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| Year : 2014 | Volume
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| Issue : 2 | Page : 349-350 |
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| Calcific uremic arteriolopathy induced breast necrosis masquerading as breast cancer |
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Smiley Annie George, Suad Abdeen
Department of Histopathology, Mubarak Al Kabir Hospital, Jabriya, Kuwait
Click here for correspondence address and email
| Date of Web Publication | 19-Jun-2014 |
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How to cite this article:
George SA, Abdeen S. Calcific uremic arteriolopathy induced breast necrosis masquerading as breast cancer. Indian J Pathol Microbiol 2014;57:349-50 |
Sir,
Calciphylaxis/calcific uremic arteriolopathy (CUA) is a rare, life-threatening condition of cutaneous necrosis secondary to small and medium-sized vessel calcification, most often seen in patients with end-stage renal disease, preferentially involve areas of higher fat deposition such as the abdomen, buttocks, and upper thighs and is associated with secondary or tertiary hyperparathyroidism. CUA of the breast is rare. [1],[2],[3] Diagnosis of CUA in the breast is imperative as it can closely mimic inflammatory breast cancer.
A 70-year-old female patient presented to the surgical outpatient department with the complaints of tenderness of the left breast with black discoloration of the skin. Physical examination showed painful skin ulceration with surrounding induration. Her past medical history was significant for chronic renal failure, diabetes mellitus, and hypertension and was on regular hemodialysis. A breast biopsy was performed to exclude malignancy. Her laboratory investigations were significant for serum creatinine 363 μmol/L, serum phosphate 6.65 mg/dL and parathyroid hormone level of 86.56 pmol/L. Histology showed skin ulceration, extensive fat necrosis with abscess formation [Figure 1]a and b]. The parenchymal small to medium-sized vessels showed intimal and medial calcification highlighted by von Kossa stain [Figure 2]a and b]. Some of the vessels showed intimal fibrosis and endothelial proliferation with luminal occlusion [Figure 2]c]. There were no features of malignancy. She was treated with wound debridement, antibiotics, and bisphosphonates.
Calcific uremic arteriolopathy, a complex disorder with a multifactorial etiology was first described as a condition of induced hypersensitivity in which tissues respond to appropriate challenging agents with calcium deposition. The current understanding of the pathogenesis of this process implicates elevated calcium and phosphate levels, which exceed their solubility and subsequently deposit as CaPO 4 in vessels. CUA commonly occurs in the setting of renal failure; however, the presence of the same is not an absolute requirement for its causation. Diabetes, obesity, corticosteroids use, immunosuppressive drugs, warfarin use, female sex, and protein C or S deficiency are the risk factors for the development of CUA. [4]
Females are more affected with proximal and distal pattern of involvement; the lower extremities being commonly involved. The lesions of CUA are painful and start as tender red areas developing into a livedoid pattern progressing into indurated plaques, eschar followed by frank ulceration, gangrene or sepsis. [4]  | Figure 1: (a) Extensive skin necrosis (H and E, ×100). (b) Breast tissue with fat necrosis and abscess formation (H and E, ×100)
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 | Figure 2: (a) Special stain highlights calcium deposits in the vessel wall (von Kossa stain, ×200), (b) Small and medium-sized vessel calcification away from the site of necrosis (von Kossa, ×200), (c) Vessel wall with intimal and medial calcification, intimal fibrosis, and luminal occlusion (H and E, ×200)
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Histology shows small to medium-sized vessel mural calcification with or without endovascular fibrosis, extravascular calcification (amorphous luminal calcium) and thrombotic vaso-occlusion leading to ischemic necrosis. [5] The pathological differential diagnosis include dystrophic calcification and Monckeberg's medial calcific sclerosis. Dystrophic calcification occurs in the injured or necrotic tissue with normal serum calcium and phosphate level. Monckeberg's sclerosis show medial calcification of large vessels without associated intimal thickening.
Calcific uremic arteriolopathy has a dismal prognosis often due to secondary sepsis of the ulcers. Treatment modalities include proper wound care, bisphosphonates, sodium thiosulfate, hyperbaric oxygen therapy and parathyroidectomy. [4]
Though CUA of the breast is uncommon, it can mimic a breast malignancy and should be in the differential diagnosis particularly in the clinical setting of chronic renal failure. A timely recognition is required as a delay in diagnosis is associated with high mortality.
 References | |  |
| 1. | Thornton JJ, Dolph J. Breast necrosis: Calciphylaxis a rare cause. Can J Plast Surg 2008;16:165-7. 
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| 2. | Padovan C, Bonfigli F, Ulessi B, Gottardi C, Giansante C, Trevisan G. Calciphylaxis of the breast: A rare disease in the differential diagnosis of breast cancer. Acta Dermatovenerol Croat 2008;16:83-6.
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| 3. | Kay PA, Sanchez W, Rose JF, Farley DR, Reynolds C. Calciphylaxis causing necrotizing mastitis: A case report. Breast 2001;10:540-3.
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| 4. | Bhambri A, Del Rosso JQ. Calciphylaxis: A review. J Clin Aesthet Dermatol 2008;1:38-41.
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| 5. | Essary LR, Wick MR. Cutaneous calciphylaxis. An underrecognized clinicopathologic entity. Am J Clin Pathol 2000;113:280-7.
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Correspondence Address:
Smiley Annie George
Department of Histopathology, Mubarak Al Kabir Hospital, P.O. Box 43787, 32052 Jabriya
Kuwait
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.134749
[Figure 1], [Figure 2] |
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