CLINICAL HISTORY:

Signs and symptoms:  The lesions are asymptomatic

Previous Treatment:  None

Other information:  The patient believes the condition started three years ago and has slowly progressed. The patient denies pain, pruritus, or burning of the skin. There are no aggravating or alleviating factors

PHYSICAL EXAM:

The patient has diffuse, prominent telangiectasias on his forearms, posterior arms, flanks, thighs, calves, and superior to the umbilicus. There is no significant scale. Mucosal membranes appear normal.

LABORATORY TESTS:

CBC, CMP, cholesterol panel, ANA, all normal

DERMATOHISTOPATHOLOGY:

Right posterior upper arm, and right posterior calf: “Both specimens are characterized by ectasias of the superficial plexus with the dilated vessels remarkable for a thickened hyalinized eosinophilic rim. This cuticle stains completely with an immunohistochemical marker directed against collagen IV confirming a composition of collagen.”

DIFFERENTIAL DIAGNOSIS:

1.   Generalized Essential Telangiectasia
2.   Hereditary hemorrhagic telangiectasia
3.   Cutaneous Collagenous Vasculopathy
4.  
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Cutaneous Collagenous Vasculopathy

DISCUSSION:

Cutaneous collagenous vasculopathy (CCV) is a microangiopathy of unknown origin that affects the superficial cutaneous vessels of the horizontal dermal plexus. Review of the literature does not show a genetic predisposition for this condition. This entity was first described in 2000 by Salama and Rosenthal in a 54 year-old male with a five-year history of spreading, asymptomatic, and generalized cutaneous telangiectasias.
The histopathological findings in CCV are what make this entity unique. CCV is characterized by dilation of superficial cutaneous vessels with perivascular deposition of eosinophilic hyaline material which is PAS positive and diastase resistant. Immunohistochemical studies demonstrate that the deposits are composed of type IV collagen that possesses an abnormal ultrastructural banding pattern (Luse bodies). Luse bodies are not unique to CCV, since they have also been described in other entities, including benign schwannomas.
Since CCV presents in the 5th to 6th decade, disorders of childhood containing telangiectasias can be ruled out as a possible cause. Differential diagnoses that may be considered are hereditary hemorrhagic telangiectasia (HHT) and generalized essential telangiectasia (GET).
HHT, also known as Osler-Weber-Rendu syndrome, is primarily an autosomal dominant disorder, but may present without a family history. Classic signs and symptoms of HHT include: telangiectasias of the mucous membranes and acral skin, epistaxis, recurrent hemorrhages of the GI tract, and AVMs of the lung, liver, and CNS.
Generalized essential telangiectasia, first described by McCrea and Winkelmann in 1927, is typified by symmetrical telangiectasias starting on the feet, ankles, and distal legs, and gradually progresses to involve the trunk and arms. Generalized essential telangiectasia like CCV does not exhibit visceral involvement or display a hemorrhagic tendency. However, the perivascular eosinophilic hyaline material and positive staining for type IV collagen seen in CCV is not a feature found in the dilated vessels of GET, thus allowing a distinction between the two entities.
Capillary telangiectasias have proven to be relatively refractory to treatment. No treatment modality has consistently been successful in removing capillary telangiectasias, although case reports describe benefit in using photothermal coagulation with laser and intense pulsed light.
CCV is strictly limited to the skin, and the reported cases have yet to demonstrate systemic disease. At this point long-term prognosis is unknown, and future insights into this condition may show cutaneous collagenous vasculopathy to be a marker for connective tissue or vascular diseases.

TREATMENT:

No treatment is necessary as the lesions are asymptomatic. Intense pulsed light may aid in reducing the appearance of the lesions.

REFERENCES:

1. Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol. 2000:27;40-48.
2. Davis TL et al. Collagenous vasculopathy: a report of three cases. J Cutan Pathol. 2008:35;967-970.
3. Kanitakis J, Faisant M, Wagschal D, Haftek M, Claudy A. Cutaneous collagenous vasculopathy: Ultrastructural and immunohistochemical study of a new case. Am J Clin Dermatol. 2010:11;63-66.

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