A.S. Paller
Pediatric dermatologists often make the diagnosis of
rheumatologic disease because of the prominent
mucocutaneous manifestations in this group of disorders.
Four of the newer and now validated criteria for systemic
lupus erythematosus are dermatologic manifestations
(malar rash/photosensitivity; discoid rash; oral ulcerations;
nonscarring alopecia), and the risk of developing SLE when
the presentation is discoid lupus erythematosus is greater in
children than in adults. Lupus has been linked with activation
of interferon signaling, and new therapies that suppress
IFN are now being tested. Babies with neonatal lupus
most commonly present with annular lesions, and do not
have a risk of LE after clearance beyond that of family
members. The telangiectatic manifestations of juvenile
dermatomyositis are usually the earliest clue to diagnosis.
Early aggressive management is key to reduction in the
occurrence of calcifications and long-term sequelae.
Pediatric JDM patients generally do not show the myositisspecific
antibodies seen in adults, although newer antibodies
seen in JDM have been described in children. Linear
morphea is much more common in children than in adults,
and lead to problems when on the face or over joints.
Methotrexate remains the treatment of choice for complicated
morphea, although mycophenolate mofetil and UVA1
are additional useful monotherapy or adjunctive therapy.
Most patients have permanent sequelae and a long-term
risk of recurrence, leaving the optimal timing for cosmetic
correction still unclear.