence of giant cells, dermal edema, intragranuloma edema, granuloma fraction 31-50%, and presence of medium to large giant cells. Conclusion: This study has shown that T1R are still underdiagnosed histologically in comparison with clinical assessments. The key variables for diagnosing type 1 reaction were proposed

New insights in the pathogenesis of type 1 and type 2 lepra reaction.

In the current scenario of leprosy elimination, lepra reactions (LRs) remain a major persistent problem. Type 1 LR (T1LR) and type 2 LR (T2LR) are the major causes of nerve damage and permanent disabilities. The immunopathogenesis of LR have recently become an important fi eld of research, since it may provide the relevant targets for the early detection and control of these episodes. Presently, there are no uniformly acceptable laboratory markers for LR. Genetic and serum markers in human host may predict susceptibility to reactions as well as progression of nerve damage in leprosy. Therefore, a deeper understanding of the molecular mechanisms involved in LR may provide a rational strategy for early diagnosis and prevention of the catastrophic consequences of LR.

A Case Report on Recurrent Lepra Reaction-Type 2.

Lepra Reactions (LR) comprises immunologically mediated inflammatory states that cause considerable morbidity. Type-2 LR occurs exclusively in patients with the lepromatous end of the leprosy spectrum (BL-LL). In 90% of the cases it follows during or after completion of chemotherapy, generally within 2 years. Here we report a case of type-2 LR. He is a twenty years old fisherman with history of hypopigmented skin lesion 3 years back and was treated as a case of lepromatous Leprosy (LL). After six months of completion of treatment he developed type-2 LR presenting with recurrent bouts of fever, polyarthritis, erythema nodusum, orchitis, mononeuritis multiplex and was treated in Faridpur Medical College Hospital (FMCH) on three occasions within last one and half years.

Lepra reactions: A 10-year retrospective analysis

Introduction Leprosy is a chronic granulomatous infection caused by Mycobacterium leprae. Drug treatment is effective in eradicating the bacilli but does not prevent lepra reaction. Despite much attention being focused on the problem of lepra reactions, very limited data has been published on the epidemiology of lepra reactions especially this part of the world. The aim of the study is to improve the understanding of lepra reaction and to determine the demographics and clinical patterns of lepra reactions in Penang General Hospital. Materials and Methods This retrospective study covers a 10-year period from 1997 to 2006. Demographic characteristic and clinical patterns of lepra reactions were analysed with SPSS 13.0 version. Results Of the 95 patients who were enrolled in the study, 67 (70.5%) were male and 28 (29.5%) were females. The mean age at presentation was 40.4 ± 17.9 years (range 3-91 years). There were 35 Malays (36.8%), 34 Chinese (35.8%), 5 Indians (5.2%) and 21 foreigners (22.2%). 35.8% of patients presented with LL (n=34), 18.9% BT (n=18), 17.9% TT (n=17), 13.7% BB (n=13) and 13.7% BL (n=13). In our series, the lepra reaction rate among leprosy patient was 51.6% (n=49). Among those with lepra reaction, 53.1% cases were type 1 reaction (n=26), 44.9% cases were type 2 reaction (n=22) and 2.0% cases were Lucio phenomenon (n=1). Common manifestations observed in lepra reaction were worsening of skin lesions (100%), inflammatory oedema of hands, feet and face (53.1%), nerve pain (46.9%), fever (20.8%) and nerve tenderness (20.4%). Only 4 cases had involvement of other organs like the eye and joint. 30.6% of the reactions observed in our cohort were severe. Type 1 reaction commonly involved those in borderline spectrum whereas type 2 reaction commonly involved those in the lepromatous spectrum. Lepra reactions occurred before treatment (24.5%), during treatment (71.4%) or even after treatment has been stopped (4.1%). Most of the lepra reactions occurred during the treatment period especially the first 12 months of therapy. Conclusion Our study showed a more severe and higher reaction rate compared to other studies. Lepra reaction is a common presentation of leprosy. Type 1 reaction commonly involved those with borderline disease but type 2 reaction commonly involved those with lepromatous spectrum of disease. Lepra reaction occurred before, during and even after the treatment has stopped. Most of the lepra reactions occurred during treatment period especially the first 12 months of therapy.