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Cutaneous Collagenous Vasculopathy
To the Editor:
Cutaneous microangiopathy describes pathology of the small blood vessels within the dermis.
We report a case of CCV in a 41-year-old woman who presented for evaluation of a rash on the bilateral lower extremities of 7 to 8 months’ duration. The eruption had started on the left ankle and spread over several weeks to the bilateral dorsal feet followed by the ankles and shins. The patient noted associated swelling and a pressure like dysesthesia of the lower legs. She was otherwise in good health, though she had started an oral contraceptive 1 year prior for heavy menstrual bleeding. A review of systems was negative for deep vein thrombosis, pulmonary embolus, and other thromboembolic phenomena, and the patient had no history of hepatic or renal dysfunction, cancer, or heart disease. Her family history was negative for clotting disorders or bleeding diatheses.
On physical examination, telangiectatic matting was present on the bilateral ankles and dorsal feet with an associated blanchable erythema (Figure 1). The matting extended into a fine, mottled, pretibial telangiectasia associated with Schamberg purpura. She had no pitting edema, and both dorsalis pedis and posterior popliteal pulses were intact and symmetric bilaterally. No popliteal lymphadenopathy or palpable cords were present.
Two punch biopsies taken from the erythematous telangiectatic area on the left foot and metatarsal region demonstrated an unremarkable epidermis without interface change, thickening of the epidermal basement membrane, or single-cell dyskeratosis. There was mild dilatation of blood vessels within the superficial dermis with mild perivascular lymphocytic inflammation and rare extravasated erythrocytes. Leukocytoclastic debris, fibrinoid necrosis of vessel walls, and endothelial cell necrosis were not seen. As is classic in CCV, the vessel walls appeared thickened by eosinophilic hyaline material, which was periodic acid–Schiff positive and diastase resistant (Figure 2). Sclerotic thickening of collagen bundles or absence of periadnexal adipose tissue was not seen. CD34 immunohistochemical staining demonstrated normal retained CD34 interstitial dermal positivity, which excluded morphea. Additionally, direct immunofluorescence testing was negative for IgG, IgA, IgM, C3, fibrin, and C1q. Nodular reduplication of vessels or other changes of stasis were not seen. Fibrin thrombi or neoplastic cells were not identified. The clinical and histopathologic findings were suggestive of CCV.
Prior case reports of CCV have described a similar clinical manifestation with blanching macules that occur symmetrically on the lower extremities and spread cephalically.1-6 A distinction from hereditary hemorrhagic telangiectasia is the noninvolvement of mucous membranes and nails. The etiology of this rare microangiopathy has not been elucidated, though disease concurrence with local trauma, stressful events such as childbirth, and diabetes mellitus has been documented.6 As the body of literature continues to grow, more research regarding the etiology, mechanism, prognosis, and treatment options will enhance our understanding of CCV.
- Bondier L, Tardieu M, Leveque P, et al. Cutaneous collagenous vasculopathy: report of two cases presenting as disseminated telangiectasias and review of the literature. Am J Dermatopathol. 2017;39:682-688.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol. 2000;27:40-48.
- Lloyd BM, Pruden SJ, Lind AC, et al. Cutaneous collagenous vasculopathy: report of the first pediatric case. Pediatr Dermatol. 2011;28:598-599.
- Salama S, Chorneyko K, Belovic B. Cutaneous collagenous vasculopathy associated with intravascular occlusive fibrin thrombi. J Cutan Pathol. 2014;41:386-393.
- Perez A, Wain ME, Robson A, et al. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol. 2010;63:882-885.
- Burdick LM, Losher S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
To the Editor:
Cutaneous microangiopathy describes pathology of the small blood vessels within the dermis.
We report a case of CCV in a 41-year-old woman who presented for evaluation of a rash on the bilateral lower extremities of 7 to 8 months’ duration. The eruption had started on the left ankle and spread over several weeks to the bilateral dorsal feet followed by the ankles and shins. The patient noted associated swelling and a pressure like dysesthesia of the lower legs. She was otherwise in good health, though she had started an oral contraceptive 1 year prior for heavy menstrual bleeding. A review of systems was negative for deep vein thrombosis, pulmonary embolus, and other thromboembolic phenomena, and the patient had no history of hepatic or renal dysfunction, cancer, or heart disease. Her family history was negative for clotting disorders or bleeding diatheses.
On physical examination, telangiectatic matting was present on the bilateral ankles and dorsal feet with an associated blanchable erythema (Figure 1). The matting extended into a fine, mottled, pretibial telangiectasia associated with Schamberg purpura. She had no pitting edema, and both dorsalis pedis and posterior popliteal pulses were intact and symmetric bilaterally. No popliteal lymphadenopathy or palpable cords were present.
Two punch biopsies taken from the erythematous telangiectatic area on the left foot and metatarsal region demonstrated an unremarkable epidermis without interface change, thickening of the epidermal basement membrane, or single-cell dyskeratosis. There was mild dilatation of blood vessels within the superficial dermis with mild perivascular lymphocytic inflammation and rare extravasated erythrocytes. Leukocytoclastic debris, fibrinoid necrosis of vessel walls, and endothelial cell necrosis were not seen. As is classic in CCV, the vessel walls appeared thickened by eosinophilic hyaline material, which was periodic acid–Schiff positive and diastase resistant (Figure 2). Sclerotic thickening of collagen bundles or absence of periadnexal adipose tissue was not seen. CD34 immunohistochemical staining demonstrated normal retained CD34 interstitial dermal positivity, which excluded morphea. Additionally, direct immunofluorescence testing was negative for IgG, IgA, IgM, C3, fibrin, and C1q. Nodular reduplication of vessels or other changes of stasis were not seen. Fibrin thrombi or neoplastic cells were not identified. The clinical and histopathologic findings were suggestive of CCV.
