The skin can offer clues to help make the correct diagnosis for many pediatric rheumatology diseases, and a session at ACR Convergence 2020 shared some of the most important things to look for when making a diagnosis.
Skin Deep: Derm Findings in Pediatric Diseases used four specific cases to illustrate the important presentations in many diseases, including pediatric systemic lupus erythematosus (pSLE), juvenile dermatomyositis (jDM), systemic juvenile idiopathic arthritis (sJIA), and psoriatic juvenile idiopathic (PsJIA). Registered ACR Convergence 2020 attendees can watch the session through March 11.
Pediatric nurse practitioner Annelle Reed, MSN, CRNP, Children’s of Alabama, started each of the cases with a look at each case’s specifics and later reviewing the specific treatment plans and outcomes for all four patients. Board-certified pediatric dermatologist Amy Theos, MD, associate professor, University of Alabama-Birmingham, discussed details about each disease and what rheumatologists can learn from each case.
The cutaneous domain of pSLE includes four elements, Dr. Theos said: Non-scarring alopecia, which has replaced photo sensitivity in the recently revised EULAR/ACR classification for SLE; oral ulcers; subacute cutaneous or discoid lupus, but it is quite uncommon in children; and acute cutaneous lupus.
Acute cutaneous lupus, or malar rash, is the most common lupus-specific rash in pSLE, Dr. Theos said. It’s also highlight photosensitive.
“Importantly, it spares the nasolabial folds, for reasons we don’t understand, which can help differentiate it from the facial erythema that we can see in our patients with dermatomyositis,” Dr. Theos said.
In jDM, specific skin findings include heliotrope rash and Gottron’s papules or sign. jDM patients are also photosensitive and can develop red to violet erythema on the face or other sun-exposed skin, Dr. Theos said.
“Pruritus, or itching, is a frequent complaint in these patients, which can help differentiate this from lupus rashes, which are generally asymptomatic,” Dr. Theos said. “Children with dermatomyositis have higher rates of developing calcinosis cutis as well as vasculopathy, which manifests as dusky skin lesions, skin ulcerations, and generally indicates more severe or life-threatening disease.”
Recommended treatments include aggressive photoprotection, antipruritic agents, topical corticosteroids and calcineurin inhibitors, and oral therapies beginning with hydroxychloroquine for mild skin disease.
Skin rash is one of the most common extra-articular signs in sJIA, Dr. Theos said. While the morphology of the rash is not very specific, the timing of the rash is extremely important.
The rash often coincides with fevers, Dr. Theos said, and is most common on the trunk, proximal extremities, and cheeks. It also is usually not pruritic, and rashes caused by skin trauma or scratching have a linear appearance.
“Lymphadenopathy can be quite impressive in patients with systemic JIA, and these patients may be mistakenly referred to oncology for concern for lymphoma,” Dr. Theos said.
PsJIA accounts for about 5% of JIA cases, and arthritis precedes psoriasis in 80% of children. Diagnostic criteria are arthritis in the presence of psoriasis and arthritis with at least two of the following: Nail pits, onycholysis, dactylitis, and first-degree family history of psoriasis.
Treatment depends on location, extent of the disease, and whether the child has arthritis or other comorbidities. First-line treatments for localized PsJIA are mid- to high-potency topical corticosteroids, followed by and often in conjunction with topical calcipotriene and calcitriol, tar-based treatments, and keratolytics.
“The thing with psoriasis is that it can be quite focal and easy to miss,” Dr. Theos said. “Or it can be quite obvious and difficult to miss. So, it is important to do thorough skin exams in these patients. In patients with darker skin tones, the erythema as well as the scale can be difficult to appreciate.”