A fatal case of malignant atrophic papulosis in a pediatric patient

Lauren S. Schaefer, Theresa Wampler Muskardin, Jan Mendelt Tillema, Carilyn Wieland, Megha M. Tollefson

Research output: Contribution to journalArticlepeer-review

Abstract

A 17-year-old Caucasian boy presented with progressive left-sided weakness, transient slurred speech, and skin lesions characterized by 3-5 mm, pink, asymptomatic papules with white atrophic centers on his central abdomen, back, and lower extremities. Skin biopsy confirmed the diagnosis of malignant atrophic papulosis, a rare vasculopathy that leads to the occlusion of small- and medium-sized arteries. He was treated with cyclophosphamide, eculizumab, treprostinil, pentoxifylline, heparin, and acetylsalicylic acid. Despite the aggressive immunosuppression, humanized monoclonal antibodies, and antiplatelet therapy, he died two months after presentation. We report this case to highlight diagnostic features, as well as to highlight the importance of early diagnosis and treatment.

Keywords

  • degos disease
  • eculizumab
  • malignant atrophic papulosis
  • MAP
  • treprostinil

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

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