Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman - 14/11/16

Doi : 10.1016/j.jaad.2016.09.015

Brittany Oliver, BS ^a, Manfred Boehm, MD ^b, Douglas R. Rosing, MD ^c, Lee S. Shapiro, MD ^d, Daniel T. Dempsey, MD ^e, Peter A. Merkel, MD ^f, Chyi-Chia Richard Lee, MD ^g, Edward W. Cowen, MD, MHSc ^h,^⁎
^a National Institutes of Health, Bethesda, Maryland
^b Center for Molecular Medicine, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland
^c National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland
^d Center for Rheumatology, Saratoga Springs, New York
^e Department of Surgery, University of Pennsylvania, Philadelphia, Pennsylvania
^f Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania
^g Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland
^h Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland

^∗Correspondence to: Edward W. Cowen, MD, MHSc, Dermatology Branch, Center for Cancer Research, National Cancer Institute, Bldg 10/Room 12N238, 10 Center Dr, MSC 1908, Bethesda, MD 20892-1908.Dermatology BranchCenter for Cancer ResearchNational Cancer InstituteBldg 10/Room 12N238, 10 Center Dr, MSC 1908BethesdaMD20892-1908

Abstract

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.

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Key words : complement, Degos disease, eculizumab, interferon, malignant atrophic papulosis, treprostinil, vasculopathy

Plan

Significant diagnostic studies

Diagnosis

Follow-up

Discussion

	Supported by the Intramural Program of the National Institutes of Health (NIH), the Center for Cancer Research, the National Cancer Institute, and the Medical Research Scholars Program, a public-private partnership supported jointly by the NIH and generous contributions to the Foundation for the NIH from the Doris Duke Charitable Foundation, the American Association for Dental Research, the Howard Hughes Medical Institute, and the Colgate-Palmolive Company, as well as other private donors.
	Disclosure: Dr Merkel reports receiving research support from ChemoCentryx Inc. Ms Oliver and Drs Boehm, Rosing, Shapiro, Dempsey, Lee, and Cowen have no conflicts of interest to declare.
	Reprints not available from the authors.