Abstract

A 65 y.o. Caucasian female with a past medical history of alpha-1 antitrypsin (AAT) deficiency and ductal carcinoma in situ of the left breast presented to dermatology clinic with a one month history of painful nodules on the trunk and extremities. With regard to her AAT deficiency, she was followed yearly by her local pulmonologist and had never required therapy. Laboratory review was notable for an unremarkable complete blood count and a low alpha-1 antitrypsin level of 46 mg/dL (range: 83 - 199 mg/dL). Punch biopsy specimen from an erythematous, subcutaneous nodule with oily drainage on the left upper back demonstrated an interstitial infiltrate of neutrophils, eosinophils, and histiocytes with dermal and subcutaneous fat necrosis. Tissue, fungal, and acid fast bacillus cultures were negative. A diagnosis of alpha-1 antitrypsin deficiency panniculitis was rendered based on pathology. The patient was initiated on doxycycline 100 mg twice daily. Augmentation therapy and alternative options such as dapsone were considered but not initiated given improvement with doxycycline. As AAT deficiency panniculitis most commonly occurs in areas of trauma, there was concern for worsening of her panniculitis following surgical intervention for her breast cancer. Ultimately, the decision was made to proceed with prophylactic mastectomy of her left breast. After a successful mastectomy and three months of doxycycline, the patient has had no new subcutaneous nodules arise and prior nodules have healed. She recently underwent definitive oncoplastic reconstruction of her left breast with future plans to taper her doxycycline provided her panniculitis remains quiescent.

Financial Disclosure:
No current or relevant financial relationships exist.