• Users Online: 1571
  • Print this page
  • Email this page


 
  Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 7  |  Issue : 1  |  Page : 40-42  

Cutaneous collagenous vasculopathy: A rare case report


1 Department of Dermatology, Seth GS Medical College, KEM Hospital, Mumbai, Maharashtra, India
2 Consultant Dermatologist New Snehadeep Skin and Eye Hospital, Mumbai, Maharashtra, India

Date of Web Publication18-Jan-2016

Correspondence Address:
Dr. Kinjal Deepak Rambhia
Department of Dermatology, Seth GS Medical College, KEM Hospital, Parel, Mumbai, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.174327

Rights and Permissions
   Abstract 

Cutaneous collagenous vasculopathy (CCV) is a distinct, rare, and underdiagnosed condition. We report a case of CCV in a 50-year-old woman presenting as asymptomatic, erythematous to hyperpigmented nonblanchable macules over both the lower extremities. The clinical differential diagnosis of the lesions was pigmented purpuric dermatoses (Schamberg's purpura) and cutaneous small vessel vasculitis. Histology of the lesions revealed dilated superficial dermal vessels with abundant pink hyaline material in the vessel wall, which stained with periodic acid Schiff stain. The patient was diagnosed as CCV. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telangiectasia. Emphasis on the differentiation of CCV from its clinical and histological mimicks is made.

Keywords: Cutaneous collagenous vasculopathy, dilated superficial dermal vessels, hyaline, microangiopathy


How to cite this article:
Rambhia KD, Hadawale SD, Khopkar US. Cutaneous collagenous vasculopathy: A rare case report. Indian Dermatol Online J 2016;7:40-2

How to cite this URL:
Rambhia KD, Hadawale SD, Khopkar US. Cutaneous collagenous vasculopathy: A rare case report. Indian Dermatol Online J [serial online] 2016 [cited 2019 Sep 22];7:40-2. Available from: http://www.idoj.in/text.asp?2016/7/1/40/174327


   Introduction Top


Cutaneous collagenous vasculopathy (CCV) is a distinct and rare cutaneous microangiopathy affecting the superficial dermal blood vessels. These patients present with asymptomatic blanchable pink or red macules, telangiectasia, or petechiae distributed symmetrically on bilateral lower extremities with progressive involvement of the upper extremities and trunk. The comorbidities frequently associated in these patients are diabetes mellitus, hyperlipidemia, hypertriglyceridemia, and hypertension. Microscopically, it reveals thickened and dilated superficial dermal vessels. This condition remains largely underdiagnosed and is commonly mistaken for pigmented purpuric dermatosis or generalized essential telengiectasias.


   Case Report Top


A 50-year-old female presented with asymptomatic rash on the lower extremities since 2 years. Cutaneous examination revealed erythematous to hyperpigmented nonblanchable, nontender macules and petechial lesions over both the lower extremities [Figure 1]. There was no edema or warmth of the affected area. There was a history of diabetes mellitus in the patient for which she was currently not on any medications.
Figure 1: Nonblanchable macules and petechiae on lower extremity

Click here to view


Skin biopsy from the erythematous macule showed vasculopathy involving the small vessels of the superficial plexus. The affected vessels showed abundant pink collagenous deposit in the wall and moderately dense lymphocytic infiltrate around them [Figure 2] and [Figure 3]. The collagenous deposits in the vessel walls were Periodic Acid Schiff (PAS) positive [Figure 4]. Many of the vessels showed dilatation of the walls resembling telangiectasia. There was neither vasculitis nor any perivascular hemosiderin deposition. The case was diagnosed as cutaneous collagenous vasculopathy (CCV).
Figure 2:Dilated superficial vessels with pinkish hyaline deposit in the vessel wall. (Hematoxylin and Eosin ×100)

Click here to view
Figure 3: Dilated superficial vessels with pink hyaline deposit in the vessel wall. (Hematoxylin and Eosin ×400)

Click here to view
Figure 4: PAS positive hyaline deposit in the vessel wall. (Periodic Acid Schiff ×400)

Click here to view



   Discussion Top


CCV is a rare distinct idiopathic microangiopathy of superficial dermal blood vessels. The first case of cutaneous collagenous vasculopathy presenting with asymptomatic telangiectases was described by Salama and Rosenthal in the year 2000.[1] This condition has been described in middle-aged and elderly men. There have been descriptions of this condition in women too.[2] The exact etiology of the condition is not known. Several patients with CCV described in literature had diabetes mellitus. The occurrence of microangiopathy in diabetes mellitus could possibly contribute to the development of CCV.[3] Other factor implicated in the causation for CCV is trauma. It is also speculated that this condition may represent a genetic vascular disorder.[4] Another hypothesis is that CCV may be caused by a genetic defect in collagen synthesis.[5] The comorbidities seen in patients with CCV were diabetes mellitus, hypertension, hyperlipidemia, hypothyroidism, and venous insufficiency.[5]

Clinically, the most common presentation was in the form of asymptomatic bilaterally symmetrical progressive telangiectases on lower extremities with gradual progression to upper extremities and rarely the trunk. These lesions were clinically mistaken for generalized essential telangiectasia. Other clinical morphologies described were discrete dark pink blanchable macules,[4] petechiae,[4] red macules, and ecchymosis [5] in one patient. The patient described in this report had erythematous, petechial lesions, and nonblanching macules.

