A real-world study of low-dose thalidomide in severe erythema nodosum leprosum highlighting its mechanistic rationale in a resource-constrained target population.

BACKGROUND: Corticosteroids remain the main therapy in erythema nodosum leprosum (ENL), and long-term usage in chronic or recurrent ENL is a cause of significant morbidity and mortality. Thalidomide exerts dramatic effect in controlling ENL and helps reduce the dose of steroids, but the cost is a hindrance to its usage. METHODS: Patients of ENL (steroid naïve and steroid-dependent) were recruited over a 1-year period. An escalating dose of low-dose thalidomide with a reducing dose of prednisolone was titrated depending on the control of disease activity. The primary aim was to reduce the dose of steroids to the lowest effective dose, and the secondary aim was to stop. RESULTS: Sixteen patients of ENL were studied (mean duration of ENL 22.1 months, 15 severe ENL), and a majority (11/16, 68%) were on steroids with a mean duration of 11.27 months. All patients had steroid-related side effects (cushingoid habitus 81.8%, weight gain 54.5%, diabetes mellitus 9%, hyperlipidemia 18.18%, cataract 18.1%, osteoporosis 36.3%, striae 36.3%, acneiform eruptions 18.1%, and myopathy 9%). Steroids could be tapered in a majority of patients (n = 9) within 3 months (mean 2.44 months) with a low dose of thalidomide (25-150 mg/day, mean 78.3 mg) achieving a significant reduction in prednisolone dose (33.16 mg at baseline; 4.28 mg at 3 months, P < 0.05). Steroids could be stopped in 92% of patients by 3.03 months, and both drugs could be stopped in 80% of cases by 5.83 months. CONCLUSION: The rapid and effective control of ENL with low-dose thalidomide in our series is comparable to the historical efficacy of high-dose thalidomide regimens, making it an affordable therapy in resource-constrained settings and an excellent steroid-sparing agent. The rapid onset of disease control is likely attributable to its action via neutrophils.

Lucio phenomenon with concomitant necrotizing fasciitis and acute kidney injury.

Lucio phenomenon is a rare vasculopathy that can occur in patients with Hansen disease, particularly diffuse lepromatous leprosy. It is characterized by retiform purpura and necrotic ulcerations, most commonly affecting the extremities. Diagnosing Lucio phenomenon can be challenging, especially when secondary bacterial infections occur. We report a patient with Lucio phenomenon who presented with acute necrotizing fasciitis of his left upper extremity and a 10-year history of chronic ulcerations. Shortly following admission, he also developed acute kidney injury. The necrotizing fasciitis was treated with prompt surgical debridement and intravenous antibiotics. Biopsy and PCR of a right upper extremity ulcer confirmed the presence of Mycobacterium lepromatosis. Multidrug therapy and prednisone were used to treat the Lucio phenomenon. After initiating treatment, no new lesions developed, kidney function improved, and the patient underwent successful skin graft of his left upper extremity. Although corticosteroid use is controversial, our patient's marked response to multidrug therapy with prednisone highlights the importance of this regimen in severe presentations of Lucio phenomenon. To the best of our knowledge, only two other cases of Lucio phenomenon confirmed to be caused by M. lepromatosis have been reported in living patients (rather than retrospectively identified post-mortem), underscoring the importance of the presented clinical course and treatment regimen.

Atypical presentations of erythema nodosum leprosum: Diagnostic and therapeutic aspects.

Classic erythema nodosum leprosum (ENL) is characterized clinically by abrupt eruption of tender erythematous nodules, papules and plaques. Variable atypical patterns have been described, for example pustular, bullous, ulcerative, necrotic and Sweet's syndrome-like ENL. We aim to review previously reported cases of atypical ENL addressing the diagnostic and therapeutic aspects of these uncommon presentations. A search of medical literature for all cases of atypical ENL was conducted in the PubMed database till 2020. Data of patients with atypical ENL were collected and analyzed to describe the epidemiological, clinico-histological and therapeutic features. The major five clinically described presentations of atypical ENL include vesiculo-bullous lesions (46 % of patients), ulcero-necrotic lesions (41 %), erythema multiforme-like lesions (28 %), Sweet's syndrome-like lesions (11 %) and pustules (9 %). The skin lesions were accompanied by fever and constitutional symptoms in all patients. Oral steroids and thalidomide were the main lines of therapy in most of the reported patients. Dermatologists and pathologists should keep in mind the clinical variability of ENL to avoid misdiagnosis and delayed management. Early recognition can help control disease progression and save the patients from further complications.

Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma.

Subcutaneous panniculitis-like T cell lymphoma is a rare subtype of cutaneous lymphomas with distinct clinical, histological and immunophenotypic characteristics, as well as an indolent clinical course. Rarely, it may be complicated with hemophagocytic lymphohistiocytosis: a hyperinflammatory syndrome which, if not diagnosed early, carries a dismal outcome. In this article, we describe a case of subcutaneous panniculitis-like T cell lymphoma in a middle-aged female patient which was complicated with secondary hemophagocytic lymphohistiocytosis with a favorable outcome following etoposide-based therapy. The various histological mimics of subcutaneous panniculitis-like T cell lymphoma and the management options are also briefly discussed.

Eritema Nodoso Leproso Forunculoide: Presentación de un caso / No disponible

La Lepra es una enfermedad infectocontagiosa crónica de larga evolución que puede presentar episodios sintomáticos agudos, en ocasiones muy severos, que obligan a buscar atención médica y que se conocen con el nombre de lepra reaccional o leprorreacciones. Diagnosticar lepra reaccional no es dificultoso frente a un paciente con diagnóstico previo de la enfermedad. Aún así, se deberá estar alerta ante la aparición de síntomas generales y/o manifestaciones viscerales, en ausencia de otros signos cutáneos de reacción. Por el contrario, su reconocimiento como primer motivo de consulta puede ser, en ocasiones, un desafío diagnóstico tanto para el dermatólogo como para el médico clínico. Presentamos un raro caso de un paciente de 84 años de edad, procedente de medio rural del Paraguay, diabética, que debutó con lesiones compatibles con forúnculos, tratada previamente como tal por internista y ante falta de mejoría remitida a nuestro servicio, llegando al diagnóstico clínico, histopatológico y baciloscópico de lepra reaccional: eritema nodoso leproso atípico, de tipo forunculoide

Leprosy is a chronic infectious disease that can have longstanding acute symptomatic episodes, in severe cases, requiring them to seek medical attention and are known by the name of leproreactions of leprosy reactions. Diagnosing leprosy reaction is not difficult compared to a patient with a previous diagnosis of the disease, Still, should be alert to the appearance of general symptoms and/or visceral manifestations, in the absence of other signs o skin reaction. On the contrary its recognition as a first reason for consultation can sometimes be a diagnostic challenge for the dermatologist to the clinician. We present rare case a 84 year old female from rural area of Paraguay, diabetic, which debuted with furunculoid lesions and consistent with that was previously treated by an Internist and because the lack of improvement she was referred to our department, arriving at clinical and histopathological diagnosis of atypical erythema nodosum of furunculoid type