A tale of a reticulated erythematous patch on the breast

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Discussion

The biopsy from the reticular plaque showed proliferation of vessels with turgescent endothelial cells within the dermis and scattered extravasated erythrocytes with hemosiderin [Figure 2]. There was no mitosis or vessel wall inflammation. Immunohistochemistry showed positive staining of endothelial cells for CD34. Staining for human herpesvirus 8 was negative. [Figure 3]. Laboratory investigations were within normal limits. Doppler ultrasound of subclavian arteries did not show any stenosis. Ultrasonic mammography revealed no mass or disrupted architecture. The patient was counselled to quit smoking and reduce weight. Reduction mammoplasty was suggested. Three months later, the ulcer was completely resolved & there was regression of breast patches. [Figure 4]. She had quit smoking, lost 11 kgs & started wearing an adapted bra which she wouldn’t have due to the unavailability of a fitting bra.

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Figure 4

Healing of the ulcer and net regression of the reticular patches over the breast

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Diffuse dermal angiomatosis is a benign acquired vascular proliferation. It was recently recognised as a distinct rare variant of reactive angioendotheliomatosis with different pathological features.^1,2 It is mostly located on the extremities but there is recently an increase in the reporting cases of the breast.² It affects middle-aged women and clinically presents as reticulated, erythematous plaques that are usually multiple and bilateral. Superficial painful ulcerations may occur over the plaque, as in our case, and moreover, induration and necrosis of the plaque may also occur in some advanced cases with severe and chronic tissue hypoxia. Macromastia, obesity, subclavian artery stenosis, hypertension, hypercoagulable states, antiphospholipid syndrome and monoclonal gammopathy are associated with diffuse dermal angiomatosis of the breast.^3,4 Chronic and focal hypoxia induces an up-regulation of vascular endothelial growth factors and stimulate angiogenesis.⁵ Subclinical torsion, compression, repeated microtrauma and increased pressure of large pendulous breasts contribute to chronic hypoxia of focal breast tissue.^2,5 Furthermore, our patient and 27 % of described cases in the literature were heavy smokers.⁵ Indeed, smoking is known to cause endothelial dysfunction and a procoagulant state contributing to vascular damage and hypoxia.⁶

The distinctive histopathologic features of diffuse dermal angiomatosis of the breast are a diffuse proliferation of endothelial cells between collagen bundles, in contrast to the intraluminal proliferation of endothelial cells seen in classic reactive angioendotheliomatosis.⁴ The proliferating cells have a spindle-shaped appearance and vacuolated cytoplasm and form small vascular channels. Atypical endothelial cells and mitosis are absent. The pathological differential diagnoses include Kaposi sarcoma, post-radiation atypical vascular lesion, and low-grade angiosarcoma.⁷ Immunohistochemistry confirms the benign nature of endothelial proliferation. It shows positive vascular markers cluster of differentiation 31 and 34 (CD31, CD34), and Ets-related gene (ERG) with negative human herpesvirus 8 marker.^3,8

Treatment depends on the underlying aetiology. Anti-aggregation may be beneficial for patients with hypercoagulability, especially in the presence of anti-cardiolipin and antinuclear antibodies. Aspirin and pentoxifylline were used and showed variable degrees of success.^4,9 Furthermore, isotretinoin and corticosteroids have been described as an effective therapy in controlling the disease given their angiogenetic effects.⁵ Revascularisation of the subclavian artery has been described in cases associated with arterial obstruction. In addition, an excision of the painful and necrotic lesions may be proposed in severe cases.¹⁰ Reduction mammoplasty is proposed for patients with large pendulous breasts.^3,5,10 The strict control of cardiovascular risk factors and smoking cessation is mandatory.³