RESUMO

RESUMEN Las vasculitis leucocitoclásticas son grupos de procesos clinicopatológicos con heterogeneidad etiopatogénica y que requieren diagnóstico diferencial. En este reporte se presenta un caso de vasculitis leucocitoclástica de etiología no infecciosa sin germen aislado. Se revisan las posibles causas de este cuadro fundamentalmente cutáneo, así como su diagnóstico y tratamiento.

ABSTRACT Leukocytoclastic vasculitis is a group of clinicopathological processes with etiopathogenic heterogeneity that require differential diagnosis. This report presents a case of leukocytoclastic vasculitis of non-infectious etiology without an isolated germ. The possible causes of this fundamentally skin condition are reviewed, as well as its diagnosis and treatment.

RESUMO

RESUMEN La púrpura de Henoch-Schönlein en el adulto es un reto diagnóstico. Su baja incidencia y su sintomatología poco específica configuran un cuadro clínico que puede pasar desapercibido en diversas ocasiones o solaparse bajo el peso de diferentes sospechas diagnósticas. La púrpura de Henoch-Schönlein no es un cuadro de espectro único. Se considera un grupo de enfermedades de manifestación heterogénea con un eje patogénico común dado por el hallazgo de inflamación de la pared en vasos de pequeño calibre mediada por complejos inmunes. Este es el caso de un paciente de 70 arios quien cursa con un cuadro compatible con púrpura de Henoch-Schönlein, de inicio tardío, caracterizada por su difícil manejo y constantes recaídas. a pesar del uso cuidadoso de las pautas terapéuticas establecidas por los consensos actuales. En este paciente se documentó, de forma concomitante, una infección por citomegalovirus que al recibir tratamiento permitió el control adecuado de síntomas. Adicionalmente, este paciente presentaba una linfocitopenia que parecía ser secundaria a la infección viral.

ABSTRACT Henoch-Schönlein purpura in the adult is a diagnostic challenge. Its low incidence and its unspecific symptomatology in this age group, establish a clinical chart that can be ignored on several occasions. Henoch-Schönlein purpura is considered a group of diseases of heterogeneous manifestation with a common pathogenic axis: the finding of inflammation of the wall of the small calibre vessels, mediated by immune complexes. The case is presented of a 70-year-old patient with a difficult to treat Henoch-Schönlein purpura, with constant relapses despite the use of the therapeutic guidelines established in the current guidelines. In this patient, a concomitant cytomegalovirus infection was documented that, after receiving treatment, allowed adequate control of symptoms. Additionally, this patient also had a lymphocytopenia that was secondary to cytomegalovirus.

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We described a case of exuberant cutaneous small-vessel vasculitis in a 27-year-old male with mild CoVID-19 in Brazil. The patient presented painful purpuric papules and vesicobullous lesions with hemorrhagic content located in the larger amount in the lower limbs and, to a lesser extent in the region of the back and upper limbs, saving palms and soles of the feet. Influenza-like syndrome with anosmia and ageusia was reported seven days before the skin lesions. A real-time reverse transcription polymerase chain reaction was positive on a nasopharyngeal swab for SARS-CoV-2. Histopathological study showed leukocytoclastic cutaneous vasculitis affecting small vessels and microthrombi occluding some vessels. The patient presented an improvement in skin lesions by the fifth day of prednisone therapy. This case highlights the importance of the SARS-CoV-2 test in investigating the etiology of cutaneous vasculitis during this pandemic.

RESUMO

Se reporta un caso de edema agudo hemorrágico de la infancia, en un lactante de 18 meses, después de un episodio de otitis media. El cuadro comenzó con máculas eritematosas en los muslos, seguidas de lesiones purpúricas en los brazos, las piernas y edema en los tobillos. Se interpretó, inicialmente, como urticaria, por lo que recibió esteroides. Sin embargo, las características clínicas fueron de edema agudo hemorrágico de la infancia, una vasculitis leucocitoclástica benigna que se presenta en niños de entre 4 y 24 meses y que se caracteriza por fiebre, máculas y lesiones purpúricas. Estas se ubican, principalmente, en la cara, los lóbulos de las orejas y las extremidades, y se asocian, muchas veces, a edema. Los diagnósticos diferenciales son eritema multiforme, urticaria, vasculitis inducida por droga, enfermedad de Kawasaki, eccema infectado, meningococcemia y maltrato infantil, algunas de ellas, con riesgo de mortalidad. El manejo es conservador, sin embargo, los esteroides podrían ser una opción terapéutica.

We report a case of acute hemorrhagic edema of infancy in an 18-month-old boy after an episode of otitis media. The clinical presentation begins with skin erythematous macules on the thighs, followed by purpuric lesions in arms, legs, and ankle edema. It was initially interpreted as urticaria, whereby steroids were indicated. However, the clinical feature was acute hemorrhagic edema of infancy, a benign leukocytoclastic vasculitis that occurs in children between 4 and 24 months of age and is characterized by fever, large purpuric palpable target-like skin lesions affecting the face, lobes of the ears, limbs and frequently associated with edema. Differential diagnosis includes erythema multiforme, hemorrhagic urticaria, drug-induced vasculitis, Kawasaki disease, infected eczema, sepsis (either meningococcal or non-meningococcal) and child abuse. Some of them have risk of mortality. Management is conservative, however, steroids may be a therapeutic option.

