For more than two years, this 40-year-old African-American woman has had a lesion on her left maxilla. While it does not cause pain or discomfort, its presence is alarming to the patient. The lesion has grown steadily without responding to various topical OTC medications (tolnaftate, clotrimazole, miconazole, 1% hydrocortisone cream), oral anti-yeast medication, and antibiotics (fluconazole, erythromycin).
The patient claims to be in excellent health otherwise. She reports a strong family history of autoimmune disease, including rheumatoid arthritis and lupus.
There is an oval, scaly, atrophic patch on the upper left maxilla, just below the left nostril. The lesion measures 1.2 cm, has well-defined margins, and appears darker than the patient’s type V skin.
No redness or edema are seen, and no nodes are palpable in the area. Examination of the adjacent oral mucosal surface shows nothing amiss.
Under sterile conditions, and after local anesthesia (1% lidocaine with epinephrine) is administered, a 3-mm punch biopsy is obtained from the center of the lesion. The defect is closed with two interrupted nylon sutures.
What is the diagnosis? DISCUSSION
The results showed follicular hyperkeratosis, epidermal atrophy, acanthosis with basal-layer degeneration, periadnexal lymphocytic infiltrate, and increased mucin in the dermis. These findings—along with the clinical picture and family history—are consistent with discoid lupus erythematosus (DLE).
This manifestation of an autoimmune process is especially common in younger women of color. DLE primarily affects sun-exposed areas (eg, face, ears, neck) and involves scaly, round-to-oval patches and plaques with atrophic centers and follicular accentuation.
These lesions are frequently misdiagnosed as “fungal.” The differential also includes lichen planus and Jessner lymphocytic infiltrate.
Of the three types of cutaneous lupus erythematosus (discoid, acute, subacute), DLE is the most common. The acute form is defined by a “butterfly rash” across the face, while the subacute form involves multiple round-to-oval scaly lesions in wide photodistribution.
The chronic cutaneous lupus category includes DLE, tumid (characterized by deep, painful, indurated nodules), and panniculitis, which affects large areas of adipose tissue. DLE is, once again, the most common.
DLE can be an entity unto itself or part of a larger diagnosis of systemic lupus erythematosus (SLE). The good news: Only about 15% of patients with DLE progress to SLE. It is debatable whether patients with DLE need a full workup for SLE, since corroborative findings are rarely found.
Emphasis is placed on effective treatment of DLE, which includes the use of class 3 or 4 topical steroids and oral antimalarials (eg, hydroxychloroquine). Even with treatment, DLE can take weeks or months to resolve and often leaves permanent scarring. Ongoing sun protection is necessary to prevent recurrence.
TAKE-HOME LEARNING POINTS
- Discoid lupus erythematosus (DLE) is a common form of chronic cutaneous lupus, which has an autoimmune origin but is triggered by sun exposure.
- DLE typically presents with scaly round-to-oval patches and plaques with atrophic centers and follicular accentuation, often on sun-exposed areas.
- Women of color are at increased risk for lupus, and biopsy is often needed to confirm the diagnosis.
- Only about 15% of DLE patients ever progress to systemic lupus erythematosus (SLE), but DLE can be part of a larger SLE diagnosis.
- Treatment includes topical steroids and oral antimalarials (eg, hydroxychloroquine)—and sun protection is crucial.