Case Presentation: A 51year-old female presented with one week of worsening pain and malodorous drainage from a chronic breast wound. Her PMH included HTN induced ESRD on HD. Her wound started 6 months ago as a red rash that grew in size and became tender and ulcerative. Work up thus far included imaging such as a mammogram which showed extensive arteriovascular calcifications and biopsies that showed past angiogenesis without evidence of malignancy. She had already trialed prednisone and dapsone for presumed pyoderma gangrenosum (PG) and sodium thiosulfate in HD for possible calciphylaxis (CP).
She was afebrile with normal vital signs. On exam, there was a 9x5cm ulceration of the right medial breast with seropurulent drainage. Palpation suggested a central fluctuant mass. There were also two smaller, non-ulcerative, hyperpigmented and tender breast lesions on her left breast. Initial labs revealed a leukocytosis to 26 (89% seg neutrophils, no bands).
Work up included an autoimmune panel, which was positive for elevated lupus anticoagulant ratio, PT and anticardiolipin IgG; negative for ANA/ANCA/RF. Vancomycin/cefepime were administered due to the purulent appearance of the wound which later grew pseudomonas and streptococcus. Dermatology and nephrology were both consulted and recommended continued anti-inflammatory prednisone and dapsone as well as thiosulfate. Initial shave biopsy was without evidence of dense neutrophilic infiltrate characteristic of pyoderma gangrenosum; calciphylaxis was favored and a deeper rebiopsy was recommended. Wound debridement with incisional biopsy was performed demonstrating evidence of atherosclerosis, arterial medial calcification, and luminal sclerosis associated with, but non-specific for diffuse dermal angiomatosis (DDA). Definitive pathological identification was impossible due to the extensive necrosis of the tissue sample.
Discussion: DDA is a rare cutaneous vascular disorder associated with atherosclerosis and is characterized by painful, poorly demarcated plaques with central ulceration. While it generally involves the extremities, DDA of the breast (DDAB) has recently been recognized as a unique clinicopathologic entity. Unfortunately, there is no consensus therapy for DDA, however isotretinoin has had limited success. Reduction mammoplasty may be the most definitive treatment in patients with macromastia.
DDA has also been histologically observed in the dermis adjacent to necrotizing ulcers in patients with CP. Among those patients, as in this case, ESRD, HTN, and parathyroidectomy are common co-morbidities. Additionally, this patient had prior mammogram with extensive arteriovascular calcifications suggestive of CP and angiogenesis on past biopsy which may be suggestive of DDA. Angiogenesis seen in DDA is likely a consequence of ischemia and critical tissue hypoxia, which are involved in the pathogenesis of both DDA and CP.
Conclusions: While diffuse dermal angiomatosis and calciphylaxis are both relatively rare entities, they should be considered in patients with non-healing ulcerative breast wounds and may occur in tandem histopathologically.