Cutaneous Collagenous Vasculopathy: A Retrospective Clinical and Histopathologic Analysis of 34 Cases.

BACKGROUND: Cutaneous collagenous vasculopathy (CCV) is a rare acquired microangiopathy that commonly affects middle-aged, fair-skinned individuals. It presents with telangiectatic lesions involving the lower extremities. Histologic analysis is mandatory for diagnosis. OBJECTIVES: To describe and characterize the patients diagnosed with CCV at the Mayo Clinic. METHODS: A multicenter retrospective observational analysis was performed on patients with a clinical and histological diagnosis of CCV at the Mayo Clinic in Florida, Arizona, and Rochester from January 2000 to October 2023. RESULTS: The study included 34 patients, 22 (64.7%) females. The median age at diagnosis was 52 years (range, 12-80 years). CCV lesions were found to affect the lower extremities in 29 (85.3%) patients, followed by the trunk in 14 (41.2%). Nineteen (55.9%) patients presented 1 concomitant medical condition, 8 (23.5%) 2 conditions, and 5 (14.7%) 3 or more medical conditions, such as arterial hypertension 10 (29.4%), type 2 diabetes mellitus 3 (8.8%), hyperlipidemia 3 (8.8%), cardiac disease 3 (8.8%), and hematologic malignancy 3 (8.8%). Thirty-one patients (91.2%) were taking medications, with 19 (55.9%) taking 3 or more. Pathology staining revealed periodic acid-Schiff positivity in 23 of 34 cases (67.6%), and collagen IV in 26 of 34 cases (47.1%). Treatments included pulsed dye laser 3 (8.8%) and topical sirolimus 1 (2.9%). CONCLUSIONS: CCV is an uncommon microangiopathy that affects pediatric, middle-aged, and elderly patients. It involves the lower extremities, trunk, and upper extremities. Patients often present with associated metabolic and cardiovascular conditions and are usually taking at least 1 medication. Treatment options are limited.

Cutaneous collagenous vasculopathy in a pediatric patient.

We report the case of a 13-year-old female who presented with punctate, erythematous macules coalescing into patches on the upper extremities and left thigh. A skin biopsy demonstrated dilated capillary-sized blood vessels in the papillary dermis consistent with a diagnosis of cutaneous collagenous vasculopathy (CCV). To our knowledge, this is the youngest patient to present with CCV and will represent the third pediatric case in the literature.

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

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.

Multiple progressive annular telangiectasias: A clinicopathological variant of cutaneous collagenous vasculopathy?

Telangiectasias are the clinical manifestation of diverse processes affecting blood vessels. Herein we report the case of a 60-year-old man presenting long-standing asymptomatic annular telangiectatic lesions with whitish centers. The histopathologic examination revealed thickened blood dermal vessel walls in the superficial dermis showing reduplication of the basement membrane resembling cutaneous collagenous vasculopathy (CCV). We suggest that this atypical clinicopathological presentation may represent either a localized annular variant of CCV or a previously unreported clinical form of multiple cutaneous telangiectasias.

Cutaneous collagenous vasculopathy: Report of first Japanese case and review of the literature.

Cutaneous collagenous vasculopathy (CCV) is a rare acquired idiopathic microangiopathy characterised by the progressive development of diffuse asymptomatic telangiectasias over the skin. Histologically, the presence of a thick hyaline collagenous wall around the affected capillaries, comprising the accumulation of collagen type IV, is noted. We herein report the case of a 17-year-old Japanese boy with symmetrical patches of diffuse telangiectasias on the bilateral extremities that persisted for 10 months. A histological examination revealed dilated capillaries in the papillary dermis surrounded by thick perivascular deposition of hyaline-like materials, which stained positive for periodic acid-Schiff and collagen type IV. We additionally performed a review of 26 CCV patients previously reported in the English literature and summarised the clinical and histological features of generalised telangiectatic disorders, such as CCV, generalised essential telangiectasia and hereditary haemorrhagic telangiectasia. To establish an accurate diagnosis, it is important for dermatologists to recognise the clinical and histological characteristics of CCV and the importance of the histological analysis.

Extensive Acquired Telangiectasias: Comparison of Generalized Essential Telangiectasia and Cutaneous Collagenous Vasculopathy. / Telangiectasias extensas adquiridas: comparación entre telangiectasia esencial generalizada y vasculopatía colágena cutánea.

The late development of symmetrical, ascending telangiectasias over an extensive area of the skin with no associated systemic manifestations is a common presentation of generalized essential telangiectasia (GET). It was recently suggested that cutaneous collagenous vasculopathy (CCV) is clinically identical to GET but that the 2 conditions can be distinguished by their distinctive histopathologic findings. We present 2 patients, both women, with multiple telangiectasias and describe the histopathologic findings that led to the diagnoses of GET and CCV. Dermoscopic findings in both cases were similar, except that the older telangiectasias in the patient with CCV were violaceous and distributed in a tortuous, serpentine pattern. During follow-up 12 years for the woman with GET and 42 years for the woman with CCV we saw that in GET the lesions remained stable in appearance whereas in CCV there was progressive darkening and morphological changes eventually resulting in superficial varicose veins.

A rare case of cutaneous collagenous vasculopathy.

Cutaneous collagenous vasculopathy is a rare microangiopathy first described by Salama and Rosenthal in 2000. Several cases have been reported to date, describing distinct histological findings of thick hyaline collagenous blood vessel walls in the superficial dermis. Clinical confusion can arise with generalised essential telangiectasia. We report a case occurring in a 76-year-old woman who presented with a 2-year history of a telangiectatic rash progressing from her knees upwards. The diagnosis was confirmed on skin biopsy and treatment with pulsed dye laser was later initiated at the patient's request.

Cutaneous Collagenous Vasculopathy: A Rare Entity Treated With Pulsed Dye Laser.

Cutaneous collagenous vasculopathy (CCV) is a rare idiopathic dermal microangiopathy. Clinically, it presents as diffuse cutaneous telangiectasias that are indistinguishable from other benign vascular entities, thereby posing a diagnostic challenge. We present a case of CCV successfully treated with pulsed dye laser (PDL). A 27-year-old male presented with generalized erythematous macules, diagnosed as CCV via histopathology. After a successful test spot, PDL treatment resulted in significant improvement. The pathogenesis of CCV involves altered dermal microvasculature and veil cell activation. Epidemiologically, it primarily affects Caucasians, most often in the middle-aged adult population. A negative family history of similar lesions can help narrow down the differential diagnosis. Diagnosis requires biopsy, with histopathological examination demonstrating vessel ectasia and collagenous vessel wall thickening. Given its rarity, CCV presents diagnostic and management challenges though PDL emerges as a promising treatment modality for this condition.

A case of cutaneous collagenous vasculopathy / 中华皮肤科杂志

A 22-year-old female patient presented with skin flushing in the bilateral legs for 4 years, which gradually spread throughout the whole lower limbs and forearms 6 months ago. Skin examination showed diffuse flushing and dilated capillaries in the lower limbs and both forearms, and the flushing faded after a press. Histopathological examination of the skin lesion on the leg showed hyperkeratosis in a basket-like shape, increased pigmentation in the basal layer, infiltration of the superficial dermis with scattered lymphocytes, with no obvious red blood cell overflow; periodic acid-Schiff staining showed thickened and homogeneous deposits around the blood vessels; immunohistochemical staining showed thickened blood vessel walls and positive staining for type Ⅳ collagen. Diagnosis: cutaneous collagenous vasculopathy.

Cutaneous collagenous vasculopathy: A rare case report.

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.