Thursday, October 01, 2015

Unusual Vascular Pattern

28 yo man with progressive vascular anomaly of both legs.

History:  The patient is an otherwise healthy 28 years old man with a life-long history of superficial blood vessels of both lower extremities.  This is spreading centripetally. It is painful and has a burning sensation.  No history of edema or ulceration.  No family history.

O/E:  Both lower extremities show prominent superficial arborizing blood vessels.  The red color suggests these are arterioles.  There is no evidence of hemosiderin deposition.

Clinical Images:

Pathology:  A 4 mm punch biopsy was taken.
The specimen exhibits dilated superficial blood vessels surrounded by PAS positive amorphous pink hyaline material. This is consistent with cutaneous collagenous vasculopathy. 
Histopathological Photos taken by  

Hyejin Leah Chung, Dermatopathology Fellow at Boston Medical Center

1. low magnification with dilated superficial blood vessels (H&E, x10)

2. higher magnification with dilated superficial blood vessels surrounded by amorphous pink hyaline material (H&E, x20)

3. PAS stain highlights the amorphous pink material. (PAS, x 20)

Diagnosis:  This does not fit any disorder I have seen. The diagnosis of cutaneous collagenous vasculopathy is interesting.  I think generalized essential telangiectasia needs to be considered as well.

Questions:  Has anyone seen a case?  Any treatment?  Lasers?
1. Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients.  Perez A, Wain ME, Robson A, Groves RW, Stefanato CM.
Abstract: Cutaneous collagenous vasculopathy is characterized by generalized cutaneous telangiectasia and unique microscopic and ultrastructural vascular changes, consisting of marked collagen deposition within the vascular walls of the post-capillary venules in the superficial dermis. There are only 4 previous cases described in the medical literature, all in males, mostly middle-aged. We have recently seen two female patients with clinical and histopathologic features diagnostic of cutaneous collagenous vasculopathy, indicating that it is not restricted to males. As cutaneous collagenous vasculopathy can be clinically indistinguishable from generalized essential telangiectasia, and histopathologic studies are rarely performed for this condition, it is likely that cutaneous collagenous

2. Generalized essential telangiectasia.
Gordon Spratt EA. Dermatol Online J. 2012 Dec 15;18(12):13.
Abstract: Generalized essential telangiectasia, which is a rare condition that is characterized by the progressive development of telangiectases on the skin, is a clinical diagnosis of exclusion. We present a 65-year-old man with a ten-month history of an asymptomatic eruption of the trunk and proximal aspects of the arms and hands that was comprised of macules and patches of telangiectases. The clinical presentation, associated diseases, hypotheses regarding pathogenesis, differential diagnoses, and reports on treatment modalities are reviewed. The relatively new association of this entity with systemic signs that include hemorrhage as well as the occurrence of generalized essential telangiectasia in patients with a history of hepatitis is discussed.

IMAGES IN CLINICAL MEDICINE. Cutaneous Collagenous Vasculopathy.
Ma DL, Vano-Galvan S. N Engl J Med. 2015 Sep 17;373  Free Full Text.  or DHC.

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