This 48-year-old woman experienced slowly progressive pruritic plaques beginning on the neck and upper chest in 2001. Since then, the plaques have extended onto the upper back and shoulders. She also had a history of progressive nonspecific interstitial pneumonitis and severe restrictive lung disease requiring continuous oxygen. Review of system was also significant for esophageal dysmotility, Raynaud pehnomenon, and supraventricular tachycardia. She was treated successfully with cyclophosphamide and prednisone. In December 2002 she received a left lung transplant, and she was doing well off supplemental oxygen. Her only medications were for post-transplantation immunosuppression.
There is a normal epidermis with subjacent pigment incontinence in the papillary dermis. The reticular dermis is replacec by a thick band-like area of collagen. There is a decrease in adnexal structures with those remaining lacking surrounding adipose tissue. One specimen demonstrated a mild superficial perivascular lymphocytic infiltrate.