A 42 year old woman with a one-year history of Raynaud's phenomenon. Previous extensive investigations were not significant: the patient had anti-Scl-70 antibodies and an initial scleroderma pattern on capillaroscopy but no evidence of visceral or cutaneous involvement.
One month before coming to our Department (while being treated with nifedipine 10mg bid alone) she noticed the appearance of a slightly indurated plaque on both sides of the neck. Physical examination revealed two 4-5 cm in diameter superficial yellow-brown plaques extending to the lateral sides of the neck and the supraclavear area. The skin appeared thickened but still somewhat elastic and the surface was smooth but irregular due to a network of closely packed broad based papules (beading). There was no sclerodactyly, pitted scars, telangectases or other cutaneous evidence of systemic sclerosis.
She was treated with griseofulvin (500 mg/d) for 9 months with initial improvement. However, during the following months, she started complaining of hand stiffness and developed slight sclerodactyly. Penicillamine treatment was therefore started.