#dermpathJC November 2017:
Thursday, November 30th, 9pm EST
Article discussed: Cutaneous Collagenous Vasculopathy: Report of Two Cases Presenting as Disseminated Telangiectasis and Review of the Literature.
Authors: Laure Bondier, Mathilde Tardieu, Perrine Leveque, Isabelle Challende, Nicole Pinel, Marie T. Leccia.
American Journal of Dermatopathology, 2017; 39: 682–688.
Free access for 4 weeks at: http://journals.lww.com/amjdermatopathology/Abstract/2017/09000/Cutaneous_Collagenous_Vasculopathy___Report_of_Two.6.aspx
Summary author: Dr. Silvija P. Gottesman (@SGottesmanMD).
Journal Club Summary:
Cutaneous collagenous vasculopathy (CCV) – an idiopathic microangiopathy, acquired telangiectasias localized mainly on the extremities. CCV can also involve the trunk, but usually spares the face. It is asymptomatic & slowly progressive.
To date, 34 cases reported since its initial description in 2000. Cases more frequently seen in women, median age 63.5, median time to patient reporting skin changes to a clinician is more than 7 years, with range from 0.4yrs to >20 yrs.
No mucosal or nail involvement. Systemic involvement was not noted in any patient. Family history is negative for telangiectasias or bleeding disorders, and no autosomal dominant pattern of inheritance was found.
Cutaneous collagenous vasculopathy clinical ddx. Clinically telangiectasias 2/2 liver disease, Osler-Weber-Rendu syndrome, hereditary benign telangiectasia and CREST syndrome can look similar.
Proposed etiology of endothelial injury due to comorbidities such as HTN and dyslipidemia seems reasonable.
CCV has distinct histology findings: dilated vessels in the superficial dermis with vessel walls thickened with hyaline material that stains for type IV collagen. The hyaline material is accentuated with a PAS stain.
This is similar to thickened hyaline vessels in PCT, which are also highlighted with PAS stain. But in CCV there will be no dermal fibrosis, no deep thickened vessels. Can be used to differentiate from vascular thickening in stasis dermatitis.
Electron Microscopy of cutaneous collagenous vasculopathy: thickened vascular walls contain collagen fibrils scattered in a fine granular material. Luse bodies (long spacing collagen) noted.
Luse body – presence of abnormal long-spaced collagen in the outer vessel walls was reported in only 9 cases of CCV. Luse bodies have low specificity and can be found in various other conditions (scleroderma, blue nevus, melanoma.). Their presence is not necessary for diagnosis.
Interestingly, in this review 4 (11.7%) cases were treated with calcium channel blockers. The amount of effectiveness of this treatment is yet to be reported.
Thanks to all who participated! Next dermpath journal club scheduled for 12/28/2017 at 9PM EST! See you soon!