Prior case reports of CCV have described a similar clinical manifestation with blanching macules that occur symmetrically on the lower extremities and spread cephalically.1-6 A distinction from hereditary hemorrhagic telangiectasia is the noninvolvement of mucous membranes and nails. The etiology of this rare microangiopathy has not been elucidated, though disease concurrence with local trauma, stressful events such as childbirth, and diabetes mellitus has been documented.6 As the body of literature continues to grow, more research regarding the etiology, mechanism, prognosis, and treatment options will enhance our understanding of CCV.
To the Editor:
Cutaneous microangiopathy describes pathology of the small blood vessels within the dermis.
We report a case of CCV in a 41-year-old woman who presented for evaluation of a rash on the bilateral lower extremities of 7 to 8 months’ duration. The eruption had started on the left ankle and spread over several weeks to the bilateral dorsal feet followed by the ankles and shins. The patient noted associated swelling and a pressure like dysesthesia of the lower legs. She was otherwise in good health, though she had started an oral contraceptive 1 year prior for heavy menstrual bleeding. A review of systems was negative for deep vein thrombosis, pulmonary embolus, and other thromboembolic phenomena, and the patient had no history of hepatic or renal dysfunction, cancer, or heart disease. Her family history was negative for clotting disorders or bleeding diatheses.
On physical examination, telangiectatic matting was present on the bilateral ankles and dorsal feet with an associated blanchable erythema (Figure 1). The matting extended into a fine, mottled, pretibial telangiectasia associated with Schamberg purpura. She had no pitting edema, and both dorsalis pedis and posterior popliteal pulses were intact and symmetric bilaterally. No popliteal lymphadenopathy or palpable cords were present.
Two punch biopsies taken from the erythematous telangiectatic area on the left foot and metatarsal region demonstrated an unremarkable epidermis without interface change, thickening of the epidermal basement membrane, or single-cell dyskeratosis. There was mild dilatation of blood vessels within the superficial dermis with mild perivascular lymphocytic inflammation and rare extravasated erythrocytes. Leukocytoclastic debris, fibrinoid necrosis of vessel walls, and endothelial cell necrosis were not seen. As is classic in CCV, the vessel walls appeared thickened by eosinophilic hyaline material, which was periodic acid–Schiff positive and diastase resistant (Figure 2). Sclerotic thickening of collagen bundles or absence of periadnexal adipose tissue was not seen. CD34 immunohistochemical staining demonstrated normal retained CD34 interstitial dermal positivity, which excluded morphea. Additionally, direct immunofluorescence testing was negative for IgG, IgA, IgM, C3, fibrin, and C1q. Nodular reduplication of vessels or other changes of stasis were not seen. Fibrin thrombi or neoplastic cells were not identified. The clinical and histopathologic findings were suggestive of CCV.
Prior case reports of CCV have described a similar clinical manifestation with blanching macules that occur symmetrically on the lower extremities and spread cephalically.1-6 A distinction from hereditary hemorrhagic telangiectasia is the noninvolvement of mucous membranes and nails. The etiology of this rare microangiopathy has not been elucidated, though disease concurrence with local trauma, stressful events such as childbirth, and diabetes mellitus has been documented.6 As the body of literature continues to grow, more research regarding the etiology, mechanism, prognosis, and treatment options will enhance our understanding of CCV.
- Bondier L, Tardieu M, Leveque P, et al. Cutaneous collagenous vasculopathy: report of two cases presenting as disseminated telangiectasias and review of the literature. Am J Dermatopathol. 2017;39:682-688.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol. 2000;27:40-48.
- Lloyd BM, Pruden SJ, Lind AC, et al. Cutaneous collagenous vasculopathy: report of the first pediatric case. Pediatr Dermatol. 2011;28:598-599.
- Salama S, Chorneyko K, Belovic B. Cutaneous collagenous vasculopathy associated with intravascular occlusive fibrin thrombi. J Cutan Pathol. 2014;41:386-393.
- Perez A, Wain ME, Robson A, et al. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol. 2010;63:882-885.
- Burdick LM, Losher S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
- Bondier L, Tardieu M, Leveque P, et al. Cutaneous collagenous vasculopathy: report of two cases presenting as disseminated telangiectasias and review of the literature. Am J Dermatopathol. 2017;39:682-688.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol. 2000;27:40-48.
- Lloyd BM, Pruden SJ, Lind AC, et al. Cutaneous collagenous vasculopathy: report of the first pediatric case. Pediatr Dermatol. 2011;28:598-599.
- Salama S, Chorneyko K, Belovic B. Cutaneous collagenous vasculopathy associated with intravascular occlusive fibrin thrombi. J Cutan Pathol. 2014;41:386-393.
- Perez A, Wain ME, Robson A, et al. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients. J Am Acad Dermatol. 2010;63:882-885.
- Burdick LM, Losher S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
Practice Points
- In cutaneous collagenous vasculopathy (CCV), skin biopsy may demonstrate eosinophilic hyaline thickening of superficial dermal blood vessels with mild perivascular lymphocytic inflammation and rare extravasated erythrocytes.
- Lack of mucous membrane and nail involvement differentiates CCV from hereditary hemorrhagic telangiectasia.