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A 42-year-old man presented with a moderate and diffuse enlargement of the left breast. The skin presented as a red and scaly patch, centered in the nipple (Fig. 1). Laboratory parameters, including IgG and IgM, anti-Borrelia burgdorferi antibodies, and luetic determinations by rapid plasma reagent (RPR), were normal.Histopathologic study demonstrated the typical characteristics of lupus profundus: a mixed lobular and septal lymphocytic panniculitis (Fig. 2), some lymphoid follicles in the infiltrate, lymphocytic vasculitis (Fig. 3), and vacuolar alteration all along the dermoepidermal junction with a smudged appearance, and a thickened basement membrane (Fig. 4).Immunohistochemical study showed a mixed population of B (CD20+) and T (CD3+, CD4+, CD8-) lymphocytes, as well as hystiocytes (CD68+). Molecular studies by polymerase chain reaction (PCR) demonstrated polyclonal rearrangement of IgH (CDR2, CDR3), as well as of TCR-gamma. Serum IgG antinuclear antibodies, lupic anticlotting factor, double-strained DNA antibodies, and extractable nuclear antigen antibodies were all negative.

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