#dermpathJC August 2020:
Thursday, August 27th, 9 pm EST
Article Discussed: Diffuse dermal angiomatosis associated with calciphylaxis: A 5‐year retrospective institutional review
Authors: Heather M. O’Connor, Qiong Wu, Steven D. Lauzon, Jessica A. Forcucci
Temporary free access courtesy of Journal of Cutaneous Pathology: https://doi.org/10.1111/cup.13585
Summary prepared by: Stanton Miller, MD (@StanMiller17)
Editor: Silvija P. Gottesman, MD (@SGottesmanMD)
Journal Club Summary:
Diffuse dermal angiomatosis (DDA) is a rare cutaneous vascular disorder, which was first reported by Krell et al. in 1994. It presents with violaceous plaques on the lower legs or affects pendulous breasts and it frequently ulcerates. Contributing factors are smoking, anticardiolipin antibodies and atherosclerosis. Associated conditions are cutis marmorata telangiectatica congenita and calciphylaxis, the latter being an inspiration for the study.
Histologic findings of Diffuse dermal angiomatosis show a vascular proliferation in the superficial and reticular dermis devoid of atypia and significant amount of inflammation. Vascular immunohistochemical markers can be used to highlight the extent of the proliferation.
- The aim of the study was to investigate any association between calciphylaxis and diffuse dermal angiomatosis (DDA). There are some theories that DDA is a reactive vascular proliferation secondary to local ischemia and local production of vascular endothelial growth factor.
- Calciphylaxis is associated with end-stage renal disease (elevated levels of calcium and phosphate). Patients with non-uremic calciphylaxis usually have either obesity, liver disease, hypercoagulability, use of warfarin or systemic steroids or autoimmune diseases. Histologically, deposition of calcium within small vessel walls of the subcutis is seen.
- The authors noticed that some calciphylaxis biopsies have evidence of DDA in the overlying dermis and that it can be used as a helpful clue to dissect deeper in the fat and look for any evidence of small vessel obstruction by calcium.
- The study showed statistically significant relationship between DDA and African-American race and Chronic Heart Failure. This is newly reported information in the literature. Patients with calciphylaxis and CHF had 33.2 times the odds of having associated DDA in their biopsies compared to those without CHF, and African-Americans with calciphylaxis had 22.2 times the odds of having associated DDA in their biopsies as compared to Caucasians.
- End-stage renal failure, diabetes mellitus, immunosuppressive or hypercoagulable states, arrhythmias, Body Mass Index, hypertension, coronary artery disease, age, duration of calciphylaxis symptoms, and gender were NOT found to have statistically significant associations.
- There is a histologic distinction between DDA and Reactive angioendotheliomatosis and whenever possible the correct entity should be used in signout.
- Communication between the pathologist and the clinician remains of paramount importance.
Take home point:
- While Diffuse dermal angiomatosis has been seen in some cases of calciphylaxis, the most common scenario among the dermatopathologists have been of a benign vascular proliferation involving pendulous breasts. Always consider clinical presentation and location when making the diagnosis.
Until next month #dermpathJC, stay safe!