Clinico-pathological features of erythema nodosum leprosum: A case-control study at ALERT hospital, Ethiopia.

BACKGROUND: Leprosy reactions are a significant cause of morbidity in leprosy population. Erythema nodosum leprosum (ENL) is an immunological complication affecting approximately 50% of patients with lepromatous leprosy (LL) and 10% of borderline lepromatous (BL) leprosy. ENL is associated with clinical features such as skin lesions, neuritis, arthritis, dactylitis, eye inflammation, osteitis, orchitis, lymphadenitis and nephritis. ENL is treated mainly with corticosteroids and corticosteroids are often required for extended periods of time which may lead to serious adverse effects. High mortality rate and increased morbidity associated with corticosteroid treatment of ENL has been reported. For improved and evidence-based treatment of ENL, documenting the systems affected by ENL is important. We report here the clinical features of ENL in a cohort of patients with acute ENL who were recruited for a clinico-pathological study before and after prednisolone treatment. MATERIALS AND METHODS: A case-control study was performed at ALERT hospital, Ethiopia. Forty-six LL patients with ENL and 31 non-reactional LL matched controls were enrolled to the study and followed for 28 weeks. Clinical features were systematically documented at three visits (before, during and after predinsolone treatment of ENL cases) using a specifically designed form. Skin biopsy samples were obtained from each patient before and after treatment and used for histopathological investigations to supplement the clinical data. RESULTS: Pain was the most common symptom reported (98%) by patients with ENL. Eighty percent of them had reported skin pain and more than 70% had nerve and joint pain at enrolment. About 40% of the patients developed chronic ENL. Most individuals 95.7% had nodular skin lesions. Over half of patients with ENL had old nerve function impairment (NFI) while 13% had new NFI at enrolment. Facial and limb oedema were present in 60% patients. Regarding pathological findings before treatment, dermal neutrophilic infiltration was noted in 58.8% of patients with ENL compared to 14.3% in LL controls. Only 14.7% patients with ENL had evidence of vasculitis at enrolment. CONCLUSION: In our study, painful nodular skin lesions were present in all ENL patients. Only 58% patients had dermal polymorphonuclear cell infiltration showing that not all clinically confirmed ENL cases have neutrophilic infiltration in lesions. Very few patients had histological evidence of vasculitis. Many patients developed chronic ENL and these patients require inpatient corticosteroid treatment for extended periods which challenges the health service facility in resource poor settings, as well as the patient's quality of life.

Recognizing and managing the immunologic reactions in leprosy.

Immunologic reactions are an important aspect of leprosy that significantly impacts the course of the disease and the associated disability. Reversal reaction (type 1), erythema nodosum leprosum (type 2), and Lucio phenomenon are the 3 leprosy reactions, and they are most commonly seen in patients with the lepromatous and borderline categories of the disease. Because these forms of leprosy are the most common types seen in the United States, it is particularly important for physicians to be able to recognize and treat them. The reactions may occur before, during, or after treatment with multidrug therapy. Reversal reactions are the most common cause of nerve damage in leprosy, and erythema nodosum leprosum may also lead to neuritis. Although there have not been enough studies to confirm the most effective management regimens, treatment of reversal reaction and Lucio phenomenon with prednisone and of erythema nodosum leprosum with thalidomide and/or prednisone may help improve symptoms and prevent further disability.

Cutaneous vasculitides: clinico-pathological correlation.

BACKGROUND: Cutaneous vasculitis presents as a mosaic of clinical and histological findings. Its pathogenic mechanisms and clinical manifestations are varied. AIMS: To study the epidemiological spectrum of cutaneous vasculitides as seen in a dermatologic clinic and to determine the clinico-pathological correlation. METHODS: A cohort study was conducted on 50 consecutive patients clinically diagnosed as cutaneous vasculitis in the dermatology outdoor; irrespective of age, sex and duration of the disease. Based on the clinical presentation, vasculitis was classified according to modified Gilliam's classification. All patients were subjected to a baseline workup consisting of complete hemogram, serum-creatinine levels, serum-urea, liver function tests, chest X-ray, urine (routine and microscopic) examination besides antistreptolysin O titer, Mantoux test, cryoglobulin levels, antineutrophilic cytoplasmic antibodies and hepatitis B and C. Histopathological examination was done in all patients while immunofluorescence was done in 23 patients. RESULTS: Out of a total of 50 patients diagnosed clinically as cutaneous vasculitis, 41 were classified as leukocytoclastic vasculitis, 2 as Heinoch-Schonlein purpura, 2 as urticarial vasculitis and one each as nodular vasculitis, polyarteritis nodosa and pityriasis lichenoid et varioliforme acuta. Approximately 50% of the patients had a significant drug history, 10% were attributed to infection and 10% had positive collagen workup without any overt manifestations, while 2% each had Wegener granulomatosis and cryoglobulinemia. No cause was found in 26% cases. Histopathology showed features of vasculitis in 42 patients. Only 23 patients could undergo direct immunofluorescence (DIF), out of which 17 (73.9%) were positive for vasculitis. CONCLUSIONS: Leukocytoclastic vasculitis was the commonest type of vaculitis presenting to the dermatology outpatient department. The workup of patients with cutaneous vasculitis includes detailed history, clinical examination and investigations to rule out multisystem involvement followed by skin biopsy and DIF at appropriate stage of evolution of lesions. Follow up of these patients is very essential as cutaneous manifestations may be the forme fruste of serious systemic involvement.

