Image IQ: Erythema on Nose, Cheeks, Upper Back

April 2, 2020

A 16-year-old African American girl reports sudden erythema covering her nose, cheeks, and upper back. Can you diagnose this patient?

A 16-year-old African American girl went to the doctor for what she described as the sudden appearance of erythema covering her nose, cheeks, and upper back. She said the rash appeared after a day trip to an amusement park where she was exposed to a lot of sun. The rash was associated with a few scaly plaques. Her mother is concerned because her daughter has also experienced weight loss. Her daughter also stated that she was constantly fatigued and has experienced joint pain and recurring headaches over the past few weeks.

Can you diagnose the patient? Use the differential builder in VisualDx as a guide.

A. Scleroderma
B. Sarcoidosis
C. Dermatomyositis
D. Systemic lupus erythematosus

See the next page for the correct diagnosis.

The correct diagnosis is D) Systemic lupus erythematosus

Synopsis:  Systemic lupus erythematosus (SLE) is a disease of unclear etiology characterized by immune abnormalities and multiorgan involvement. Approximately 15%-20% of patients with SLE will present during the first 2 decades of life. Childhood-onset SLE is a phenotype of SLE that presents in childhood or adolescence. It is relatively rare, with a worldwide prevalence estimated at 1.89-25.7 per 100 000 children. On average, it presents with a more aggressive phenotype than adult-onset SLE. Constitutional symptoms, fever, fatigue, anemia, weight loss, headache, mood disturbances, arthralgias, and skin findings may all be seen. Neurologic and renal involvement is a prominent characteristic in childhood SLE and often portends greater disease morbidity. Childhood SLE is also associated with chronic cardiopulmonary disease and macrophage activation syndrome.

The onset of the disease is usually between the ages of 10 and 15; however, it can occur at any age. Girls outnumber boys 8:1, but the ratio drops to 4:1 in adolescent cases. The disease is more common in people of color. It is found worldwide. Genetic factors play a role, and a family history of SLE or lupus erythematosus or an inherited complement deficiency in any form is a risk factor for developing the disease.

Childhood SLE is a chronic, noncurable disease that is treated with a variety of medications that impact immune function.

There are drug-induced forms of the disease with a differing pattern of autoimmunity and clinical profile.

To learn more about this condition, visit VisualDx.