Chronic Cutaneous Lupus Erythematosus

CCLE consists of scarring plaques.
DLE is, by far, the most common form of CCLE.
Other CCLE variants include hypertrophic lupus erythematosus, lupus erythematosus panniculitis, and lupus profundus.
DLE is a chronic, scarring, photosensitive dermatosis that may occur in approximately 25% of patients with SLE.
If the initial workup of patients who present solely with localized lesions of DLE shows no evidence of SLE, then those patients are considered to be at low risk (less than 5%) for SLE to ultimately develop.

Clinical Manifestations ⬆ ⬇

Lesions begin as well-defined, erythematous plaques that evolve into atrophic, disc-shaped plaques, characterized by scale, accentuated hair follicles, follicular plugging, and a combination of hypopigmentation and hyperpigmentation.
DLE often involves the scalp and produces scarring alopecia (Fig. 34.22), which can be quite extensive.
Lesions of DLE are relatively asymptomatic, but they may itch or be tender.
Rarely, squamous cell carcinoma develops into hypertrophic chronic lesions.

CCLE Variants

Hypertrophic lupus erythematosus is a warty-appearing form of CCLE.
Lupus erythematosus panniculitis is an inflammation of subcutaneous tissue.
When lupus panniculitis occurs with an overlying lesion of CCLE, it is then referred to as lupus profundus.

Diagnosis ⬆ ⬇

Differential Diagnosis

Cutaneous Sarcoidosis (see below)

Lichen Planus

Atrophic lesions may also closely resemble those of DLE (see image on page 4 Introduction).

Management

Broad-spectrum sunscreens that block both ultraviolet A (UVA) and ultraviolet B (UVB) are recommended.
Potent topical steroids are generally effective for treating isolated lesions. Facial lesions should be treated with low- to medium-potency agents. If necessary, high-potency or superpotent agents may be used for short periods.
Intralesional steroid injections are helpful in CCLE lesions that are refractory to topical therapy.
Systemic agents may be indicated when lesions are widespread or unresponsive to topical or intralesional therapy. Agents such as the antimalarials hydroxychloroquine (Plaquenil) and chloroquine (Aralen) comprise the first line of systemic therapy.
Systemic steroids, dapsone, oral retinoids, gold, intravenous immunoglobulin, clofazimine, cyclosporine, cyclophosphamide, low-dose methotrexate, thalidomide, tetracycline or erythromycin combined with niacinamide, and mycophenolate mofetil (CellCept), have proved to be helpful in selective cases.
Biological response modifiers with anti-tumor necrosis factor-alpha therapy, particularly adalimumab and infliximab, are promising options for patients with recalcitrant disease.

Helpful Hints

Sun protection whenever possible between 10 AM and 2 PM.
Encourage smoking cessation.
Most patients with CCLE do not have, and will not develop, SLE. Even so, many patients who are given the diagnosis of CCLE describe themselves as having “lupus” or systemic lupus erythematosus, and are mistakenly convinced that they have the more serious systemic disease.

Point to Remember