Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel

Abstract: A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity.

Livedoid vasculopathy as a marker of systemic disease: report of two cases

The livedoid vasculopathy is an obstructive vascular process of etiology not yet fully known, being possibly associated with several prothrombotic events. It is clinically characterized by the presence of painful and recurring purpuric lesions, which usually suffer ulceration and evolve with formation of white atrophic scars usually located in the lower limbs. Two cases are here reported of painful ulcerated lesions on the lower limbs, in which the identification of VL enabled the diagnosis of systemic diseases.

Livedo reticular associado com Anemia Hemolítica Autoimune: remissão prolongada induzida pelo Transplante de Células-Tronco do Sangue Periférico com recaída após 10 anos e restauração dos níveis de hemoglobina por rituximabe / Livedo reticularis associated with autoimmune hemolytic anemia: prolonged remission induced by peripheral blood stem cell transplantation relapse after 10 years and restoration of hemoglobin levels by rituximab

A anemia hemolítica autoimune (AHAI) é uma doença na qual são produzidos anticorpos diretamente contra as glicoproteínas adsorvidas na superfície da membrana dos eritrócitos. Algumas medicações e outras associações têm sido implicadas. Descrevemos e discutimos um caso de livedo reticular associado à AHAI tratado com transplante de células-tronco de sangue periférico (TCTSP) e que entrou em total remissão por 10 anos. Após esse período, a paciente apresentou recaída, foi tratada com anticorpo anti-CD20 (rituximabe), e atualmente encontra-se em total remissão. O papel do TCTSP e o uso de rituximabe no tratamento de AHAI serão discutidos neste relato de caso.

Autoimmune hemolytic anemia (AIHA) is a disease where patients produce antibodies against erythrocytes directed towards membrane glycoproteins adsorbed onto the erythrocyte surface. Drugs and other associations have been implicated. It is described and discussed a case of livedo reticularis associated with AIHA treated with peripheral blood stem cell transplantation (PBSCT) that went into full remission for 10 years. After that period the patient relapsed and was treated with antibody anti-CD20, rituximab, and is now in full remission. The role of PBSCT and rituximab in the treatment of AIHA will be discussed.

Vasculopatia livedoide: uma doença cutânea intrigante / Livedoid vasculopathy: an intringuing cutaneous disease

A vasculopatia livedoide é uma afecção cutânea oclusiva dos vasos sanguíneos da derme, de caráter pauci-inflamatório ou não-inflamatório. Caracteriza-se pela presença de lesões maculosas ou papulosas, eritêmato-purpúricas, nas pernas, especialmente nos tornozelos e pés, as quais produzem ulcerações intensamente dolorosas, que originam cicatrizes atróficas esbranquiçadas, denominadas "atrofia branca". Nesta revisão, abordamos os estudos e relatos de caso da literatura médica referentes às associações etiopatogênicas da doença, particularmente as que se referem aos estados de trombofilia, seus achados histopatológicos e abordagens terapêuticas empregadas na difícil condução clínica destes casos.

Livedoid vasculopathy is a skin disease that occludes the blood vessels of the dermis. It has a pauciinflammatory or non-inflammatory nature. It is characterized by the presence of macular or papular, erythematous-purpuric lesions affecting the legs, especially the ankles and feet, and producing intensely painful ulcerations, which cause white atrophic scars called "atrophie blanche". This review includes studies and case reports found in the medical literature regarding the etiopathogenic associations of the disease, particularly those related to thrombophilia, their histopathological findings and the therapeutic approaches used in the difficult clinical management of these cases.