Marfan syndrome resulting from a rare pathogenic FBN1 variant, ascertained through a proband with IgG4-related arteriopathy.

A 57-year-old man with a family history of aortic aneurysm was found, during assessment of unexplained fever, to have an infrarenal aortic aneurysm requiring immediate repair. Dilatation of popliteal and iliac arteries was also present. Progressive aortic root dilatation with aortic regurgitation was documented from 70 years leading to valve-sparing aortic root replacement at 77 years, at which time genetic studies identified a likely pathogenic FBN1 missense variant c.

Isolated Mesenteric Calciphylaxis With Ischemic Colitis in a Hemodialysis Patient Without Active Cutaneous Calciphylaxis: A Case Report of Calcific Uremic Arteriolopathy.

Calciphylaxis, also known as calcific uremic arteriolopathy, is a devastating systemic disease most commonly associated with chronic kidney failure. Its hallmark histopathologic features of small-vessel calcification, intimal hyperplasia, and microthrombi lead to microvascular occlusion and tissue necrosis. Clinically, it typically presents with painful cutaneous lesions that may be distal or proximal, with proximal lesions associated with higher mortality.

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club: Peritoneal Lipofuscinosis and Deciduosis in Pregnancy.

Peritoneal lipofuscinosis is a very rarely recognized condition occurring during pregnancy characterized by brown pigmentation of the omentum and peritoneum, a decidual reaction and benign mesothelial cells. The iron negative pigment, which is likely to be confused with hemosiderin in the hematoxylin and eosin stain, is lipofuscin. The seminar case, apparently the third published, arose in a 37-year-old woman who presented in October 2015 at 24 weeks pregnancy with abdominal pain.

Selected Case From the Arkadi M. Rywlin International Pathology Slide: Massive Localized Lymphedema in Morbid Obesity Complicated by a Nonspecific Subcutaneous Abscess.

A 59-year-old morbidly obese female developed an ulcerated, slowly growing, 25 cm, subcutaneous, pendulous mass in the right groin which became infected and was excised in January 2014. The excised skin and subcutaneous fat weighed 1901 g. The skin exhibited a cobblestone appearance, the dermis was thickened and edematous, and the subcutaneous fat was traversed by fibrous septae.

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club: Polypoid Endometriosis in the Pouch of Douglas in a Perimenopausal Woman.

A 50-year-old female had a hysterectomy and bilateral salpingo-oophorectomy for adenomyosis, uterine leiomyomas, ovarian and cervical endometriosis. Nine months later, organ imaging revealed a 43×74×52 mm, apparently malignant extraperitoneal mass lying between the vagina and rectum. The patient had been receiving a progestogen.

Selected case from the Arkadi M. Rywlin International Pathology Slide Club: aggressive angiomyxoma, left labial region in a postmenopausal woman.

Club members unanimously agreed with the diagnosis of an unencapsulated 8×2.5×3.6 cm aggressive angiomyxoma, which was invading the voluntary muscles of the pelvic floor beneath the left labium of a female aged 65.