Acquired Cutaneous Lymphangiectasia With Mesothelial Cells Reflux in a Patient With Cirrhotic Ascites

Abstract

A previously undescribed case of acquired cutaneous lymphangiectasias on the abdomen in a patient with cirrhotic ascites where peritoneal mesothelial cells refluxed in the skin is discussed. A 56-year-old man previously submitted to liver transplantation presented with vesiculobullous lesions on the developed as his cirrhotic ascites progressed. Histology showed dilated lymphatic channels in the upper dermis lined by a single, discontinuous layer of flattened, monomorphous endothelial cells with endoluminal papillary projections. In the deep reticular dermis, we observed irregular thin- often jagged-walled vascular channels lined by a single layer of bland endothelial cells, dissecting the collagen bundles. Vessels in the lumen were medium to large bizarre-shaped polygonal cells with abundant eosinophilic cytoplasm and hyperchromatic and irregular nuclei, arranged in small clusters or as solitary units, focally in close contact with the endothelial lining or free floating within vessel cavities. Immunohistochemistry indicated atypical intraluminal cells to be positive for calretinin, a specific marker for mesothelial cells. Pathophysiologic mechanisms and problems of differential diagnosis of this unique clinicopathologic entity are discussed.

Benign mesothelial nodules reflux within acquired cutaneous lymphangiectasia associated with huge ovarian clear cell carcinoma

“…Our case showed no local invasion, metastasis or peritoneal dissemination, and the cells comprising the intralymphatic glomeruloid nodules displayed much lower cellular/structural atypia in comparison to ovarian carcinoma; thus, it was easy to clinicopathologically and immunohistochemically distinguish from the possibility of extensively spreading carcinoma cells. Besides, we agree with previous studies, which indicate the importance of applying a wide panel of immunohistochemical antibodies in order to make a conclusive diagnosis of BMNR in the cutaneous lymphatics . In addition to testing the submitted specimen for mesothelial markers, further analyses, including FISH and other immunochemical analyses would be powerful supplementary tools for excluding the possibility of malignancy, including malignant mesothelioma .…”

“…We would like to know the pathogenesis and pathophysiological mechanism(s) underlying the development of BMNR in the present case, which might represent a possible new entity within acquired cutaneous lymphangiectasia. In line with the three reported cases of cutaneous lymphatic BMNR, the significantly accelerated intraabdominal pressure might have had various causes, including a huge ovarian tumor and/or severe ascites, leading to the obstruction of local lymphatic drainage and resulting in the passive mechanical transport of the peritoneal mesothelial/inflammatory nodules. This mechanism is very similar to the mesothelial cell inclusions within lymph nodes, as previously reported .…”

“…Although mesothelial cell inclusions within the mediastinal or abdominal lymph nodes are a well known phenomenon, BMNR in the cutaneous lymphatics is rarely reported and potentially represents a new morphological and clinicopathological entity. To the best of our knowledge, BMNR was first described as the lymphatic dissemination and embolization of mesothelial cells by Rossi Suárez‐Viela and Izquierdo‐Garcia in the late 1990s, and to date, only four cases of BMNR (including our own) have been reported in the English literature . BMNR in the cutaneous lymph vessels is an extremely uncommon and unestablished entity; however, we should be aware that pathologists might misinterpret BMNR as the lymphatic dissemination of malignant cells.…”

“…There have been some interesting reports describing a unique case of benign mesothelial nodule reflux (BMNR) in the cutaneous lymph vessels (the benign mesothelial nodules are composed of polygonal mesothelial cells admixed with a variable number of inflammatory cells, especially the histiocytes) including: (i) ‘Embolization of mesothelial cells’ in lymphatics of a 60‐year‐old male on the abdominal skin with hernia, accompanied by alcoholic cirrhosis and severe ascites; (ii) ‘Mesothelial cells reflux’ within acquired cutaneous lymphangiectasia of a 56‐year‐old male on the abdominal skin, accompanied by hepatitis C virus‐induced cirrhosis and severe ascites; and (iii) ‘Benign mesothelial nodules’ in lymphatics of a 55‐year‐old female on the umbilical skin with huge hernia, associated with massive ovarian fibroma . Although mesothelial cell inclusions within the mediastinal or abdominal lymph nodes are a well known phenomenon, BMNR in the cutaneous lymphatics is rarely reported and potentially represents a new morphological and clinicopathological entity.…”

“…To the best of our knowledge, BMNR was first described as the lymphatic dissemination and embolization of mesothelial cells by Rossi Su arez-Viela and Izquierdo-Garcia in the late 1990s, 1 and to date, only four cases of BMNR (including our own) have been reported in the English literature. [1][2][3] BMNR in the cutaneous lymph vessels is an extremely uncommon and unestablished entity; however, we should be aware that pathologists might misinterpret BMNR as the lymphatic dissemination of malignant cells. We showed the first case of BMNR within acquired cutaneous lymphangiectasia, which was associated with huge ovarian clear cell carcinoma and ascites in an obese patient.…”