Accelerating Healing and Relieving Pain: High-Intensity Laser Therapy for Paraneoplastic Cutaneous Vasculitis Associated with Multiple Myeloma.

BACKGROUND Leukocytoclastic vasculitis (LCV) is an atypical form of cutaneous paraneoplastic manifestation. Its association with multiple myeloma (MM) is even rarer and is associated with poor prognosis and short survival, regardless of the therapy instituted. Different treatment approaches are necessary. We present a case in which LCV was the first manifestation of MM, and high-intensity laser therapy (HILT) was used as an option to accelerate healing and control pain. CASE REPORT A 76-year-old woman presented with pain and paresthesia in her lower limbs, associated with palpable purpura. The clinical diagnosis was small-vessel vasculitis. Laboratory tests showed an elevated gamma globulin monoclonal peak on protein electrophoresis. The immunophenotypic study of bone marrow aspirates led to the diagnosis of MM. Due to pain refractory to conventional analgesics, and the progressive evolution of the lesions, despite corticosteroid therapy, we performed photo-biomodulation with a neodymium-doped yttrium aluminum garnet laser (Nd: YAG), wavelength 1064 nanometers, using a 7-mm probe and energy density 6 J/cm². After the first session, the patient was referred for pain management, and after 5 weeks, we observed complete healing in ulcerated lesions and involution of bullous lesions. CONCLUSIONS This case report shows the positive effects of the Nd: YAG laser in modulating healing and reducing pain. HILT is an innovative, non-invasive, and effective treatment and should be considered a promising technique to accelerate healing and controlling pain.

Systemic disease in leukocytoclastic vasculitis: a focus on direct immunofluorescence findings.

BACKGROUND: Direct immunofluorescence (DIF) panels are usually ordered for clinically suspected cutaneous vasculitis, but their positivity rate is variable, and their prognostic significance is not clear to date. OBJECTIVE: The study aims to investigate the systemic involvement rate in leukocytoclastic vasculitis (LCV) patients and the potential clinical and laboratory associations with systemic involvement, including DIF findings. METHODS: A retrospective study of patients with histopathologically proven cutaneous LCV examined in the dermatology department between 2013 and 2017 was performed. RESULTS: Of the 81 patients (mean age, 50.6 years), 42 (52%) were male. The mean time between the appearance of skin lesions and biopsy was 23.1 days, ranging from 2 to 180 days. DIF showed overall positivity of 90.1%, and C3 was the most frequent immunoreactant (82.7%). Any kind of extracutaneous involvement was present in 47 (58%) of patients, with renal involvement being the most frequent (53.1%), followed by articular (18.5%) and gastrointestinal (11.1%) involvement. The presence of renal disease was associated with the detection of IgG in the lesional skin (p = 0.017), and with the absence of IgM in the lesional skin (p = 0.032). There was a significant association between C3 deposition and joint involvement (p = 0.05). STUDY LIMITATIONS: This is a single-center study with a retrospective design. CONCLUSION: DIF seems to be a useful ancillary diagnostic tool in the evaluation of cutaneous vasculitis, but the relationship between DIF findings and systemic involvement needs to be further elucidated due to contradictory data in the current literature.

Erythema elevatum diutinum in a patient with rheumatoid arthritis.

Erythema elevatum diutinum (EED) is a rare cutaneous neutrophilic vasculitis with many associated diseases reported in the literature. We report a 65-year-old woman with painful and itchy lesions on her elbows, hands, knees, and foot for a year. Histopathologic examination confirmed the diagnosis of erythema elevatum diutinum and treatment with dapsone produced significant clinical improvement within few weeks. Erythema elevatum diutinum is a rare disease that should be considered in patients with violaceous nodular plaques located over the extensor regions of the limbs. Knowledge of this unusual pathology and its association helps to avoid misdiagnosis and late treatment.

Leukocytoclastic vasculitis in a patient with syphilis and HIV coinfection.

