Los 100 años de Tratamiento de lepra en Fontilles: 2º parte / No disponible

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A case of lepromatous leprosy complicated by hemophagocytosis misdiagnosed as hemophagocytic lymphohistiocytosis.

Leprosy is an infectious chronic granulomatous disease caused by Mycobacterium leprae. The disease mainly affects the skin, peripheral nerves, mucosa, and viscera. The World Health Organization has reported that most countries with high endemicity have reached the goal of eliminating leprosy (defined as reaching a prevalence of <1 leprosy case per 10 000 population) at the national level, after years of proactive control campaigns. The incidence of leprosy has been decreasing across the globe year by year. However, misdiagnosis happens occasionally due to the complexity of clinical manifestations and lack of physician awareness of this disease. We report a case of lepromatous leprosy complicated by hemophagocytosis misdiagnosed as hemophagocytic lymphohistiocytosis.

Disseminated blastomycosis in a child with a brief review of the Indian literature.

Disseminated blastomycosis is rare in India, particularly in the pediatric population. We discuss the clinical picture, progress and outcome of disseminated blastomycosis in a 4-year-old child. We also present a brief review of the literature focussing on the scenario of blastomycosis in India.

Successful management of cryoglobulinemia-induced leukocytoclastic vasculitis with thalidomide in a patient with multiple myeloma.

Leukocytoclastic vasculitis (LV) is a systemic inflammatory disorder involving mostly the small vessels. It is characterised by segmental angiocentric neutrophilic inflammation, endothelial cell damage and fibrinoid necrosis. LV is related to a variety of clinical disorders including cryoglobulinemia and, very rarely, multiple myeloma (MM), among many others. The development of LV in patients with MM has been linked to cryoglobulinemia, infections, drugs and paraneoplasia. It has been speculated that myeloma patients with a poorer prognosis and progressive disease are more prone to develop LV. Thalidomide is a rediscovered old drug with anti-angiogenic, immunomodulatory and anti-inflammatory properties. It is highly effective in the treatment of MM and other clinical disorders such as leprosy, various cancers, graft-versus-host disease and autoimmune diseases. We report here a female patient with Durie-Salmon stage IIA MM who initially presented with cryoglobulinemia and LV. LV in this patient was primarily considered to be the result of progressive cryoglobulinemia, which was closely associated with MM. She was successfully managed with thalidomide and dexamethasone.

Deep vein thrombosis: a rare complication of thalidomide therapy in recurrent erythema nodosum leprosum.

A case of deep-vein thrombosis is reported in a female patient with multibacillary leprosy who received pulses of dexamethasone and cyclophosphamide for recurrent ENL that had not responded to prednisone and thalidomide.

Larva migrans visceral: forma mista de apresentaçäo em adulto: aspectos clínicos e laboratoriais / Visceral larva migrans: a mixed form of presentation in adult patient: clinical and laboratorial features

We relate a case of an 18-year-old man, resident of Xapuri (state of Acre, Brazil), with a history of repeated episodes of meningoencephalitis (three in one year), each one was examined by a local doctor. In our service (Emílio Ribas Institute of Infectology) we observed a patient with polyjoint aches, radiological and bronchoscopic pulmonary alterations (without clinical features), meningeal and brain stem manifestations--with normal brain computed tomography and cerebrospinal fluid. Blood eosinophils and serological Toxocara canis test (ELISA) were greatly increased. With the hypothesis of Toxocariasis (visceral larva migrans) we administered thiabendazole that brought complete clinical and laboratory remission. Inspite of a new episode of headache with meningeal manifestation approximately one month later (treated with dexamethasone resulting in a full remission after three days) we have not found other manifestations in approximately three and a half years of ambulatory care.

[Visceral larva migrans: a mixed form of presentation in an adult. The clinical and laboratory aspects]. / Larva migrans visceral: forma mista de apresentação em adulto. Aspectos clínicos e laboratoriais.

We relate a case of an 18-year-old man, resident of Xapuri (state of Acre, Brazil), with a history of repeated episodes of meningoencephalitis (three in one year), each one was examined by a local doctor. In our service (Emílio Ribas Institute of Infectology) we observed a patient with polyjoint aches, radiological and bronchoscopic pulmonary alterations (without clinical features), meningeal and brain stem manifestations--with normal brain computed tomography and cerebrospinal fluid. Blood eosinophils and serological Toxocara canis test (ELISA) were greatly increased. With the hypothesis of Toxocariasis (visceral larva migrans) we administered thiabendazole that brought complete clinical and laboratory remission. Inspite of a new episode of headache with meningeal manifestation approximately one month later (treated with dexamethasone resulting in a full remission after three days) we have not found other manifestations in approximately three and a half years of ambulatory care.

Chronic anterior uveitis in leprosy: an insidious cause of blindness.

Chronic low grade anterior uveitis is the commonest cause of blindness in leprosy. It is usually asymptomatic until the late stages, and often patients seek help only after irreversible visual impairment has occurred. We present herewith several cases of this entity to emphasise the insidious nature of the disease, the extent of ocular damage it can cause, and the importance of early detection and treatment.

Does dexamethasone suppress the Mazzotti reaction in patients with onchocerciasis?

The Mazzotti reaction is a frequent complication in patients with onchocerciasis being treated with diethylcarbamazine (DEC); and more severe manifestations of this reaction may be unacceptable in many patients. It has recently been demonstrated that prednisone modifies the severity of this reaction and reduces the microfilaricidal activity of DEC. A clinical trial was performed at the National Leprosy Training Center in Wau, Sudan, to evaluate the clinical and histologic effect of the use of corticosteroids in patients receiving DEC. Administration of a low dose of dexamethasone (3 mg/day), begun after onset of the Mazzotti reaction, modifies the progression of the Mazzotti reaction without interfering with the microfilaricidal efficacy of DEC. Pretreatment with low-dose dexamethasone--prior to beginning DEC therapy--prevents the development of the Mazzotti reaction and greatly reduces the microfilaricidal activity. Administration of diphenhydramine, after onset of the Mazzotti reaction, has no effect on the course and intensity of the Mazzotti reaction nor on microfilaricidal activity. We recommend that low-dose corticosteroids be administered in conjunction with DEC--after onset of the Mazzotti reaction--and that they be tapered rapidly.

La dexametasona en el tratamiento de las leprorreacciones

Se exponen los problemas que aún plantean las leprorreacciones recidivante y continuas, que las autores llaman "Status reaccional" y las posibles causas de él. A continuación se estudian 10 casos de enfermos lepromatosos (7 hombres y 3 mujeres) en leprorreaccion y que han sido tratados con Dexametosona (Millicorten Ciba) a dosis máxima de 4 mg. Los resultados han sido excelentes en cuanto a la remisión del cuadro clínico, considerándolo más activo y menos tóxico quelos restantes corticosteroides, aunque como ellos, en los casos de "Status reaccional", haya evitado las recidivas.