Linear Sclerodermoid Lupus Erythematosus Profundus in a Child

Abstract

Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Microscopic findings from 2 biopsies included lymphocytes at the dermoepidermal junction with mild interface dermatitis, a dense lymphocytic infiltrate that was concentrated around adnexae and subcutaneous fat in concert with thickened collagen bundles and mild widening of fibrous septae surrounding fat lobules. Although the clinical differential diagnosis included panniculitis or a sporotrichoid infection, 1 biopsy showed a dense lymphocytic infiltrate histologically bordered on that of cutaneous lymphoid hyperplasia or a late stage of Lyme disease, and a second also demonstrated more prominent sclerodermoid collagen bundles rendering the diagnosis of linear sclerodermoid LE profundus.

Adult-onset linear discoid lupus erythematosus on the forehead mimicking en coup de sabre: a case report

“…According to the literature review, a total of 102 cases diagnosed with LCLE have been reported. The most common pathological diagnosis of CLE in the linear distribution was discoid lupus erythematosus (DLE) (39.2%), followed by lupus erythematosus panniculitis (LEP) (21.6%), lupus erythematosus tumid (LET) (2.9%), subacute lupus erythematosus (SCLE) (2%) and bullous lesions of SLE (1%) [4][5][6][7][8][9][10][11][12][13]. LDLE mainly occurs in children and young adults.…”

Lupus Erythematosus Profundus: A Case Series and Review of Literature

“…A linear configuration of LE panniculitis has been reported with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. Elbendary et al reported a case of linear sclerodermoid LE profundus [18].…”

Panniculitides of particular interest to the rheumatologist

“…A positive lupus band test may be used to support a diagnosis of LEP. The predominant immune-deposit in direct immunofluorescence is IgM, and C3 and IgG are seen in half of the cases [43,50].…”

“…It presents clinically as deep subcutaneous nodules and erythematous to violaceous infiltrated plaques, which occasionally ulcerate and often resolve leaving depressed areas [42,48]. A linear configuration of LEP has been sometimes reported, especially in children and adolescents [50]. Serologic abnormalities include antinuclear antibodies, usually at low titers, anti-double-stranded-DNA and anti-ENA-antibodies [51].…”