Pediatric Leprosy Profile in the Postelimination Era: A Study from Surabaya, Indonesia

Children who live in leprosy-endemic areas are susceptible to infection due to early and frequent exposure to Mycobacterium leprae. Indonesia is on the verge of eliminating this disease (prevalence rate < 1/10,000 population), but pediatric leprosy continues to occur in low-endemic areas. This study aimed to evaluate pediatric leprosy over a decade in a tertiary hospital in Surabaya, Indonesia. A retrospective study of leprosy in children under 15 years old between 2010 and 2019 was conducted in the Morbus Hansen Division, Outpatient Clinic at Dr. Soetomo Hospital in Surabaya, Indonesia. Seventy pediatric leprosy cases were identified between 2010 and 2019, consisting of 58 multibacillary (MB)-type cases and 12 paucibacillary (PB)-type cases. Slit skin smear (SSS) was positive in 26 cases. There were two cases of grade-2 disability and 15 cases of leprosy reaction (erythema nodosum leprosum) in children at the time of diagnosis. There was an insignificant decline in the number of pediatric leprosy cases in the last 10 years. Cases and disabilities in children were found in some leprosy pocket areas even though the national elimination rate has been achieved. MB infections, disability, and treatment defaults were common problems in pediatric leprosy.

Cutaneous ulceration as a presenting feature of type 1 lepra reaction: A case report

Reactions in leprosy represent sudden shift in the immunological response and are seen in 11–25% of affected patients. It can be seen before, during or after the completion of multidrug therapy (MDT). 1 Two types of reactions are recognized; Type 1 reaction (T1R), seen in borderline leprosy, affecting mainly skin and nerves; type 2 reaction (T2R) or erythema nodosum leprosum (ENL), seen in lepromatous leprosy, characterized by systemic features in addition to cutaneous lesions. Trophic ulcers and ulcerating ENL are well known entities while cutaneous ulceration in T1R is extremely rare; we describe an immune-competent woman with cutaneous ulceration as a presenting feature to highlight the need to recognize this entity at the earliest opportunity.

Erythema nodosum leprosum (type 2 reaction) in a patient with neurofibromatosis type 1

Being a region endemic for leprosy, clinical practitioners in India often encounter myriad manifestations and diverse complications of the disease. However, the masking of the obvious clinical presentations due to the coexistence of a closely resembling unrelated disorder, a ‘mimicker’, would indeed pose a serious diagnostic predicament unless a high degree of clinical suspicion is maintained. Leprosy, also known as Hansen’s disease is a chronic infectious disease caused by Mycobacterium leprae complex that involves the skin and peripheral nerves. Neurofibromatosis type 1 (NF1) also known as von Recklinghausen’s disease is an autosomal dominant genetic disorder that presents with skin changes and benign peripheral nerve sheath tumours called neurofibromas. Here, we present the case of a 35-year-old man with NF1 who presented with type 2 lepra reaction (erythema nodosum leprosum, ENL) and the skin biopsy unmasked ENL nodule among the group of NF1 nodules.

Methotrexate in erythema nodosum leprosum: Pitfalls to avoid

We read with interest the short report by Rani et al. entitled “An uncommon variant of erythema nodosum leprosum responding well to methotrexate: Report of two cases.” The article describes two cases of erythema nodosum leprosum (ENL) with ‘atypical features’ and good response to low dose methotrexate. The authors address a few concerns regarding methotrexate in ENL, emphasizing the rational usage of this agent.

Host-Related Laboratory Parameters for Leprosy Reactions

Leprosy reactions are acute inflammatory episodes that complicate the course of a Mycobacterium leprae infection and are the major cause of leprosy-associated pathology. Two types of leprosy reactions with relatively distinct pathogenesis and clinical features can occur: type 1 reaction, also known as reversal reaction, and type 2 reaction, also known as erythema nodosum leprosum. These acute nerve-destructive immune exacerbations often cause irreversible disabilities and deformities, especially when diagnosis is delayed. However, there is no diagnostic test to detect or predict leprosy reactions before the onset of clinical symptoms. Identification of biomarkers for leprosy reactions, which impede the development of symptoms or correlate with early-onset, will allow precise diagnosis and timely interventions to greatly improve the patients' quality of life. Here, we review the progress of research aimed at identifying biomarkers for leprosy reactions, including its correlation with not only immunity but also genetics, transcripts, and metabolites, providing an understanding of the immune dysfunction and inflammation that underly the pathogenesis of leprosy reactions. Nevertheless, no biomarkers that can reliably predict the subsequent occurrence of leprosy reactions from non-reactional patients and distinguish type I reaction from type II have yet been found.

Case Report: Rapid Response to Low-Dose Thalidomide in a Case of Severe Steroid Recalcitrant Erythema Nodosum Leprosum

Erythema nodosum leprosum (ENL), or type 2 lepra reaction, presents with crops of evanescent, tender erythematous nodules accompanied by fever, arthralgia, weight loss, malaise, and organ-specific manifestations, and is seen in borderline and lepromatous leprosy. The drugs approved for ENL include nonsteroidal anti-inflammatory drugs, systemic steroids, thalidomide, and clofazimine. The management of ENL is challenging because long-term steroid use leads to steroid dependence. Our patient had severe steroid recalcitrant ENL with vesicular and pustular lesions mimicking Sweet’s syndrome, and was treated effectively with a low-dose thalidomide regimen (100 mg/d) as opposed to the high dose (400 mg/d) recommended in the literature. We discuss the patho-mechanics and clinical utility of a low-dose thalidomide regimen as an effective treatment option for ENL.