Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman

Key teaching points
- Malignant atrophic papulosis is a rare vasculopathy that may cause isolated cutaneous disease or severe, widespread disease involving the skin, gastrointestinal tract, central nervous system, and cardiovascular system.
- Early skin lesions of malignant atrophic papulosis appear as multiple rose-colored papules that evolve over several weeks to form a scar with an atrophic, porcelain-white center surrounded by an inflamed, telangiectatic border.
- Traditional therapeutic approaches appear to be ineffective, but new drugs, including eculizumab and treprostinil, have shown benefit in a small number of patients.