A Case Series of Cutaneous Lupus Erythematosus Progressing to Systemic Lupus Erythematosus in a Lightning Phase

Abstract

Discoid Lupus Erythematosus (DLE) is a chronic, disfiguring, inflammatory skin disease characterised by erythematous, indurated, well-defined scaly plaques of varying sizes that resolve with atrophy, scarring, and pigmentary changes. Although discoid cutaneous lesions are typical of DLE, they are also seen in as many as 14% of patients with Systemic Lupus Erythematosus (SLE). The aetiopathogenesis of Cutaneous Lupus Erythematosus (CLE) is thought to be related to the same autoimmune abnormality responsible for the systemic components of LE. The key feature in the pathogenesis being upregulation of Interferon-α (IFN-α) signaling. Hence, the morphology and associated clinical features of DLE can be considered as a forerunner in assessing the development of SLE. This case series highlights some potential risk factors to watch out for in the progression to SLE in patients with DLE. Some parameters that are likely to predict early progression towards SLE from DLE are: early age at diagnosis (<25 years), female gender (2:1), Fitzpatrick skin type V and VI, presence of disseminated DLE lesions, arthralgia, anaemia (Hb-<10 g/dL), lymphopenia (lymphocyte count-<20%), isolated lesions at photoprotected sites, average disease duration ranging from 5.7 to 8 years, elevated ESR, high baseline ANA titres ≥1:320, anti-ds-DNA, anti-SS-A, and anti-Sm antibody positivity. Hence, assessment of the above-mentioned parameters in initial and follow-up visits might aid in the early diagnosis of SLE. This case series consists of four patients (33 years old female, 54 years old female,34 years old female and 49 years old male patients, all married), all of whom had multiple potential risk factors indicating rapid progression towards the development of systemic symptoms. Hence, this shifts the prior paradigm that DLE patients do not typically develop severe SLE. Risk score analysis may be a helpful tool in assessing ongoing subclinical inflammation and aiding in the early diagnosis of SLE. Appropriate therapeutic intervention could be key in stemming disease progression, reducing morbidity and mortality, and improving the patient’s quality of life.