Diffuse dermal angiomatosis of the breast

What is diffuse dermal angiomatosis of the breast?

Diffuse dermal angiomatosis of the breast is a rare but distinctive disorder of the skin usually affecting large pendulous female breasts and presenting with painful ulceration and reticulate erythema.

 

 

Who gets diffuse dermal angiomatosis of the breast?

Although diffuse dermal angiomatosis (DDA) affects both sexes, diffuse dermal angiomatosis of the breast (DDAB) has so far only been reported in women, particularly those with large pendulous breasts, who are obese, and are current active or past smokers. The age range has been 20 to 62 years (mean age 47). Diffuse dermal angiomatosis is usually diagnosed in association with peripheral vascular disease.

What causes diffuse dermal angiomatosis of the breast?

Diffuse dermal angiomatosis is considered to be a variant of reactive cutaneous angioendotheliosis with excess vascular proliferation resulting from chronic hypoxaemia of the skin. Breast skin hypoxia is thought to be due to the weight of breast tissue stretching the overlying skin and impeding skin blood flow. DDAB has been reported in women with smaller breasts who have internal mammary artery arteriosclerosis.

What are the clinical features of diffuse dermal angiomatosis of the breast?

Diffuse dermal angiomatosis of the breast is usually bilateral and symmetrical, although it can begin on just one breast. It begins over the lower quadrants on the breasts and may spread to the skin of the upper quadrants.

• Reticulate telangiectatic blood vessels [see Telangiectasia]
• Dusky purple reticulate areas
• Painful recurrent ulceration
• Crusting
• Scars at sites of previous ulcers

How do clinical features vary in differing types of skin?

Diffuse dermal angiomatosis of the breast has been reported in African American women as violaceus to erythematous reticular plaques with induration and ulceration. Telangiectatic vessels may be more difficult to appreciate in skin of colour.

 

Image reproduced with permission from: Adams BJ, Goldberg S, Massey, Takabe K. A cause of an unbearably painful breast, diffuse dermal angiomatosis. Gland Surgery. 2012;1(2):132-5.

What are the complications of diffuse dermal angiomatosis of the breast?

• Bacterial infection of ulcerated skin
• Oozing from ulcers staining underwear

How is diffuse dermal angiomatosis of the breast diagnosed?

Diffuse dermal angiomatosis of the breast can be diagnosed on the history and clinical examination.

Investigations may include:

• Mammography and fluorescent angiography
• Skin biopsy — proliferation of superficial and deep dermal blood vessels showing strong expression of CD31 highlighting the endothelial nature of the proliferating cells.

CD31 immunohistochemistry highlights blood vessels 

Image reproduced from: Galambos J, Simmen CM, Schmid R, Steinmann LS, Kempf W. Diffuse dermal angiomatosis of the breast: a distinct entity in the spectrum of cutaneous reactive angiomatoses – clinicopathologic study of two cases and comprehensive review. Case Rep Dermatol. 2017;9:194-205.

What is the differential diagnosis for diffuse dermal angiomatosis of the breast?

• Livedo reticularis
• Occlusive vasculopathy [see livedoid vasculopathy]
• Cutaneous polyarteritis nodosa

What is the treatment for diffuse dermal angiomatosis of the breast?

General measures

• Smoking cessation
• Weight reduction
• Local wound care
• Analgesia

Specific measures

• Isotretinoin has anti-angiogenic effects and may be beneficial
• Breast reduction surgery
• Treatment of associated vascular disease
• Pentoxifylline
• Topical propranolol
• Topical glyceryl trinitrate

What is the outcome for diffuse dermal angiomatosis of the breast?

Diffuse dermal angiomatosis of the breast tends to follow a recurrent course of episodic painful ulceration. Surgery for peripheral vascular disease or large pendulous breasts can lead to permanent remission.