Diffuse dermal angiomatosis of the breast D18.01

Diffuse dermal angiomatosis (DDA) is a rare, reactive angioproliferation of the skin and is considered a subtype of diffuse cutaneous angiomatosis. DDA primarily affects the extremities of patients with severe vascular disease and other comorbidities. In the past, involvement of the breasts was considered an absolute rarity.

However, it appears that diffuse cutaneous angiomatosis is more common on the breast than previously thought (Galambos J et al 2017). The predisposing concomitant circumstances differ from those of DDA at other body sites, thus making diffuse dermal angiomatosis of the female breast a definable clinicopathologic entity in the spectrum of cutaneous reactive angiomatoses.

Age at manifestation: 47-58 years (Tollefson MM et al. 2014); mean age: 48.4 years.

Women with large breasts are often affected (Sanz-Motilva Vet al. 2014). Furthermore, the disease is associated with various underlying diseases (obesity, vascular diseases, heavy smoking, adipose tissue necrosis, etc.), many of which can lead to local tissue ischemia. Vascular diseases such as occlusion of the subclavian artery have been described in association with the clinical picture. Furthermore, the clinical picture has been described in association with a large calcified and thrombosed artery with concomitant adipose tissue necrosis (Strausburg MB et al. 2016).

Clinically, this rare disease manifests as red-purple, purplish papules and/or extensive plaques (some with a tendency to necrosis and ulceration) of one or both breasts. The "breast" localization typically affects middle-aged women with macromastia, overweight, or obesity. Individual cases are reminiscent of diffuse growing breast carcinoma or chronic recurrent erysipelas, with patchy, irregularly bordered, reddish-blue discolored plaques. Others rather a livedo vasculitis(livedo racemosa). The pain symptoms vary and range from mild to unbearable. It is not uncommon for painful ulceration to occur over the violet lesions (Adams BJ et al 2012).

Mammography showed diffuse cutaneous and trabecular thickening DD: inflammatory breast carcinoma.

Histological findings showed diffuse proliferation of spindle-shaped endothelial cells with focal formation of small vessels that occupied the entire thickness of the dermis, with few extravasated erythrocytes that were positive for CD31, CD34 and SMA-α and negative for D2-40 (Frikha F et al 2018).

Breast carcinoma; chronic erysipelas of the breast;

Currently, there is no consensus on the best therapeutic approach. Isotretinoin and other drug therapies have been used with limited success. Revascularization (if appropriate), oral corticosteroids (if livedoid), oral anticoagulation (not very successful), and smoking cessation (Yang H et al. 2006; Reusche R et al. 2015) have been shown to be useful.

Surgical breast reduction appears to be a viable treatment option for DDAB in women with macromastia and may provide a definitive cure.

1. Adams BJ et al (2012) A cause of unbearably painful breast, diffuse dermal angiomatosis. Gland Surg 1:132-135.
2. Nguyen N et al (2020) Diffuse dermal angiomatosis of the breast. Proc (Bayl Univ Med Cent) 33: 273-275.
3. Hui Y et al (2018) Diffuse dermal angiomatosis mimicking inflammatory breast carcinoma. Breast J 24:196-198.
4. Yang H et al (2006) Diffuse dermal angiomatosis of the breast. Arch Dermatol 142:343-347.
5. Sanz-Motilva Vet al (2014) Diffuse dermal angiomatosis of the breast: clinical and histopathological features. Int J Dermatol 53:445-449.
6. Frikha F et al. (2018) Diffuse dermal angiomatosis of the breast with adjacent fat necrosis: a case report and review of the literature. Dermatol Online J 24:13030/qt1vq114n7
7. Galambos J et al. (2017) Diffuse Dermal Angiomatosis of the Breast: A Distinct Entity in the Spectrum of Cutaneous Reactive Angiomatoses - Clinicopathologic Study of Two Cases and Comprehensive Review of the Literature. Case Rep Dermatol 9:194-205.
8. Hui Y et al (2018) Diffuse dermal angiomatosis mimicking inflammatory breast carcinoma. Breast J 24:196-198.

9. Nguyen N et al (2020) Diffuse dermal angiomatosis of the breast. Proc (Bayl Univ Med Cent). 33:273-275.

10. Reusche R et al. (2015) Diffuse dermal angiomatosis of the breast: a series of 22 cases from a single institution. Gland Surg 4:554-560.
11. Strausburg MB et al (2016) Diffuse dermal angiomatosis of the breast With an Apparent Etiology of Underlying Calcified Thrombosed Artery With Adjacent Fat Necrosis. Am J Dermatopathol 38:838-841.
12. Tollefson MM et al (2014) Diffuse dermal angiomatosis of the breast: clinicopathologic study of 5 patients. J Am Acad Dermatol 71:1212-1217.