Presented by: Jeanyoung Kim, MD and Carrie Ann Cusack, MD
Drexel University College of Medicine
May 16, 2016
Lupus panniculitis is a rare type of chronic cutaneous lupus accounting for only 1-3% of patients with cutaneous lupus. The association with systemic lupus ranges from 10-42% and most patients are women in their late 30s and early 40s. Subcutaneous nodules usually occur on the shoulders, proximal extremities, buttocks and face. Sparing of the distal extremities favors lupus panniculitis over other panniculitides, such as erythema nodosum or nodular vasculitis. Early inflammatory lesions of lupus panniculitis may be painful. Some nodules develop surface change resembling discoid lupus, in which case the term “lupus profundus” is employed. Lesions can progress to develop scarring, atrophy and calcifications.
On histopathology, lupus panniculitis demonstrates a lobular or mixed panniculitis. There is a lymphoplasmacytic infiltrate in the fat lobules, as well as lymphoid follicles with germinal centers, mucin deposition, calcification, and hyaline fat necrosis. Lesions with clinical surface change can show epidermal changes characteristic of discoid lupus on biopsy, such as follicular plugging and basement membrane thickening.
Treatment is most effective when initiated early in the disease course, with the overall goal being to reduce inflammation and scarring. Systemic therapy is usually required given the deep location of the inflammation. First-line treatment includes oral antimalarials such as hydroxychloroquine or chloroquine. Quinacrine may be added for additional therapeutic benefit. Smoking cessation should be emphasized since smoking has been associated with poor response to antimalarials. Other treatment options for recalcitrant disease include the immunomodulators thalidomide, mycophenolate mofetil, azathioprine, methotrexate and dapsone. Intralesional triamcinolone (5 mg/cc) can be used, but must be done with extreme caution to avoid atrophy and ulceration. Topical corticosteroids are of little benefit given the deep nature of the inflammation.
Unlike other forms of cutaneous lupus, lupus panniculitis occurs in sun-protected areas and patients do not complain of photosensitivity. Therefore, the role of sun protection in the treatment of lupus panniculitis remains to be elucidated but is always a prudent recommendation in any lupus patient. In cases of recalcitrant or progressive disease, it is prudent to re-biopsy new lesions to exclude other panniculitides such as subcutaneous panniculitis-like T-cell lymphoma.
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