Cutaneous Hypertrophic Lupus Erythematosus in a Patient With Systemic Involvement
Authors: Carla Cardinali , Barbara Giomi , Marzia Caproni , Paolo Fabbri 
Published: June 2007

A 30‐year‐old white woman was diagnosed with systemic lupus erythematosus (LE) 5 years before presenting to our department. When the disease was first diagnosed she had sharply limited papules on her face (lips and nose), scalp, and back and oral ulcerations. Leukopenia (2.9×103/mm3), anemia (3.5×104/mm3), elevated antinuclear antibodies (homogeneous pattern, 1:320), positive antibodies to native DNA (1:10), and low levels of the fourth component of complement (C4) (8 mg/dL) were also present. The patient was treated with 60 mg/d deflazacort and 400 mg/d hydroxychloroquine and was free of symptoms for 4 years. Twelve months before admission in our department, the patient noted onset of fairly sharply limited, painful erythematous patches on the oral mucosa and, in particular, on the palate and tongue before menses. Upon examination, we noted erythematous, raised, indurated, hyperkeratotic lesions localized on the dorsa of her hands, in particular in the peri‐ungual areas and over the proximal joints (Figure 1). The most characteristic lesion was a large, dull red, indurated plaque with rolled borders on the extensor surface of the first finger of the right hand. This lesion was hyperkeratotic in the center with very adherent scales (Figure 1). Erythematous‐infiltrative lesions with central erosions were present on her lips and sharply marginated, painful erosions were evident on the palate and tongue (Figure 2). She also had a blepharitis of the lower eyelids with hyperplastic features in the lower fornix. Laboratory analysis revealed leukopenia (2.8×103/mm3), hypergammaglobulinemia (25.7%), and elevated levels of immunoglobulin G (1820 mg/dL), low C4 levels (9.3 mg/dL), circulating immune complexes binding the third component of complement (C3) (16.4 μg eq/mL), antinuclear antibodies (homogeneous pattern, 1:80), positive anti‐extractable nuclear antigens antibodies (Scl‐70), antiphospholipid antibodies (immunoglobulin M, 27 MPL), and anti‐β2‐GP‐1 (15 U/mL). Human leucocyte antigens analysis revealed an A2; B44, 35; CW4, W5; DR6, 11, w52; DQ 1,3 extended phenotype. The results of the capillaroscopy were indicative for Raynaud's phenomenon with nailfold capillary abnormalities consisting in a modified intercapillary distance and capillary width. A biopsy taken from a lesion on the second finger of the right hand showed focal epidermal atrophy, diffuse damage of the basal cells with evident liquefaction degeneration, colloid bodies in the upper dermis, and an inflammatory round cell infiltrate of lymphocytes and histiocytes in the superficial dermis and around dermal appendages and vessels. A biopsy from the first finger of the right hand showed marked acanthosis and hyperkeratosis in addition to the other characteristic histologic features of discoid LE but no epidermal atrophy (Figure 3). Biopsy from the oral mucosa showed hydropic degeneration of the basal layers and a mononuclear cell infiltrate near the dermo‐epidermal junction. Direct immunofluorescence performed on sun‐protected non‐lesional skin showed microgranular deposits of immunoglobulin M at the dermo‐epidermal junction and of C3 in the perivascular sites. On the basis of these clinical, histologic, and immunopathologic data the previous diagnosis of systemic LE was confirmed and the cutaneous manifestations were classified as hypertrophic LE. The patient was treated with 400 mg/d hydroxychloroquine and 8 mg/d methylprednisone and showed marked improvement of the skin lesions. Erythematous‐hyperkeratotic lesions on the periungual areas and on the proximal joints of the fingers image Sharply marginated painful erosions on the palate image Hematoxylin‐eosin stain of lesional skin of the extensor surface of the first finger showing marked acan‐thosis and hyperkeratosis and pronounced cell infiltrate in the upper dermis image