Generalized Lentiginosis, Short Stature, and Multiple Cutaneous Nodules—Quiz Case

A 29-year-old man with chronic lymphedema in the left lower extremity for 8 years presented with an enlarging and ulcerated nodular plaque on his left leg. The lesion had started out as a bruise, then progressively enlarged and ulcerated over 4 months. He went to many physicians during this time and was tested and/or treated for possible infection, deep vein thrombosis, vasculitis, and a torn muscle. The patient’s family history was remarkable in that his mother and sister both have chronic bilateral lymphedema. A physical examination of his left leg revealed a 20-cm blue and black hemorrhagic nodular plaque with large central eschar and a few smaller satellite bluish nodules (Figure 1). An open incisional biopsy specimen of the lesion was obtained (Figure 2 and Figure 3). What is your diagnosis?