Abstract

A 33-year-old female with a history of endometriosis presents after laparoscopic abdominal surgery with jagged ulcerations on her abdomen. Of note, her surgery was complicated by post-operative abdominal pain, requiring constant ice pack topical therapy. Laboratory investigation including ANCA, ANA, Anti-dsDNA, infectious workup and cryoglobulins was negative. Punch biopsy demonstrates vascular occlusion and vessel wall necrosis with interstitial neutrophils and superficial eccrine coil necrosis. Additionally, there are areas of full thickness ischemic epidermal necrosis, superficial and deep perivascular and periadnexal inflammation, and fibrin microthrombi formation within small caliber vessels. The histologic and clinical findings are those of cold-induced vasculopathy. Vasculopathy leading to full thickness epidermal necrosis, in addition to perivascular and periadnexal inflammation – features reminiscent of connective tissue disease – has never been reported in cold-induced dermatitis [1]. Distinction from lupus can be made with clinical presentation and laboratory investigation, although a pronounced vacuolar change may favor this diagnosis [1]. Reported cases of cold-induced dermatitis include perniosis, which is characterized by pronounced dermal edema and a lichenoid inflammatory infiltrate with peri-eccrine involvement [1,2]. Cold-induced panniculitis and dermo-hypodermitis have also been reported [3]. The lack of deep-seated inflammation present at the dermal-subcutaneous interface precludes the diagnosis of a panniculitis in our patient. Another important differential diagnosis to exclude for cold-induced skin ulceration is cryoglobulinemic vasculitis, characterized histologically by small and medium vessel vasculitis with immune complex deposition [4]. Finally, cold-induced urticaria and urticarial vasculitis have also been reported, but these patients do not present with skin ulceration [5,6].