Púrpura de Schönlein-Henoch Buloso. Caso clínico / Bullous Henoch-Schönlein purpura. Case report

Resumen: Introducción: El púrpura de Schonlein-Henoch (PSH) O Vasculitis IgA es la vasculitis sistémica más frecuente de la edad pediátrica. Se manifiesta clínicamente como púrpura palpable, artralgias, dolor abdominal y compromiso renal. El púrpura palpable buloso a diferencia de lo que ocurre en la edad adulta, es muy infrecuente en la infancia. Objetivo: Reportar una forma infrecuente de presentación cutánea del PSH en niños. Caso clínico: Niña de 14 años con historia de 2 semanas con ampollas dolorosas múltiples y confluentes en ambas extremidades inferiores asociado a artralgias. A la histo-patología destacan vesículas intracórneas, epidermis con acantosis y espongiosis e infiltrado dérmico perivascular. Inmunofluorescencia directa (+) para IgA. Se plantea el diagnóstico de PSH ampollar y se realiza tratamiento inicial con corticoides intravenosos. A los tres días del traslape a corticoides orales aparecen nuevas lesiones equimóticas en ambas piernas. Se decide asociar azatriopina e iniciar descenso de corticoides, obteniéndose buena respuesta. Conclusión: Si bien la formación de bulas en el PSH no agrega morbilidad, suele ser un fenómeno alarmante que requiere realizar diagnóstico di ferencial con otras patologías. El uso de corticoides estaría indicado en estos casos ya que disminuiría la producción de las metaloproteinsas responsables de la formación de las bulas.

Abstract: Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. Objec tive: To report an unusual cutaneous manifestation of HSP in children. Case report: A 14-year-old girl complained about a 2-week painful bullous rash in both lower extremities and multiple arthral gias. There was no history of abdominal pain or urinary symptoms. In both lower extremities, there were numerous palpable purpura and hemmorrhagic bullae. In light of clinical findings, laboratory tests and skin biopsy are requested. The histopathology described intraepidermal blisters, acanthosis, spongiosis and perivascular dermal infiltrate. Direct immunofluorescence (IFD) (+) for IgA. The diagnosis of bullous HSP was made and treatment with endovenous corticosteroids was initiated. Three days after overlapping to oral corticosteroids, new ecchymotic lesions appeared in both legs. Due to the persistence of cutaneous involvement and negative control tests, azathioprine was associa ted obtaining a good response. Conclusion: Although bullous lesions in HSP does not add morbidity, it is often an alarming phenomenon with multiple differential diagnoses. The anti-inflamatory effect of corticoids is likely to be beneficial in the treatment of patients with severe cutaneous involvement through inhibition of proinflammatory transcription factors and decreasing the production of the metalloproteinases.

Undiagnosed purpura: a case of autoerythrocyte sensitization syndrome associated with dermatitis artefacta and pseudo-ainhum.

A 23-year-old young woman presented with recurrent episodes of painful bruising along with linear erosions on the accessible areas of the body of nine years duration with a pseudo-ainhum of her left nipple for the past three months. Her case history included repeated visits to various physicians at different centers and an extensive investigative profile. A diagnosis of autoerythrocyte sensitization was made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any organic cause for her ailment. She was placed on psychiatric management and has remained symptom-free after six months follow-up. The case is reported for its rarity, as well as for the association of autoerythrocyte sensitization syndrome with frank dermatitis artefacta and pseudo-ainhum, which to the best of our knowledge has not yet been reported in the literature.

Pneumococcemia com características clínicas de meningococcemia: relato de três casos associados com "purpura fulminans" / Pneumococcal infections with clinical characteristics of meningococcal infections: report of 3 cases associated with purpura fulminans

Säo apresentados três casos de pacientes adultos com pneumococcemia fulminante simulando meningococcemia. Estas säo situaçöes clínicas extremamente graves, sobretudo quando associadas à falência múltipla orgânica. Seriam tais lesöes produzidas por fenômenos citotóxicos diretamente dos germes ou seriam conseqüentes à virulência e à secreçäo de toxinas por parte dos mesmos? Atualmente, nestas e em outras infecçöes catastróficas, têm sido enfatizadas como causas dos maiores danos ao hospedeiro suas próprias reaçöes inflamatórias e imunológicas que poderiam ser exageradas, determinando elevada liberaçäo de citocinas, as quais poderiam ser responsáveis pelas graves lesöes tissulares, inclusive pela própria falência múltipla orgânica. O papel dos superantígenos também tem sido valorizado nestas dramáticas situaçöes clínicas.

Crioglobulinemia mista policlonal associada a hepatite crônica persistente / Polyclonal mixed cryoglobulinemia associated with persistent chronic hepatitis

Relata-se caso de paciente portadora de hepatite crônica persistente, que após cinco meses do quadro da hepatite desenvolveu crioglobulinemia mista policlonal, na forma de síndrome de Meltzer. Concluiu-se que muitos casos de "crioglobulinemia mista essencial", na verdade, säo secundárias a doenças hepáticas preexistentes

Febre purpúrica do Brasil

Os autores relatam sua experiência com Febre Purpúrica do Brasil. Abordam de modo sumarizado a epidemiologia, microbiologia do Haemophilus aegyptius, a modificaçäo de sua açäo patogênica atribuída a um plasmídeo, o quadro clínico dos casos e a fisiopatologia, procurando correlacioná-la aos sinais e sintomas constatados e achados de necrópsia. Apresentam o esquema de tratamento e a profilaxia, adotados durante o surto de Serrana, SP

Síndrome hipereosinófílico de tipo purpúrico / Hypereosinophilic syndrome of purpuric type

El síndrome de hipereosinofílico (SH) se caracteriza por eosinofilia de causa desconocida y disfunción orgánica sistémica. Diversas formas de compromiso cutáneo (Dermatitis hipereosinofílica) se han observado en este síndrome. Publicamos el caso peculiar de una paciente de 40 años, que presentaba un cuadro purpúrico en napas, no referido en la literatura consultada