Lupus panniculitis (lupus profundus)
New York University Departent of Dermatology
This 45-year-old woman presented with a three-month history of multiple, asymptomatic, erythematous plaques and nodules on the lower extremities. Ten years previously, while living in Russia, she had a similar episode that resolved spontaneously and resulted in atrophic, non-erythematous plaques on her upper legs. She denied arthralgia, arthropathy, myalgia, fatigue, fever, Raynaud's phenomenon, gastrointestinal symptoms, and lesions occurring on the head, trunk, and upper extremities. Treatments included intralesional triamcinolone, indomethacin, and hydroxychloroquine.
Multiple, erythematous nodules and indurated plaques were present on the lower extremities. On the right thigh and medial left knee there were skin-colored, atrophic, scarred plaques.
The erythrocyte sedimentation rate was 3 mm/hr. A complete blood count with differential analysis, electrolytes, blood urea nitrogen, creatinine, liver function tests, urinalysis, and a chest radiograph were normal. Antinuclear antibody titer was greater than 1:80 with a speckled pattern. Antibodies to double-stranded DNA, SS-A, and SS-B were negative. Anti-streptococcal antibody titers were negative. Levels of C3 and C4 were normal.
There is a lobular panniculitis with a dense infiltrate of lymphocytes, plasma cells, and macrophages. Focal hyalinization of the adipocytes is present.
Lupus panniculitis, or lupus profundus, is a variant of lupus erythematosus that primarily affects subcutaneous fat. In nearly all cases there are deep, erythematous plaques and nodules, and some ulcers, which usually involve the proximal extremities, trunk, breasts, buttocks, and face. Lesions may be tender and painful and frequently heal with atrophy and scars. In 70 percent of patients with lupus panniculitis there will be either preceding, subsequent, or concomitant lesions of discoid lupus erythematosus. Further, lupus panniculitis occurs in two to five percent of patients with systemic lupus erythematosus . Conversely, between ten and 50 percent of patients with lupus panniculitis will have or eventually develop systemic lupus erythematosus. Most patients are adults between 20 and 60 years old, with a female to male ratio of approximately two to one. Lupus panniculitis is a chronic condition that often involves persistent lesions that subsequently heal with disfigurement.
Diagnosis is confirmed primarily by both clinical and histologic findings. Histologic features include epidermal atrophy, hydrophic degeneration of the basal-cell layer of the epidermis, and perivascular and periappendageal lymphocytic inflammation that extends into the subcutaneous fat and that may be accompanied by hyalinized fat necrosis. Mucinous changes and foci of calcification can be seen.
Although often normal, serological analysis may show a positive antinuclear antibody titer. Less frequently anti-double stranded DNA antibodies will be present. Syphilis serology may be falsely positive. Other possible laboratory findings are lymphopenia, anemia, reduction of C4 levels, and rheumatoid factor.
Lupus panniculitis often responds to treatment with antimalarials, such as hydroxychloroquine (200 mg once or twice a day). Some cases respond to a combination of antimalarials (for example, hydroxychloroquine 200 mg and quinacrine 100 mg daily) when monotherapy is ineffective. Systemic glucocorticoids should be reserved for widespread and resistant lesions. Intralesional glucocorticoids are usually ineffective and may exacerbate the atrophic healing process. Success with dapsone, azathioprine, and thalidomide has been described in isolated case reports. Surgical debridement or resection of individual lesions may be attempted when all other modalities have failed and there is appreciable debilitation.
ReferencesWatanabe T, Tsuchida T. Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset? Br J Dermatol 134:123, 1996
Chung H-S, Hann S-K. Lupus panniculitis treated by a combination therapy of hydroxychloroquine and quinacrine. J Dermatol 24:5;69, 1997
Kundig TM, et al. Lupus profundus/panniculitis. Dermatology 195:99, 1997
Martens PB, et al. Lupus panniculitis: clinical perspectives from a case series. J Rheumatol 26:68, 1999
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