Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre

Abstract

Background Paediatric cutaneous lupus erythematosus (LE) is uncommon and inadequately described in the literature. Similar to adults, children with cutaneous LE develop LE-specific and/or LE-nonspecific skin findings. Similarities and differences in demographics and clinical course between paediatric and adult cutaneous LE have not been sufficiently described. Objectives The purpose of this study is to detail the demographic and clinical features of paediatric cutaneous LE and then compare these findings to those reported in the adult literature. Methods A retrospective chart review was performed of 53 children seen in a paediatric dermatology clinic with cutaneous manifestations of LE. Results Patients presented with all five major subtypes of cutaneous LE, with some notable differences from adult cutaneous LE and previously published reports of paediatric cutaneous LE. Progression from discoid LE to systemic lupus erythematosus (SLE) did not occur in our cohort. Patients with subacute cutaneous LE were more likely than adults to have lesions below the waist as well as concomitant SLE. Sex distribution for cutaneous LE in our study was equal prior to puberty and female-predominant in post-pubertal patients. Conclusions Children with cutaneous LE have variable clinical presentations and progression to SLE that may be different from adult disease. Specifically, children with acute and subacute cutaneous LE may be more likely than adults to have systemic disease; therefore, patients with these subtypes should be monitored closely for evidence of SLE. Study limitations included small patient numbers that may limit ability to generalize this data and relatively short follow-up intervals.

The natural history of pediatric-onset discoid lupus erythematosus

“…15,[21][22][23]26 Overall, 38% of patients developed SLE, 15% of whom were given the diagnosis of DLE as a manifestation of concurrent SLE and 26% of whom developed progression to SLE, consistent with the up to 25% rate of progression noted in prior pediatric studies of DLE. 15,23,27 Patients with SLE progression were at greatest risk within the first year after diagnosis, although mean follow-up duration was limited to 5.0 years.…”

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

“…LE specific skin lesions Malar rash44-85% [2, 5, 6]40-52% [5, 6]Generalized lupus rash30% [28]N/A [5, 6]Subacute cutaneous LERare [8, 33, 34]7-27% [8, 9]Discoid rash<10% [1, 2, 4, 18, 28, 29]20-50% [5, 36]Generalized DLE10-37% [31, 34–36]40-49% [36]Lupus panniculitis/ profundus<1% [34, 37]1-3% [10] 2. LE nonspecific skin lesions Cutaneous vasculitis16-45% [11, 21, 47]11-70% [5, 7, 12, 13]Photosensitivity35-50% [2, 4, 5]63% [7]Oral and nasal ulcers20-40% [2, 4, 5, 49, 52]18-30% [6, 7, 49, 52]Non-scarring alopecia15-30% [2, 5]25-55% [2, 5, 53]Livedo reticularis6-12% [14, 30, 33]22-35% [12]Raynaud’s phenomenon6-12% [15, 16]10-45% [15, 16, 60, 61]Bullous SLE…”

“…They are particularly common on the face and upper extremities and usually heal without scaring [32]. Skin involvement in of the lower extremities is rare in adults but is more common in children [33]. …”

“…This is a rare presentation in children particularly below the age of 10 [12, 22, 23, 26]. Discoid lupus has rarely been reported in children without systemic symptoms [28, 33, 34]. These lesions occur most commonly on the scalp (vertex), face and ears.…”

Recent advances in paediatric dermatology

“…Recent work by Dickey et al reviewed the clinical characteristics of cutaneous lupus erythematosus at a paediatric referral centre 18. All subtypes were represented: acute (eg, malar rash); subacute; and chronic or discoid lupus.…”