Dermatologic Manifestations, Histologic Features and Disease Progression among Cutaneous Lupus Erythematosus Subtypes: A Prospective Observational Study in Asians

Abstract

Introduction Cutaneous manifestations are central to the primary diagnosis of systemic lupus erythematosus (SLE). However, information on the clinical, histopathologic, and direct immunofluorescence (DIF) features among subtypes of cutaneous lupus erythematosus (CLE), as well as longitudinal prospective observational study to evaluate the natural history and the progression to SLE, is lacking among Asians. Our objectives are to summarize the differences in the clinical, histopathologic, and DIF characteristics and serological profiles between various subtypes of CLE, and to provide its natural history and the association with disease activity in our Asian population. Methods A prospective observational study on CLE patients was performed between May 2016 and May 2020. Patients underwent full physical/dermatologic examination, skin biopsy for histology, and DIF. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores and laboratory data were evaluated. Time schedule and characteristics for resolution and/or the disease progression to SLE were recorded in subsequent follow-ups. Results Of 101 biopsy-proven CLE patients, 25 had acute CLE (ACLE), 8 had subacute CLE (SCLE), 39 had chronic CLE (CCLE) only, 22 had CCLE with SLE, and 7 had LE-nonspecific cutaneous lesions only. Patients with exclusive CLE showed lower female preponderance, serological abnormalities, and correlation to systemic disease. However, when CLE was accompanied with any LE-nonspecific cutaneous manifestations, they were associated with high antinuclear antibody (ANA) titer, renal, hematologic, joint involvement, and greater SLEDAI score. Of 207 biopsy sections, SCLE/CCLE regardless of systemic involvement showed significantly higher percentage of superficial/deep perivascular and perieccrine infiltration than ACLE. On DIF, deposition of multiple immunoreactants was associated with higher systemic disease. Approximately 10% of CLE-only patients later developed SLE but had mild systemic involvement. Conclusion Our findings support that each CLE subtype has a diverse and unique character. Comprehensive understanding of the differences among CLE subtypes is important for achieving the correct diagnosis and providing appropriate disease monitoring and management. Supplementary Information The online version contains supplementary material available at 10.1007/s13555-020-00471-y.

A Systematic Review of the Progression of Cutaneous Lupus to Systemic Lupus Erythematosus

“…In our review of prior studies, the most common risk factor reported was a positive ANA (15,17,21,28,35,41). Other common risk factors included hematologic abnormalities, age at CLE onset, lupus specific antibodies like dsDNA, and mucocutaneous criteria (15,21,25,28,35,38,41). Disparities in risk factor reporting can be attributed to differences in study design, population, and methods of reporting SLE diagnosis.…”

“…Four studies pre-dated the development of the 1982 ACR criteria and used ARA criteria ( 25 , 27 , 28 , 33 ). Two studies used the 2012 SLICC classification criteria ( 21 , 31 ). None have employed the 2019 EULAR/ACR criteria.…”

“…Heterogeneous data on risk factors for CLE to SLE progression and time to progression were available from a minority of studies. From the adult studies, the most common patient and clinical risk factors associated with SLE development included positive ANA (5/25), hematologic abnormalities (2/25), and number of classification criteria met at baseline (2/25) ( 15 , 17 , 21 , 25 , 28 , 35 ). Studies often differed on significant risk factors.…”

A Case of Progressive Thickening and Furrowing of Facial Skin and Scalp with Scarring Alopecia

“…However, our case and a previously reported case have brought attention to the association between scalp involvement of PDP and the development of scarring alopecia. Regarding histopathological features of our case, we theorize that the mechanism may involve the compression of hair follicles by surrounding thickening connective tissues and extracellular matrix, leading to follicular degeneration and further develop scarring alopecia [8][9][10][11][12].…”

Histopathological Review on Discoid Lupus Erythematous Mimicking Granuloma Faciale

“…Histopathological examination can be an alternative diagnostic tool to differentiate between various types of CLE, while serology profile has been identified as a marker of systemic involvement of CLE. 14…”