A case of a recalcitrant painful skin ulceration

A 57-year-old African American female with a past medical history of avascular necrosis of the bilateral hips and shoulder, longstanding smoking, miscarriage, diabetes, chronic kidney disease, and hepatitis C presented for a several months’ history of an extremely painful ulceration on her right lateral malleolus (Figure 1). The patient described the lesion as worse in the summertime. She was previously treated with topical and systemic steroids and antibiotics with no improvement. Physical exam revealed a tender stellate deep ulcer overlying the right lateral malleolus with peripheral hyperpigmentation and pinpoint white scarring. Serology was notable for elevated levels of ANA, C4, lipoprotein A, and rheumatoid factor levels, with a heterozygous mutation of the MTHFR A1298C. Histopathology showed hyalinization and focal fibrin deposition within the small vessel wall, vascular congestion and extravasated red blood cells within the deep dermis, and focal thrombosis at the interface of the deep dermis and subcutaneous tissue (Figure 2). The patient was started on high potency topical steroids, aspirin 325 mg daily, indomethacin 25 mg twice daily, and doxycycline 100 mg twice daily. Smoking cessation was strongly advised and, after a doppler ultrasound and a normal ankle-brachial index of 0.99 ruled out any underlying arterial compromise, compression stockings were also recommended. A follow-up visit at 2 months showed remarkable ulcer improvement and resolution in her pain (Figure 3). What is your diagnosis: A) Polyarteritis nodosa, B) Cryoglobulinemia, C) Livedoid vasculopathy, or D) Ischemic ulcer Figure 1 Ulceration of the right lateral malleolus