Leukocytoclastic vasculitis (LCV) is a small vessel vasculitis characterized by an inflammatory infiltrate composed of neutrophils with fibrinoid necrosis and "leukocytoclasia", a term that refers to nuclei disintegration into fragments. LCV is related to multiple conditions including ANCA-associated vasculitis, cryoglobulinemia, IgA vasculitis, infectious and systemic diseases such as rheumatoid arthritis and systemic erythematous lupus (SLE) as well as infections and malignancy. We describe the clinical case of severe systemic vasculitis in a young male patient with secondary syphilis and HIV coinfection manifested by cutaneous and neurological involvement, as well as peripheral necrosis that requires bilateral lower limb amputation. The skin biopsy revealed histopathological changes compatible with endarteritis obliterans and LCV related to treponemal infection. This case highlights the plethora of clinical manifestations of treponemal infection and the diagnostic challenge this poses in current clinical practice.

Crioglobulinemia: una entidad heterogénea / Cryoglobulinemia: a heterogeneous entity

La crioglobulinemia se define como la presencia de inmunoglobulinas en el suero que se precipitan reversiblemente a bajas temperaturas. Se la clasifica en tipos I, II y III, según las características de las inmunoglobulinas. La primera suele asociarse a enfermedades linfoprolife-rativas y las de tipos II y III, denominadas crioglobulinemias mixtas, a infección por el virus de la hepatitis C, seguida de las enfermedades autoinmunes. Las manifestaciones clínicas se relacionan con obstrucción intravascular en el caso de la crioglobulinemia de tipo I, mientras que las de tipos II y III se manifiestan con vasculitis por depósito de inmunocomplejos. El compromiso cutáneo es el hallazgo principal, seguido del articular, el neurológico y el renal. Se presentan 3 casos de crioglobulinemia que, por los datos de laboratorio y las enfermedades asociadas, difieren de la descripción clásica publicada en los textos.

Cryoglobulinemia is defined as the presence of immunoglobulins in serum that reversibly precipitate at low temperatures. It is classified into types I, II and III on the basis of immunoglobulin characteristics. Type I is associated with lymphoproliferative disorders, type II and III known as mixed cryoglobulinemia, are associated with hepatitis C virus infection and autoimmune diseases. Clinical manifestations are related with occlusion of small and medium blood vessels common in type I cryoglobulinemia while immune-mediated vasculitis is frequent in mixed cryoglobulinemia. Cutaneous damage is the main manifestation, followed by joint, peripheral nerves and renal involvement. We present three cases of cryoglobulinemia that differ from the literature due to their laboratory findings and associated diseases.

Dengue associated with severe cutaneous leukocytoclastic vasculitis and pericardial effusion: a case report.

Dengue is a viral disease, caused by an arbovirus of the genus Flavivirus. In Brazil, its incidence rate is high with a broad clinical spectrum. This report discusses a rare case of dengue associated with cutaneous leukocytoclastic vasculitis and pericardial effusion with eminence of cardiac tamponade in a previously healthy patient with no comorbidities. The serology for dengue was positive and the histopathological analysis of the cutaneous lesions confirmed the diagnosis of leukocytoclastic vasculitis. After receiving treatment, the patient's condition greatly improved.

Diagnóstico e terapêutica precoce de vasculite leucocitoclástica: relato de caso / Early diagnosis and treatment of Leukocytoclastic Vasculitis: case report

Resumo Paciente do sexo feminino, 46 anos de idade, procurou o pronto socorro do Hospital Municipal Universitário apresentando lesões necróticas em membros inferiores associadas à síndrome consumptiva. Após anamnese e exame físico, obteve-se o diagnóstico de vasculite leucocitoclástica de modo imediato e econômico por meio da utilização de algoritmo específico de vasculites primárias, permitindo início precoce da terapêutica adequada. A boa evolução do quadro clínico ratificou a necessidade de se obter diagnóstico definitivo e início rápido da terapêutica.

Abstract A 46-year-old female patient presented at the emergency department of a Municipal University Hospital with necrotic lesions in lower limbs associated with wasting syndrome. She was diagnosed with leukocytoclastic vasculitis after physical examination and history-taking in a fast and cost-effective manner, using an algorithm specifically for primary vasculitis, enabling early and appropriate treatment. The good clinical outcome demonstrates the need to quickly make a definitive diagnosis and start treatment.

