Dr. Jean Bolognia and the Many Faces of Lupus

Source: Next Steps in Derm

This information was presented by Dr. Jean Bolognia at the 16th Annual ODAC Dermatology, Aesthetics and Surgical Conference held January 18^th-21^st, 2019 in Orlando, FL.  The highlights from her lecture were written and compiled by Dr. Daniel Yanes.

Just as systemic lupus erythematosus (LE) can have protean systemic manifestations, cutaneous LE can present in many different ways. When confronted with the many faces of mucocutaneous LE, the following pearls can be valuable.

1. Be Aware of the SLICC Criteria

In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) developed a set of clinical and immunologic criteria to assist in the diagnosis of systemic LE.

To meet criteria for systemic LE, a patient must fulfill at least four criteria, with at least one clinical criterion and one immunological criterion OR have biopsy-proven lupus nephritis in the presence of ANA or anti-dsDNA antibodies. Of the 11 clinical criteria, 4 are mucocutaneous: (1) acute or subacute cutaneous LE; (2) chronic cutaneous lupus; (3) non-scarring alopecia; and (4) oral or nasal ulcers. Of note, the dermatologist doesn’t just establish the diagnosis of cutaneous LE, but can also determine the specific types of autoantibodies the patient is forming as well as if the patient has systemic involvement (e.g. hematologic or renal abnormalities). Remember the latter requires a urinalysis in addition to bloodwork. Acute cutaneous LE is more closely associated with systemic LE than subacute cutaneous LE, which in turn is more closely associated with systemic LE than discoid LE. The cutaneous manifestations of LE per the SLICC classification scheme are as follows:

Acute cutaneous lupus

• Lupus malar rash (does not count if malar discoid)
• Bullous eruption of systemic LE
• Toxic epidermal necrolysis variant of systemic LE [also sometimes referred to as acute syndrome of apoptotic pan-epidermolysis (ASAP)]
• Maculopapular lupus rash
• Photosensitive lupus rash in the absence of dermatomyositis

Subacute cutaneous lupus

Chronic cutaneous lupus

• Classic discoid LE
• localized (above the neck)
• disseminated (above and below the neck)
• Hypertrophic (verrucous) LE
• Mucosal LE
• Lupus panniculitis (profundus)
• LE tumidus
• Chilblain lupus
• Discoid LE/lichen planus overlap

2. A Positive ANA Does Not Equate to Systemic LE.

While ANA titer is positive in nearly all patients with systemic LE, not all patients with a positive ANA titer have LE.

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