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.

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.

Case of leprosy mimicking preseptal cellulitis: a diagnostic dilemma

A 36-year-old Asian man presented with swelling over the left frontal region involving the upper eyelids, with associated erythema and tenderness for 1 month duration. Clinically he was diagnosed as a case of preseptal cellulitis, however, the lesion did not improve on broad-spectrum systemic antibiotics. CT showed superficial soft tissue swelling in the forehead extending till the superior part of orbit. Histopathological assessment of the lesion revealed clusters of epithelioid cells with multinucleate giant cells in the dermis along with perivascular and periadnexal lymphocytic infiltrates, suggestive of leprosy. The patient was started on oral steroids with multidrug therapy, following which the patient showed early resolution of the lesion within 10 days of treatment. Leprosy is endemic in India, leprosy with reactional episodes mimics other inflammatory and infective etiologies making diagnosis difficult. Leprosy should be present in an ophthalmologist’s diagnostic repertoire while dealing with periorbital swellings for early clinical diagnosis and favourable outcomes.

Placental pathology in a pregnant woman with severe COVID-19 and successful ECMO treatment: a case report

Background Infection with SARS-CoV-2 during pregnancy can lead to a severe condition in the patient, which is challenging for obstetricians and anaesthesiologists. Upon severe COVID-19 and a lack of improvement after multidrug therapy and mechanical ventilation, extracorporeal membrane oxygenation (ECMO) is introduced as the last option. Such treatment is critical in women with very preterm pregnancy when each additional day of the intrauterine stay is vital for the survival of the newborn. Case presentation We report a case of a 38-year-old woman at 27 weeks of gestation treated with multidrug therapy and ECMO. The woman was admitted to the intensive care unit (ICU) with increasing fever, cough and dyspnoea. The course of the pregnancy was uncomplicated. She was otherwise healthy. At admission, she presented with severe dyspnoea, with oxygen saturation (SpO2) of 95% on passive oxygenation, heart rate of 145/min, and blood pressure of 145/90. After confirmation of SARS-CoV-2 infection, she received steroids, remdesivir and convalescent plasma therapy. The foetus was in good condition. No signs of an intrauterine infection were visible. Due to tachypnea of 40/min and SpO2 of 90%, the woman was intubated and mechanically ventilated. Due to circulatory failure, the prothrombotic activity of the coagulation system, further saturation worsening, and poor control of sedation, she was qualified for veno-venous ECMO. An elective caesarean section was performed at 29 weeks on ECMO treatment in the ICU. A preterm female newborn was delivered with an Apgar score of 7 and a birth weight of 1440 g. The newborn had no laboratory or clinical evidence of COVID-19. The placenta showed the following pathological changes: large subchorionic haematoma, maternal vascular malperfusion, marginal cord insertion, and chorangioma. Conclusions This case presents the successful use of ECMO in a pregnant woman with acute respiratory distress syndrome in the course of severe COVID-19. Further research is required to explain the aetiology of placental disorders (e.g., maternal vascular malperfusion lesions or thrombotic influence of COVID-19). ECMO treatment in pregnant women remains challenging; thus, it should be used with caution. Long-term assessment may help to evaluate the safety of the ECMO procedure in pregnant women.

Lucio’s phenomenon: A systematic literature review of definition, clinical features, histopathogenesis and management

Leprosy is a chronic disease with clinical presentations according to the immunologic spectrum. Lepromatous form is the most advanced, with the highest transmissibility and risk of causing disabilities. Lucio’s phenomenon is a rare manifestation among lepromatous patients with a rapid and severe evolution and high mortality. It is difficult to differentiate from ulcerative/necrotic erythema nodosum leprosum and has no consensus on how it should be treated. This article is a qualitative review of the literature after the introduction of multidrug therapy, aiming to bring consensus related to the clinical, laboratory and histopathological diagnostic criteria of the disease and its management.

Giant diverticulum of coronary sinus with multiple accessory pathways treated with catheter ablation

Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.

Hanseníase no brasil: breve revisão

A hanseníase é uma infecção causada pelo Mycobacterium leprae, sendo que o Brasil encontra-se no segundo lugar globalmente entre os países com os maiores números de casos. Seu tratamento poliquimioterápico tem duração de seis (paucibacilar) ou 12 meses (multibacilar). Utiliza as medicações rifampicina, dapsona e clofazimina, combinadas com posologias de acordo com o tipo da doença e peso dos pacientes. Leprosy is an infection caused by Mycobacterium leprae, and Brazil is in second place globally among the countries with the highest number of cases. Its multidrug therapy treatment lasts six (paucibacillary) or 12 months (multibacillary). It uses the medications rifampicin, dapsone and clofazimine, combined with dosages according to the type of disease and weight of the patients.

Perception of cure among leprosy patients post completion of multi-drug therapy

Background Leprosy is a treatable disease; however, the release from treatment after completion of multidrug therapy (MDT) often does not equal absence of health problems. Consequently, sequelae interfere with the patient’s perception of cure. The objective of this study was to analyze the factors associated with the perception of not being healed among people treated for leprosy in a highly endemic area in Brazil. Method A cross-sectional study of perceived cure of leprosy in the post-release from treatment period was conducted in Cáceres in the state of Mato Grosso, Brazil. The study included a total of 390 leprosy patients treated with MDT and released after completion of treatment from 1 January 2000 to 31 December 2017. The dependent variable was self-reported cure of leprosy; the independent variables included clinical, operational and socioeconomic variables. Results Out of the 390 former leprosy patients, 304 (77.9%) perceived themselves as cured and 86 (22.1%) considered themselves unhealed. Among the latter, 49 (57.0%) reported muscle weakness and joint pains. Individuals with complaints related to leprosy post-release from treatment had a 4.6 times higher chance to self-report as unhealed (OR 4.6; 95% CI 2.5–8.5). Patients with physical disabilities (PD) grade 1 and 2 at the time of the study had a 3.1 (OR 3.1; 95% CI 1.3–7.4) and 8.8 (OR 7.7; 95% CI 3.5–21.9) times higher likelihood to self-identify as unhealed, respectively. Conclusion Among successfully treated leprosy patients, a quarter self-report as unhealed of the disease. The factors associated with the perception of being unhealed are PD and complaints related to leprosy in the post-release from treatment phase.