Vasculitis crioglobulinémica con compromiso renal y síndrome de Sjögren / Cryoglobulinemic vasculitis with renal involment and Sjögren's syndrome

La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Edema agudo hemorrágico de la infancia: Lesiones alarmantes de un cuadro benigno. Reporte de caso / Acute hemorrhagic edema of infancy: alarming lesions of a benign condition. Case report

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.

[Acute hemorrhagic edema of infancy: alarming lesions of a benign condition. Case report]. / Edema agudo hemorrágico de la infancia. Lesiones alarmantes de un cuadro benigno. Reporte de caso.

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.

Clinicopathologic correlation of 282 leukocytoclastic vasculitis cases in a tertiary hospital: a focus on direct immunofluorescence findings at the blood vessel wall.

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.

Vasculitis leucocitoclástica / Leukocytoclastic Vasculitis

Introducción: la vasculitis leucocitoclástica constituye un proceso clínico patológico caracterizado por inflamación y necrosis de los vasos sanguíneos. Objetivo: describir las características clínicas de la vasculitis leucocitoclástica y el tratamiento utilizado. Resultados: se indicaron estudios de laboratorios: eritrosedimentación, células LE I, II y III, factor reumatoide, exudado faríngeo, U.S.A y biopsia de piel; arrojando este último vasculitis leucocitoclástica. Conclusiones: la vasculitis leucocitoclástica se conoce con el nombre de venulitis cutánea necrotizante y ocurre en asociación a enfermedades crónicas, puede ser precipitada por infecciones o drogas y desarrollarse por causa desconocida. Los fármacos y agentes terapéuticos que se describen son: penicilina, sulfonamida, tiamina, hidantoina, alopurinol, hidralazina propiltiuracilo y después de la estreptoquinaza recombinante, anticuerpos monoclonales y radioterapia(AU)

Introduction: Leukocytoclastic vasculitis is a clinicopathologic process characterized by inflammation and necrosis of blood vessels. Objective: Describe the clinical features of leukocytoclastic vasculitis and the treatment used. Results: Laboratory studies were initiated: ESR, LE cellsI, II and III, rheumatoid factor, throat swab, and skin biopsy; the latter showed leukocyte clastic vasculitis. Conclusions: leukocytoclastic vasculitis is known also as cutaneous necrotizing venulitis and it occurs in association with chronic diseases. Infections or drugs can precipitated it andit can be developed by unknown causes.The drugs and therapeutic agents described are penicillin, sulfonamide, thiamine, hydantoin, allopurinol, propylthiouracil and hydralazine after recombinantestreptoquinaza, monoclonal antibodies and radiotherapy(AU)

Henoch Schonlein purpura associated with bee sting: case report.

Henoch Schonlein purpura (HSP) is a common childhood vasculitis, characterized by a non-thrombocytopenic palpable purpura and systemic features. It can be triggered by conditions like infections and insect bites. We present the case of a six-year-old girl with palpable maculopapular lesions on the lower limbs, itching, mild pain, swelling of feet, limitation of limb mobility, and a history of bee sting. Thigh skin biopsy was performed, with a report of leucocytoclastic vasculitis, and was diagnosed as HSP. She was prescribed bed rest, and was given oral hydration. The patient outcome was favorable and was discharged after five days. This is the fifth report of a HSP case associated with a bee sting with an uncomplicated course, which is in contrast to previous case reports.

La púrpura de Henoch Schonlein es una vasculitis común en la infancia, caracterizada por una púrpura palpable no trombocitopénica y manifestaciones sistémicas. Está relacionada con patologías desencadenantes como las infecciones y las picaduras de insectos. Presentamos el caso de una escolar de 6 años, de sexo femenino, con lesiones maculopapulares palpables en miembros inferiores, prurito, dolor leve, edema de pies, limitación al movimiento y antecedente de picadura de abeja. Se realizó biopsia cutánea de muslo con reporte de vasculitis leucocitoclástica y se diagnosticó como púrpura de Henoch Schonlein. Se indicó reposo y se le administró hidratación oral. Evolucionó favorablemente y fue dada de alta a los cinco días. Este es el quinto caso de púrpura de Henoch Schonlein asociado a picadura de abeja, la que se presentó sin complicaciones a diferencia de los reportes de caso previos.