[The sequelae of Hansen's disease. (Pathologic viewpoint of etiologies, morphologies and countermeasures)].

The proportion of glomerulonephritis, often a sequence of arteriolitis, among the sequelae of Hansen's disease after the introduction of chemotherapy increased markedly in Japan and nullified that of once prevalent tuberculosis after 1960s. However, most significant aftermath of the disease for numbers of years in the past have been peripheral nerve injuries worldwide for which effective countermeasures are yet to be developed. In this brief autopsy cases study from 1960s to 1990s, we confirmed the presence of cases in which arteriolitis and resulted infarction of peripheral nerves and not M. leprae itself were shown to be the major cause of axonal damages. There were also cases in which the accumulation of the bacilli without vascular changes did not damage the axons. The cases as these could not be solitary but should be rather common in this time of chemotherapy. If so, the methods to reconstruct nerves and blood vessels by promoting those regeneration should be developed to cope with the situation for surgeon, assisted by pathologists.

Las vasculitis necrotizantes en lepra multibacilar

Los autores realizan un estudio retrospectivo sobre 9 pacientes de Lepra Multibacilar que presentaron episodios de vasculitis necrotizante. Destacan la gran frecuencia con que la aparición de la vasculitis determinó el diagnóstico de lepra sólo al internarse el paciente en un medio especializado. Observan la frecuencia con que la aparición del cuadro podría haber sido inducida por la administración de medicamentos no específicos para la lepra. Estiman que sería conveniente alertar a los médicos generalistas en el diagnóstico precoz de la enfermedad y a tener en cuenta a la lepra como diagnóstico diferencial en las vasculitis necrotizantes en zonas de endemia (AU)

Lepromatous vasculitis successfully treated by plasma exchange (PE).

Vascular involvement is presently considered a "common pathway" in a number of diseases that is mediated by circulating immune complexes (CIC). CIC are found in the circulation when the disease is active and in single patients their level may parallel disease activity. Lepromatous leprosis is characterized by the presence of CIC and deposits of immunoglobulins and complement in vascular lesions of the different organs and an Arthus-like mechanism is considered as the basis for the clinical picture. The same mechanism is considered to play an essential pathophysiologic role in Lucio's phenomenon, which is characterized by lymphohistiocytic vascular infiltrates with or without thrombosis and secondary cutaneous infarction. Lepromatous vascular involvement is mediated by CIC whose antigen composition is known, the same as it is with HCV mediated cryoglobulinemia, HBV positive panarteritis nodosa, rheumatoid vasculitis, or Wagner's granulomatosis, which are usually treated by PE [1-3]. PE has been employed for lepromatous vasculitis since 1979 [4] and other cases have been successfully treated afterwards [5,6]. We report on another patient successfully treated by plasma exchange.

Lepra lepromatosa, fenómeno de Lucio, paniculitis mixta más vasculitis y daño renal

El fenómeno de Lucio es una leprorreacción necrotizante infreceunte que ocurre en la forma de lepra lepromatosa difusa e infiltrativa conocida como lepra de Lucio. Se presenta el caso de un paciente con lepra de Lucio quien en el transcurso de su enfermedad desarrolló glomerulonefritis proliferaliva, fenómeno de Lucio y eritema nodoso leproso. se consiguió una evolución terapéutica satisfactoria con farmacoterapia triconjugada convencional asociada con esteroides sintémicos

Lepra de Lucio en nuestro medio: presentación de un caso y consideraciones / Lucio's leprosy: a case in point

La lepra de Lucio es una rara variedad de lepra lepromatosa caracterizada por carecer de nódulos...

Lepra de Lucio en nuestro medio: presentación de un caso y consideraciones / Lucios leprosy: a case in point

La lepra de Lucio es una rara variedad de lepra lepromatosa caracterizada por carecer de nódulos...

Pterygium unguis in a patient with recurrent type 2 lepra reaction.

A 35-year-old man with long-standing lepromatous leprosy and history of recurrent, severe type 2 lepra reaction was found to have pterygium unguis and destruction of the fingernails. We propose that the obliterative angiitis and endarteritis due to severe type 2 lepra reaction were responsible for these nail changes.