A 17-year-old Italian boy presented a 3-year history of violaceous, ma
cular lesions of the breast and internal surface of the right arm with
a characteristic linear distribution. A biopsy specimen revealed a ly
mphohistiocytic panniculitis, and periappendageal and perivascular inf
iltrates of lymphocytes occasionally involving the blood vessel walls
in the deep dermis and hypodermis. Focal hydropic degeneration of basa
l cells was also evident. Direct immunofluorescence revealed linear fo
cal deposits of IgM at the dermoepidermal junction. Antinuclear antibo
dies were positive. Histopathology, direct immunofluorescence and immu
nological assays allowed us to diagnose lupus erythematosus profundus.
To our knowledge, only one other case of linear lupus erythematosus p
rofundus has been reported in the literature until now.