White : Diseases of the Skin - Vascular Disorders

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Gary M. White & Neil H. Cox

Diseases of the Skin

15	Vascular Disorders

CLASSIFICATION OF VASCULAR ANOMALIES AND TUMORS

This is a complex area, and a detailed discussion is beyond the scope of this book. Some relevant modern references are provided.
There is a fundamental division into:

•	non-proliferative lesions (malformations), and
•	proliferative lesions (angiomas and other tumors), which may be further subdivided into congenital and acquired, and also into benign, reactive, and malignant types.

Distinction between these has led to historical confusion. For example, the term cavernous hemangioma has been applied to both venous malformations and to true angiomas, but is important in predicting outcome and both the need for and the choice of therapy. This is most relevant in the neonate and childhood in differentiating between a venous malformation and a deeply situated angioma, and is discussed further later in this chapter. Various radiologic tests, immunohistochemistry, and genetic analyses may be necessary to correctly label or to determine the extent of internal organ involvement by such lesions.
Occasionally, malformations and proliferations may coexist even within the same lesion. Examples include nodules within a capillary malformation (port wine stain), most of which develop gradually over time, but pyogenic granulomas may appear suddenly; similarly, spindle cell hemangioendotheliomas may occur on a background of Maffucci syndrome (a rare venous malformation).
Table 15.1 gives a simpli?ed classi?cation.

Fig. 15.1 (a,b) Diascopy. One quick way of con?rming that a lesion is vascular is through diascopy. A clear glass slide is applied to the lesion. With vascular lesions, the red color of contained blood may be completely removed (or nearly so). In addition, the lesion often compresses to a much smaller size.

Table 15.1 CLASSIFICATION OF VASCULAR ANOMALIES

Type of lesion

Subdivision

Example(s)

Comments

Malformations and
ectasias
(non-proliferative)

Capillary (red).

Venous (blue)

Arterial and mixed
malformations

Lymphatic

Port wine stain

Spider angioma
Telangiectasias
Others

Blue rubber bleb nevus syndrome
Glomuvenous malformation

Venous lake

Arterial

Mixed

Small cyst - type lymphangiomas
Large cyst - type lymphangiomas

Also various syndromes that include port wine stain; see
text,this chapter
See text, this chapter

Cutis marmorata telangiectatica congenita, phacomatoses
(pigmented lesions with vascular anomalies)
Note internal organ involvement
Also termed multiple glomangiomas, multiple glomus
tumors
May be solitary (usually on the lip) or multiple (usually
solar-damaged face and neck skin)
Includes arterial malformation and arteriovenous fistula

Various combinations of arterial, venous, capillary, and
lymphatic malformations, subdivided into fast-flow and
slow-flow types
Lymphangioma circumscriptum
Cystic hygroma

Angiomas and tumors (proliferative)

Benign, congenital Benign, acquired

Hemangioma, kaposiform hemangioendothelioma, tufted angioma (some) Cherry angioma, pyogenic granuloma, tufted angioma (some), targetoid hemosiderotic hemangioma, spindle cell hemangioendothelioma, angiolymphoid hyperplasia with eosinophilia, unilateral nevoid telangiectasia Kaposi sarcoma Angiosarcoma, epithelioid hemangioendothelioma

See text, this chapter See text, this chapter See also Chapter 12 and Chapter 33