Cutaneous Collagenous Vasculopathy: A Unique Case With Positive Direct Immunofluorescence Findings

Clark, Matthew; McGuinness, Ashley E.; Vidal, Claudia I.

doi:10.1097/dad.0000000000001041

Cited by 4 publications

(3 citation statements)

References 19 publications

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“…Clark et al reported IgG in thickened vessel walls. 19 Salama et al reported deposition of fibrinogen; nevertheless, this case was complicated by intravascular fibrin thrombi. 11 The diagnostic and clinical significance of DIF in CCV remains unclear.…”

Section: Discussionmentioning

confidence: 83%

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A case of cutaneous collagenous vasculopathy associated with multiple myeloma and with a pathogenic variant of the glucocerebrosidase gene

et al. 2022

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Cutaneous collagenous vasculopathy (CCV) is an extremely rare acquired microangiopathy of unknown etiology. The authors describe a case of a 68‐year‐old man, a carrier of a heterozygous pathogenic variant of the glucocerebrosidase (GBA) gene, who was diagnosed with CCV, revealing uncommon fibrinogen positivity in direct immunofluorescence. The patient was subsequently diagnosed with multiple myeloma. Treatment of the myeloma with combined chemotherapy including bortezomib, followed by autologous stem cell transplantation, led to significant reduction of cutaneous lesions. To the best of the authors' knowledge, this is the first published case of CCV in a carrier of a pathogenic variant of the GBA gene, associated with multiple myeloma and with significant regression of CCV after myeloma treatment. Direct immunofluorescence examination revealed an unusual fibrinogen deposition. Hypothetical causative role of bortezomib treatment was proposed regarding significant regression of CCV.

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Section: Discussionmentioning

confidence: 83%

“…DIF examination, performed in a limited number of cases, was negative in most of them. Clark et al reported IgG in thickened vessel walls 19 . Salama et al reported deposition of fibrinogen; nevertheless, this case was complicated by intravascular fibrin thrombi 11 .…”

Section: Discussionmentioning

confidence: 91%

A case of cutaneous collagenous vasculopathy associated with multiple myeloma and with a pathogenic variant of the glucocerebrosidase gene

et al. 2022

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“…4,5 The differential diagnosis for this pattern of microvascular basement membrane zone thickening includes immune-based etiologies attributable to antiendothelial cell antibodies (AECAs), perhaps best exemplified by dermatomyositis; however, non-immune-based endothelial cell injury can result in similar microvascular changes, including those seen in cases of porphyria, pseudoporphyria, and diabetes mellitus. 6 It has been suggested that endothelial cell injury, regardless of the mechanism, leading to endothelial cell denudement is a stimulus to microvascular basement membrane zone thickening. 7 The multiple laminations of the reduplicated basement membrane zones can be highlighted by Type IV collagen and PAS stains.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous collagenous vasculopathy: A report of three cases

et al. 2022

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Cutaneous collagenous vasculopathy is a rare pauci‐inflammatory, superficial, cutaneous vasculopathy characterized by progressive fine‐branching telangiectasias clinically, while light microscopically one observes dilated venules and capillaries within the superficial dermis exhibiting excessive Type IV collagen within the vessel wall. We present three cases of collagenous vasculopathy. Two cases were associated with certain autoimmune stigmata, including a positive serologic anti‐endothelial cell antibody assay and positive lupus anticoagulant in one, while the third case had positive anti‐ribonucleoprotein (RNP) antibodies. The latter case was associated with chronic hydroxyurea therapy for an underlying myeloproliferative disorder. We explore the role of immune‐ and non‐immune‐based endothelial cell injury in the pathogenesis of collagenous vasculopathy.

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