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February 2001

Plaques on the Face of a 26-Year-Old Woman With Usher Syndrome

Author Affiliations


Arch Dermatol. 2001;137(2):219-224. doi:

A 26-year-old woman presented with a 3-year history of a progressive growth on her upper lip and chin. She had been treated with topical metronidazole gel for suspected rosacea for several months, without improvement. Her medical history indicated that she had Usher syndrome with congenital deafness and retinitis pigmentosa. She also had systemic lupus erythematosus with arthralgias and diffuse proliferative nephritis. She was taking cyclophosphamide and oral prednisone.

Physical examination revealed a 2 × 8-cm erythematous, firm, indurated plaque with a cobblestone surface symmetrically involving the entire cutaneous portion of the upper lip. A similar appearing 1 × 6-cm plaque involved the cutaneous portion of the lower lip and chin (Figure 1). Biopsy specimens were obtained from the right and left sides of the philtrum (Figure 2 and Figure 3).

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