Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report

Abstract

Chronic cutaneous lupus erythematosus in a linear configuration is rare, particularly in children, demonstrating similar incidence in both genders, no photo-sensitivity and lower probability of progression to systemic disease. We describe the case of a 9-year-old girl who presented erythematous papules with central atrophy on the upper and lower right limbs, asymptomatic and following the lines of Blaschko, since age four. Histological examination showed atrophy of the epidermis with aggression from epidermal-dermal interface and periadnexal and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity of presentation and location of the disease, this case is reported here.

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

“…These findings are granular depositions of IgG, IgM, IgA, and/or C3 along the dermoepidermal junction and periadnexal structure. Nonetheless, a negative DIF result does not exclude the diagnosis [10].…”

“…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.…”

“…LE-associated serology such as the antinuclear antibodies are mostly negative. Photosensitivity and progression to SLE are rarely observed [7,9,10].…”

“…The differential diagnosis of LCLE includes other acquired inflammatory skin diseases distributed along the lines of Blaschko, such as linear morphea, linear lichen planus, lichen striatus, linear psoriasis, linear lichen sclerosus and linear granuloma annulare [10].…”

Progressive linear blaschkoid lupus erythematosus

“…LBLE has recently been proposed as a prominent subset of linear cutaneous lupus erythematosus in children . Its distinct features include early onset (children and young adults), equal incidence in both sexes, skin lesions showing a blaschkoid distribution predominantly in the head and neck, low photosensitivity, and absence of antibodies . Histologically, it appears identical to cutaneous LE, especially discoid and panicullitic LE.…”