Malignant atrophic papulosis: Two case reports

Abstract

Background: Malignant atrophic papulosis is a rare and potentially lethal thrombo-occlusive microvasculopathy characterized by cutaneous papules and gastrointestinal perforation. The precise pathogenesis of this disease remains obscure.

Case summary: We describe the case of a 67-year-old male patient who initially presented with cutaneous aubergine papules and dull pain in the epigastrium. One week after symptom onset, he was admitted to the hospital for worsening abdominal pain. Exploratory laparotomy showed patchy necrosis and subserosal white plaque lesions on the small intestinal wall, along with multiple perforations. Histological examination of the small intestine showed extensive hyperemia, edema, necrosis with varying degrees of inflammatory reactions in the small bowel wall, small vasculitis with fibrinoid necrosis and intraluminal thrombosis in the mesothelium. Based on the mentioned evidence, a diagnosis of malignant atrophic papulosis was made. We also present the case of a 46-year-old man with known cutaneous manifestations, abdominal pain, nausea and vomiting. His physical examination showed positive rebound tenderness. A computed tomography scan revealed free intraperitoneal air. He required surgical intervention on admission and then developed an esophageal perforation. He ultimately died of a massive hemorrhage.

Conclusion: In previously published cases of this disease, the cutaneous lesions initially appeared as small erythematous papules. Subsequently, the papules became porcelain-white atrophic depression lesions with a pink, telangiectatic peripheral rim. In one of the patients, the cutaneous lesions appeared as aubergine papules. The other patient developed multiple perforations in the gastrointestinal tract. Due to malignant atrophic papulosis affecting multiple organs, many authors speculated that it is not a specific entity. This case series serves as additional evidence for our hypothesis.

Keywords: Case report; Gastrointestinal perforation; Malignant atrophic papulosis; Papulosis; Thrombo-occlusive microvasculopathy.

Figure 1

Computed tomography scan of the abdomen. A and B: Preoperative computed tomography examination findings showed free intraperitoneal air (A) and intraperitoneal free fluid (B) in case 1; C: Computed tomography scan of the abdomen revealed free intraperitoneal air under the diaphragm and in the right abdominal area in case 2.

Figure 2

Intraoperative findings. Laparotomy showed patchy necrosis and subserosal white plaque lesions on the small bowel wall, along with multiple perforations. A: Patchy necrosis and subserosal white plaque lesions on the small bowel wall in case 1; B: Multiple perforations and subserosal white plaque lesions in case 1; C: Operative findings during initial exploratory laparotomy showed multiple perforations at the distal ileum in case 2.

Figure 3

Cutaneous lesions with aubergine papules on the lower extremities.

Figure 4

Histological findings of the gastrointestinal tract in case 1. A and B: Representative histological findings of the small bowel, which revealed extensive hyperemia, edema and necrosis (hematoxylin and eosin staining), as well as small vasculitis with fibrinoid necrosis and intraluminal thrombosis; C and D: Representative histological findings which showed inflammatory cell infiltration and congestion of the intestinal vasculature.

Figure 5

Histological findings of the skin in case 1. A and B: Representative sections from biopsies of the cutaneous lesions, which revealed the swollen endothelium of small vessels in the dermis, perivascular eosinophils, neutrophils, lymphocytes and broken leukocyte infiltration (hematoxylin and eosin staining); C and D: Representative sections from biopsies, which showed the swollen endothelium of small vessels in the dermis. Perivascular eosinophils, neutrophils, lymphocytes and broken leukocyte infiltration.

Figure 6

Postoperative digestive tract radiography and gastroscopy in case 2. A: Esophageal perforation was shown in postoperative computed tomography images; B-D: Representative images of gross anatomy showing ulcerations at the fundus and cardia of the stomach and esophagus.