Diffuse Dermal Angiomatosis of the Breast: A Case Presentation and Discussion

Diffuse dermal angiomatosis (DDA), a rare dermatological disorder and variant of reactive cutaneous angioendotheliomatosis, is characterized clinically by the presence of erythematous and violaceous lesions that have the potential to ulcerate. Although it classically presents in the extremities, a few cases have been reported of DDA involving the breast (DDAB). DDA has often been linked to vaso-occlusive and cardiac co-morbidities, and treatment has therefore usually targeted these underlying conditions. This case presents a patient with DDAB who was successfully treated with isotretinoin therapy, supporting previous reports of its benefit in the management of this patient population. Introduction In 1994, Krell et al. initially recognized diffuse dermal angiomatosis (DDA) as a rare but distinct variant of reactive cutaneous angioendotheliomatosis.1,2 Clinically, it presents as erythematous, violaceous, indurated plaques that are often ulcerated and tender. It generally involves the lower extremities, although only a total of 14 cases of DDA have been cited in the current literature to date.1,2 A form of DDA has also been reported that is localized to the breast (DDAB). Only five documented cases of DDAB have been cited.1 Patients with DDAB often present with intractable breast pain along with these cutaneous lesions.1,3,4 Due to its rarity, the pathogenesis of the disease is not fully understood, but it is thought to be a result of tissue ischemia.1 Numerous studies have reported an association with severe peripheral vascular disease among other co-morbidities.2,5,6 Histologically, diffuse dermal vascularand endothelial-cell proliferation between collagen bundles is seen, and uniform positivity is achieved with immunoperoxidase stains CD31 and CD34, vascular markers characteristic of DDA.1,4,5 The management of DDA and DDAB is centered on improving the underlying ischemia and achieving revascularization. The modalities in current practice include the use of oral corticosteroids, isotretinoin, reduction mammoplasty, and stent placement in extreme cases of vaso-occlusive disease.1,8 In this case report, we present an adult patient with a classic presentation of DDAB who was successfully treated with isotretinoin for a duration of four months. Case Report A 60-year-old Caucasian female presented with a three-month history of exquisitely tender, ulcerating and bleeding breasts, with a tremendous amount of exuded material bilaterally. This eruption started approximately six weeks after cardiac surgery. During the procedure, the patient received heparin, but was not placed on coumadin. She denied exacerbating or alleviating factors. Past medical history was significant for cardiovascular disease, transient ischemic attack, hypertension, and hypercholesterolemia. The patient was a smoker when she was evaluated for this eruption. Her medications upon evaluation included atorvastatin, clopidogrel, lisinopril, metoprolol, and topical lidocaine. Family history was noncontributory. All labs were found to be within normal limits. Physical exam revealed livedo reticularis on the breasts, bilaterally. The left breast (Figure 1) was much more affected than the right (Figure 2), with associated healed punctuate ulcerations and changes of healed infarcts. The rest of her Figure 1 Figure 2 Figure 5 Figure 4 Figure 3