A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities.

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.

Dapsone Hypersensitivity Syndrome (DHS): A Detrimental Effect of Dapsone? A Case Report.

BACKGROUND: Dapsone is a sulfone derived drug used in the treatment of leprosy and several chronic inflammatory dermatological diseases. Dapsone Hypersensitivity Syndrome (DHS) is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy. It is rare and potentially fatal. CASE REPORT: We present a case report of a 52 years old female with a recent history of antecedent dapsone exposure of 100 mg daily for 2 weeks. She developed fever 10 days after exposure to dapsone therapy and was treated in various primary and tertiary centers for features of sepsis. When she presented to us, clinical features of multi-organ dysfunction and intractable sepsis was evident. She was successfully managed with intravenous corticosteroids and other supportive therapy. This case of DHS is unique due to pulmonary, hepatic and colonic involvement in addition to secondary bacterial and fungal infection, which is associated with an increased risk of mortality. CONCLUSION: As dapsone is mainstay in the treatment several infections and inflammatory conditions, further research is needed to characterize markers to diagnose DHS and to develop screening policies prior to initiation of dapsone therapy.

Lepra de Lucio y eritema necrotizante / No disponible

Se comenta la historia y características de la lepra de Lucio y eritema necrotizante y su preferencia geográfica por México, aunque se han descrito en otros países. Se expone la experiencia personal de dos casos de Lucio y 22 eritemas necrotizantes en enfermos lepromatosos no difusos y la evolución mortal en dos pacientes (AU)

We coment the history and characteristics of the Lucio's Leprosy and the Necrotrisans erythema and it's geographical preference by Mexico even though it is also spread out other countries. We expuse our personal experience of two Lucio's cases and the 22 Necrotisans erythema in lepromatous patients not difused and the mortal evolution in two patients (AU)

Agranulocitose induzida pelo uso de dapsona em paciente com hanseníase tuberculóide / Agranulocytosis induced by use of dapsone in pacient with tuberculoid leprosy

Relato de caso de paciente do sexo feminino, sob tratamento para hanseníase tuberculóide, em uso de 100mg/dia de dapsona, que desenvolveu agranulocitose e septicemia. Discutem-se os mecanismos envolvidos, as causas possíveis e o quadro clínico. Sugere-se cautela durante o uso dessa droga, principalmente nas primeiras 12 semanas de tratamento.

Septicemia com múltiplas lesöes ósseas supurativas como complicaçäo do eritema nodoso hansênico / Septicemia with suppurative bones lesions with complication of erithema nodosum leprosum

Um homem de 57 anos de idade, relata história de hanseníase com 26 anos de duraçäo e ainda em atividade. Desde a primeira consulta ao Hospital apresenta episódios de Eritema nodoso hansênico (ENH), frequentes, intensos, muitas vezes acompanhados por neurites, adenomegalia, icterícia e anemia. Desenvolveu lesöes ulcerativas de extremidades inferiores e superiores que evoluiram para infecçäo secundária, necrose e exposiçäo de planos músculo-tendinosos e ósseos. Na última internaçäo apresentou ainda episódios de ENH, mas predominou quadro clínico caracterizado por abscessos de regiöes glúteas; dores ósseas ao nível dos membros inferiores, crista ilíaca direta e tórax; dificuldade à micçäo com progressiva diminuiçäo do volume urinário; picos eventuais; melena; distensäo abdominal e nos últimos dias dificuldade respiratória. Os exames laboratoriais evidenciaram queda dos leucócitos no sangue (9.900-3.700 mm**3); osteoporose vertebral com colapso parcial dos corpos vertebrais D4, D5, D11 e sinais de osteomielite em tíbia e perônio. Na autópsia observou-se um paciente virchoviano em estado regressivo adiantado, com pequena quantidade de bacilos granulosos em troncos nervosos e linfonodo axilar. Näo se detectaram lesöes ativas de ENH em pele, nervos e vísceras, havendo apenas um foco de necrose e supuraçäo em organizaçäo em linfonodo axilar. O paciente faleceu em choque séptico decorrente de múltiplas necrotizantes e supurativas em corpos vertebrais, arcos costais e clavículas. A rotura de um abscesso costal no hemitórax direito levou à pleurite sero-fibrino-purulento, intensa. Observou-se trombose de veia axilar direita, de veia cava inferior junto à desembocadura de suprahepática, e trombose do átrio direito; colite aguda úlcero-flegmonosa e necrotizante ao nível de ceco; nódulo de Criptococose em pulmâo esquerdo, e hiperplasia nodular da próstata. Discute-se a relaçäo entre ENH e instalaçäo do quadro séptico, lembrando-se a possibilidade de infecçäo secundária nas lesöes cutâneas de ENH, de exaustäo da resistência imune frente a episódios sub-entrantes de ENH, e açäo dos corticosteróides na queda de resistência à microorganismos patógenos habituais ou oportunistas

Klebsiella ozaenae septicemia associated with Hansen's disease.

Two cases of Klebsiella ozaenae septicemia from the National Hansen's Disease Center, Carville, La., are discussed: one fatal and one nonfatal. Although both patients had nasal complications of Hansen's disease (leprosy), the organism was grown initially from spinal fluid, blood, and wound cultures. This report confirms the potential pathogenicity of the K. ozaenae species and its widening disease spectrum.

Lucio's phenomenon.

A 38-year-old woman had diffuse, nonnodular, lepromatous leprosy and Lucio's phenomenon. Most cases of Lucio's phenomenon have been reported to have a leukocytoclastic vasculitis as the underlying pathologic abnormality. In this patient, however, the histologic picture of an early lesion of Lucio's phenomenon showed a milk, mononuclear cell infiltration, endothelial swelling, vascular thrombosis, and ischemic necrosis. Lepra bacilli were abundant around nerves and blood vessels, and many were noted in vascular walls and endothelium. Our findings raise the possibility that some cases of Lucio's phenomenon may be caused by vascular damage due to direct invasion of Mycobacterium leprae and not necessarily by leukocytoclastic vasculitis.