Malignant atrophic papulosis with motor aphasia and intestinal perforation: A case report and review of published works

Abstract

Malignant atrophic papulosis (MAP) is a rare type of obliterating vasculopathy that can present as pure cutaneous lesions or a systemic entity affecting multiple organs. Systemic disease, such as gastrointestinal or central nervous system involvement, may predispose the patients to poorer or even fatal outcomes. We present a 30-year-old female patient with systemic manifestation of MAP 10 days after delivery of a full-term pregnancy who subsequently developed motor aphasia and intestinal perforation. The patient was administrated empirical treatment with an antiplatelet, anticoagulant, methylprednisolone sodium succinate and alprostadil. Antibiotics were administrated due to intestinal perforation and secondary sepsis. Despite all treatment, the patient died a week later. We summarized all the previous reports of MAP based on thorough review of previous published work. Overall, this is the first patient with MAP combined with motor aphasia and intestinal perforation and may provide insights for future studies on the treatment of this disease.

Keywords: case report; intestinal perforation; malignant atrophic papulosis; motor aphasia; review of published work.

Figure 1

(a,b) Characteristic lesions of Degos disease with central porcelain‐white atrophy surrounding a rim of erythema on the trunk. (c,d) Histopathological sections showing absence of crests, atrophic epidermis, epidermal overlying orthokeratosis and a lymphocytic hyalinization of collagen infiltrate in the papillary dermis (hematoxylin–eosin, original magnification ×100).

Figure 2

(a,b) Left side of the parietal lobes can be seen large patchy and patchy uneven long T₁ and T₂ signals, suggesting a large area of cerebral infarction with bleeding. (c) Magnetic resonance angiography shows that the main branches of cerebral arteries are normal. (d) Magnetic resonance perfusion shows a significant reduction in infarct perfusion.