Histologically, CCV is characterized by dilated and thickened superficial dermal vessels with flat endothelial cells and the vessel wall containing hyaline material. There is paucity of inflammation and the vessel walls show no sign of damage by inflammatory cells. There is no hemorrhage or hemosiderin deposition in the perivascular area. The hyaline material stains with PAS and is diastase resistant.[6] The hyaline material of the thickened walls is highlighted by immunohistochemistry for laminin and type IV collagen but not with elastic stains, which indicates it is a derivative of the basement membrane collagen and is the result of reduplication and splitting of the basement membrane zone surrounding small vessels.[7] Histopathologically, CCV may be misdiagnosed as telangiectasia (dilated capillaries without inflammatory infiltrate), livedoid vasculopathy (deposits of fibrin in the dermal vessels without significant inflammatory infiltrate) or cutaneous small vessel vasculitis (leukocytoclasia, damage to the vascular endothelium with fibrin deposition). Other conditions showing hyaline material in the vessel walls are lipoid proteinosis (hyaline material around the eccrine gland with atrophy of the glands), porphyrias (subepidermal blister), pseudoporphyrias (subepidermal blister), secondary amyloidosis (hyaline material in papillary dermis and subcutaneous tissue with Congo-red positivity), and thrombotic vasculopathy (occlusion of the lumen by the thrombus and hemorrhage.) Electron microscopy reveals “Luse bodies” representing collagen fibers with abnormally long spaces between electron-dense bands.[8]

The exact pathomechanisms of the condition are not yet elucidated. However, it is proposed that vascular damage is followed by repair leading to a defect in collagen formation and abnormal disorganized collagen deposition in the cutaneous blood vessel wall.[8]

Cutaneous collagenous vasculopathy is an under-recognised and underdiagnosed condition. The current report emphasizes the need for awareness of this condition and its clinical and histological mimicks.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: An immunohistochemical and ultrastructural study. J Cutan Pathol 2000;27:40-8.  Back to cited text no. 1
    
2.
Perez A, Wain ME, Robson A, Groves RW, Stefanato CM. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol 2010;63:882-5.  Back to cited text no. 2
    
3.
Burdick LM, Losher S, Somach SC, Billings SD. Cutaneous collagenous vasculopathy: A rare cutaneous microangiopathy. J Cutan Pathol 2012;39:741-6.  Back to cited text no. 3
    
4.
Echeverría B, Sanmartín O, Botella-Estrada R, Vitiello M. Cutaneous collagenous vasculopathy successfully treated with pulsed dye laser. Int J Dermatol 2012;51:1359-62.  Back to cited text no. 4
    
5.
Davis TL, Mandal RV, Bevona C, Tsai KY, Moschella SL, Staszewski R, et al. Collagenous vasculopathy: A report of three cases. J Cutan Pathol 2008;35:967-70.  Back to cited text no. 5
    
6.
Salama SS. Cutaneous collagenous vasculopathy: A new case series with clinicopathologic and ultrastructural correlation, literature review, and insight into the pathogenesis. Am J Dermatopathol 2015;37:368-75.  Back to cited text no. 6
    
7.
Perez A, Wain ME, Robson A, Groves RW, Stefanato CM. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol 2010;63:882-5.  Back to cited text no. 7
    
8.
Salama S. Cutaneous collagenous vasculopathy. J Cutan Pathol 2013;40:524.  Back to cited text no. 8
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


This article has been cited by
1 A rare cause of blanching red legs: cutaneous collagenous vasculopathy
Simon F. Roy,Feras M. Ghazawi,Bertrand Veilleux,Danielle Bouffard,Annie Bélisle
International Journal of Dermatology. 2018;
[Pubmed] | [DOI]
2 Des anomalies cutanées diffuses
N. Zenati,P.H. Carpentier,S. Blaise
La Revue de Médecine Interne. 2017;
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2595    
    Printed13    
    Emailed1    
    PDF Downloaded219    
    Comments [Add]    
    Cited by others 2    

Recommend this journal