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Cutaneous Collagenous Vasculopathy

A Unique Case With Positive Direct Immunofluorescence Findings

Clark, Matthew L. MD*; McGuinness, Ashley E. BS; Vidal, Claudia I. MD, PhD*,‡

The American Journal of Dermatopathology: January 2019 - Volume 41 - Issue 1 - p 77–79
doi: 10.1097/DAD.0000000000001041
Letters to the Editor

*Department of Dermatology, Saint Louis University School of Medicine, St. Louis, MO

Saint Louis University School of Medicine, St. Louis, MO

Department of Pathology, Saint Louis University School of Medicine, St. Louis, MO

M. L. Clark and A. E. McGuinness contributed equally to this work.

The authors declare no conflicts of interest.

To the Editor:

Cutaneous collagenous vasculopathy (CCV) is an uncommon idiopathic microangiopathy of the dermal blood vessels. Clinical and histologic features aid in distinguishing CCV from similarly presenting conditions. Our case highlights unique positive direct immunofluorescence (DIF) findings that, to our knowledge, have not been previously reported in the literature.

An 80-year-old woman presented with numerous asymptomatic, symmetric blanching macules of the lower extremities. She had no mucosal or nail involvement, no positive family history of telangiectasias, and no clinical evidence of systemic disease or bleeding diathesis. Stasis dermatitis, pigmented purpura, and vasculitis were considered clinically. Punch biopsy of the left lower leg was performed, and histopathologic examination demonstrated an increased wall thickness of vessels with amorphous pink hyaline material in the superficial dermis (Fig. 1A). Extravasated erythrocytes were also seen. Vasculitis and fibrin thrombi were not appreciated. The amorphous pink hyaline material in the vessel walls was highlighted by Periodic Acid-Schiff (Fig. 1B) and was immune reactive with collagen IV (Fig. 1C). Interestingly, DIF showed homogenous vascular staining with IgG (Fig. 1D). A diagnosis of CCV was made.



Since its original description in 2000,1 44 cases of CCV have been described in the literature to date.1–18 Clinically, CCV is characterized by asymptomatic, blanching telangiectatic macules that typically begin on the lower extremities before spreading to the trunk and upper extremities. It is classically found in middle-aged to older individuals with a nearly equal distribution among men and women; however, patients diagnosed with CCV have ranged in age from 16 to 92 years old.2,3 All identified patients with CCV have been Caucasian with the exception of 1 Japanese patient.4 A wide clinical differential for CCV exists including generalized essential telangiectasia, hereditary hemorrhagic telangiectasia, hereditary benign telangiectasia, pigmented purpuric dermatosis (Schamberg's disease), small vessel vasculitis, and telangiectasia eruptive macularis perstans among others.2,4 Histologic features help distinguish CCV from similarly presenting conditions. Histologically, CCV is characterized by dilated vascular structures containing deposits of eosinophilic hyaline material within the vascular wall.1,5 The vascular deposits are derived from basement membrane collagen and are thought to result from reduplication and splitting of the basement membrane zone surrounding small vessels.6,7 Other entities that can histologically show thickened vessel walls are included in Table 1, which has been modified from that reported in Burdick et al.6 While thickened vessels in CCV are secondary to deposits located within vessel walls,6,7 the thickening of vessel walls in stasis dermatitis is due to deposits surrounding the capillary wall.19 The phenomenon of “fibrin cuffs” seen in ulcerative stasis dermatitis is the hypothesized result from raised capillary pressure leading to extravasation of plasma and fibrinogen into the interstitial space around the vessels where the fibrinogen is converted to fibrin.19,20 DIF findings have not been reported in the vast majority of CCV cases. Six cases have had reported DIF findings (Table 2).8–11 Only one other case had positive DIF findings showing 1 + fibrinogen deposits in dilated vessel walls (Table 2).9 This case, however, was also complicated by intravascular fibrin thrombi.9 The authors of that article proposed that the integration of fibrin into the vessel wall may be a result of a reparative perivascular fibrosis.9 We believe the pathogenic mechanism for the homogenous IgG vascular deposits in our case to be nonimmunologically mediated and likely a result of antibody trapping/nonspecific absorption in the thickened vessel walls. This trapping phenomenon has been reported in the literature in biopsies for porphyria cutanea tarda submitted for DIF.21,22





This case highlights a unique case of CCV with homogenous vascular staining with Ig on DIF examination and emphasizes the importance of not misinterpreting these findings as representing a separate disease entity.

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