Porfiria cutânea tarda e lúpus eritematoso sistêmico

Abstract

Abstract:The co-existence of systemic lupus erythematosus and porphyria although rare has been known for a long time. This association forces the physician to make a careful differential diagnosis of the bullous lesions that might appear in such patients and to be careful when prescribing certain drugs such as chloroquine. This drug, when used in the regular doses for treating lupus, may cause hepatotoxicity in patients.suffering from porphyria. It is described here the case of a patient with lupus who developed bullous lesions compatible with porphyria cutanea tarda. Keywords: Antibodies, antinuclear; Lupus erithematosus, systemic; Porphyrias; Porphyria cutanea tarda Resumo: A associação de lúpus eritematoso sistêmico e porfiria, embora rara, é conhecida de longa data. Ela obriga o médico a realizar um cuidadoso diagnóstico diferencial das lesões bolhosas nesses pacientes e tomar cuidados com a prescrição de certas drogas, como a cloroquina. Esta, nas doses habituais para tratamento do lúpus, pode causar hepatotoxicidade em pacientes com porfiria. Descreve-se o caso de uma paciente com lúpus que desenvolveu lesões bolhosas compatíveis com porfiria cutânea tardia.

Fotossensibilidade amplificada? Relato de porfiria cutânea tarda associada a lúpus eritematoso sistêmico

“…Regarding the fi rst hypothesis, the gene for the UROD enzyme, which is impaired in PCT, is known to be in chromosome 1, and another region of that same chromosome, 1q41-q42, has already been implicated in the immunogenetics of SLE. 7 Another fact supporting a common pathogenesis is the simultaneous occurrence of those diseases in some patients. 5 The second hypothesis assumes that photostimulated porphyrins act upon immune cells, leading to cell damage and exposure of autoantigens.…”

“…and should raise the suspicion of other entities, such as PCT, pemphigus, dermatitis herpetiformis, epidermolysis bullosa acquisita, and drug-induced lesions. 4,6,7 When the blisters are caused by lupus, they are usually chronic, disseminated, subepidermal, and leave no scar. 7 Our patient had histology of mesangial proliferative or class II lupus nephritis, 8 which usually manifests as non-nephrotic proteinuria, no change in arterial blood pressure, in complement, and in renal function, and negativity for anti-DNA (differently from that observed in our case).…”

“…4,6,7 When the blisters are caused by lupus, they are usually chronic, disseminated, subepidermal, and leave no scar. 7 Our patient had histology of mesangial proliferative or class II lupus nephritis, 8 which usually manifests as non-nephrotic proteinuria, no change in arterial blood pressure, in complement, and in renal function, and negativity for anti-DNA (differently from that observed in our case). The HCV, detected by use of serology and with an always negative quantitative CRP, can also trigger mesangial proliferative glomerulonephritis.…”

“…That risk is dose-dependent, and several authors have contraindicated the daily administration of antimalarials indicated for SLE, because of the risk of massive porphyrinuria (fever, nausea and hepatocelular injury, including hepatic necrosis). 1,4,5,7 Thus, in cases of the association of PCT and SLE, low doses of antimalarials (half of the usual dose, only twice a week) are recommended. 4 In clinical practice, a patient with lupus, signifi cant photosensitivity and blistering lesions should always be assessed for porphyrias, because of the therapeutic implication of the association of those diseases.…”

Porphyria cutanea tarda presenting as a lichenoid eruption

“…1 Likewise, while rare, systemic lupus erythematosus (SLE) and PCT have been reported to coexist. 5,6 A subtype of SLE, bullous lupus, can present with extensive vesiculobullous eruptions on both sun-exposed and non-exposed areas of the body. 5,7 While photosensitive and blistering conditions can be easily mistaken for PCT, lichenoid drug eruptions (LDEs) are thought to represent a different clinical entity.…”