Cutaneous Lupus Erythematosus in Children

Abstract

Background: The skin is commonly involved in autoimmune diseases such as lupus erythematous. Cutaneous lupus erythematosus (CLE) can manifest with or without systemic symptoms. It is advantageous from a patient and healthcare system standpoint for early diagnosis and intervention. Prevention of complications is especially important in the pediatric population. Objective: To familiarize physicians with the clinical presentation, diagnosis, evaluation, and management of pediatric cutaneous lupus. Methods: The search term "cutaneous lupus" was entered into a Pubmed search. A narrow scope was applied to the categories of "epidemiology", "clinical diagnosis", "investigations", "comorbidities", and "treatment". Meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews were included. The search was restricted to the English literature and children. A descriptive, narrative synthesis was provided of the retrieved articles. Results: A variety of innate and adaptive immune responses are being investigated to explain the pathogenesis of CLE. There are a number of variations of cutaneous manifestations varying from localized malar rash as in the case of ACLE lesions and papulosquamous psoriasiform lesions as in the case of SCLE, to the multiple subtypes within chronic CLE. First-line pharmacological treatments include topicals such as typical calcineurin inhibitors and corticosteroids or oral agents such as glucocorticoids, antimalarial drugs, and hydroxychloroquine. Conclusion: CLE is inclusive of a number of subtypes that have varying dermatological manifestations in adult and pediatric populations. Current treatment modalities based on understood molecular targets will change. Ongoing research on the mechanisms underlying CLE is necessary to derive new interventions for pediatric patients.

Xeroderma pigmentosum: an updated review

“…The differential diagnosis includes Cockayne syndrome, XP/Cockayne syndrome complex, trichothiodystrophy, XP/trichothiodystrophy complex, Cockayne syndrome/ trichothiodystrophy complex, cerebro-oculo-facio-skeletal (COFS) syndrome, UV-sensitive syndrome, Bloom syndrome (also known as Bloom-Torre-Machacek syndrome or congenital telangiectatic erythema), Rothmund-Thomson syndrome, Hartnup disease, Carney complex, De Sanctis-Cacchione syndrome, erythropoietic protoporphyria, cutaneous lupus erythematosus and LEOPARD syndrome (also known as Noonan syndrome with multiple lentigines). [83][84][85][86][87][88][89][90][91][92][93][94][95][96] The distinctive features of many of these conditions help to differentiate them from XP.…”