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

Abstract

The association of porphyria cutanea tarda (PCT) and systemic lupus erythematosus (SLE) is rare. Systemic lupus erythematosus, of complex pathophysiology and pleomorphic clinical manifestations, is similar to PCT regarding photosensitivity. One fi nding that can differentiate both diseases is the presence of cutaneous blisters, which are rare in SLE, but characteristic of PCT. We report one case of the association of PCT and SLE and revise the literature, emphasizing pathophysiological, clinical and therapeutic aspects. One relevant information for clinical practice relates to the treatment of SLE with antimalarials, which is a risk for PCT.

Systemic Lupus Erythematosus and Porphyria Cutanea Tarda: A Case Report

“…Antimalarials (such as Hydroxychloroquine and Chloroquine), common to treat SLE, can often be contraindicated or indicated in reduced doses, as they increase the risk of dose-dependent porphyrinuria, generating fever, nausea, hepatocellular damage and liver necrosis. As a result, in cases where PCT and SLE are linked, low-dose antimalarials (half the typical amount, just twice a week) are indicated (FRITSCH et al, 2012).…”

“…Sun exposure must be considered for both diseases, which can be exacerbated by sun exposure (FRANK; GUTIÉRREZ, 2010). Therefore, at the time of diagnosis, one must remember the symptoms associated with SLE and PCT, which include photosensitivity and bullous lesions (FRITSCH et al, 2012).…”

“…The association between Systemic Lupus Erythematosus (SLE) and Porphyria Cutanea Tarda (PCT) is rare and difficult to differentiate diagnostically (FRITSCH et al, 2012). SLE is an inflammatory autoimmune disease with abnormalities in autoantibodies, such as antinuclear antibodies (ANA), which commonly has varied cutaneous manifestations and a broad clinical and laboratory presentation (BORBA et al, 2008).…”

“…PCT, the most common form of porphyrias, is a bullous disease caused by an enzymatic dysfunction, requiring laboratory confirmation through the measurement of porphyrins for its diagnosis (HERMOSILLA; TORO; MOLGÓ, 2018;VIEIRA;MARTINS, 2003). Both pathologies can manifest themselves through blisters, although this is less common in SLE, and photosensitivity (FRITSCH et al, 2012).…”