Assuntos

RESUMO

We report a case of acute hemorrhagic edema of infancy in an 18-month-old boy after an episode of otitis media. The clinical presentation begins with skin erythematous macules on the thighs, followed by purpuric lesions in arms, legs, and ankle edema. It was initially interpreted as urticaria, whereby steroids were indicated. However, the clinical feature was acute hemorrhagic edema of infancy, a benign leukocytoclastic vasculitis that occurs in children between 4 and 24 months of age and is characterized by fever, large purpuric palpable target-like skin lesions affecting the face, lobes of the ears, limbs and frequently associated with edema. Differential diagnosis includes erythema multiforme, hemorrhagic urticaria, drug- induced vasculitis, Kawasaki disease, infected eczema, sepsis (either meningococcal or non-meningococcal) and child abuse. Some of them have risk of mortality. Management is conservative, however, steroids may be a therapeutic option.

Se reporta un caso de edema agudo hemorrágico de la infancia, en un lactante de 18 meses, después de un episodio de otitis media. El cuadro comenzó con máculas eritematosas en los muslos, seguidas de lesiones purpúricas en los brazos, las piernas y edema en los tobillos. Se interpretó, inicialmente, como urticaria, por lo que recibió esteroides. Sin embargo, las características clínicas fueron de edema agudo hemorrágico de la infancia, una vasculitis leucocitoclástica benigna que se presenta en niños de entre 4 y 24 meses y que se caracteriza por fiebre, máculas y lesiones purpúricas. Estas se ubican, principalmente, en la cara, los lóbulos de las orejas y las extremidades, y se asocian, muchas veces, a edema. Los diagnósticos diferenciales son eritema multiforme, urticaria, vasculitis inducida por droga, enfermedad de Kawasaki, eccema infectado, meningococcemia y maltrato infantil, algunas de ellas, con riesgo de mortalidad. El manejo es conservador, sin embargo, los esteroides podrían ser una opción terapéutica.

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This is the largest direct immunofluorescence (DIF) analysis of patients with histology-proven cutaneous leukocytoclastic vasculitis (LCV). To establish the correlation of deposition of immune complexes at the blood vessel walls with underlying causes and prognosis of LCV, we performed a retrospective study from January 2007 to December 2014. The patients are followed at the Department of Dermatology, Hospital Das Clínicas da Faculdade de Medicina da Universidade de São Paulo, a tertiary hospital at São Paulo, Brazil. We reviewed the data of 282 biopsy-proven LCV cases with DIF performed. For the statistical analysis, we included only patients with positive DIF exclusively in vessel walls (235/282 patients). We planned to find a correlation between the DIF profiles of LCV patients and the epidemiology data, underlying causes and prognosis. Ages ranged from five to 87 years old (yo), median age of 45 and 191/282 (67.73 %) were female individuals. DIF analysis showed positivity in 70.21 % of the samples, and C3 was the most frequent immunoreactant. Immunoglobulin A (IgA) deposition at the blood vessel wall was related to age and absence of autoimmune/inflammatory diseases. Immunoglobulin M (IgM) deposition at the blood vessel wall was related to females, autoimmune/inflammatory disorders, C3 and C4 consumption and antinuclear antibody and anti-SSA/anti-SSB positivity. Immunoglobulin G (IgG) deposition at the blood vessel wall was associated with age and positive ANCA; finally, C3 deposition at the blood vessel wall was associated with hematuria and renal involvement. Systemic involvement was present in 12.5 % cases of LCV patients. C3 deposits, the most frequent finding of this study, were related to renal involvement; IgA deposits to absence of autoimmune or inflammatory diseases; IgM deposition to the presence of autoimmune or inflammatory diseases and IgG deposits were associated with positive ANCA. DIF seems to be an important method to establish the prognosis and underlying etiology of LCV. Characterization of the immune complex at the blood vessel wall by DIF is relevant to determine underlying conditions related to LCV.

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Treatment with antithyroid drugs may be accompanied by side effects. We present a patient diagnosed with Grave's Disease who developed extensive vasculitis in the lower limbs during methimazole use. After suspension of the methimazole and the introduction of prednisone in immunesupressor doses the cutaneous lesions started to involute.

A terapêutica com drogas antitireoidianas pode ser acompanhada de efeitos colaterais. Apresentamos um caso de paciente com diagnóstico de Doença de Graves que na vigência da terapêutica com metimazol desenvolveu um quadro extenso de vasculite de membros inferiores. A partir da suspensão do metimazol e da introdução de prednisona em doses imunossupressoras as lesões cutâneas entraram em involução.

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