Skin nontumor
Panniculitis
Alpha-1-antitrypsin deficiency panniculitis
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Last staff update: 28 August 2020
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PubMed Search: Alpha-1-antitrypsin deficiency panniculitis
Table of Contents
Clinical features | Case reports | Clinical images | Microscopic (histologic) description | Positive stains | Differential diagnosisCite this page: Hamodat M. Alpha-1-antitrypsin deficiency panniculitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumora1antitrypsin.html. Accessed September 21st, 2023.
Clinical features
- Patients have low serum alpha-1-antitrypsin
- Cutaneous lesions are diffuse indurated erythematous plaques or nodules that drain serosangineous fluid, on extensor surfaces of arms, legs, thighs, chest, back and abdomen
- Also pulmonary effusion, embolism edema and anasarca
Case reports
- 47 year old woman with with ulcerating, painful skin lesions (Cleve Clin J Med 2008;75:414)
Clinical images
Images hosted on other servers:
Ulcer on upper extremity
Microscopic (histologic) description
- Early: diffuse neutrophilic infiltrate through dermis into subcutaneous septa, followed by destruction of collagen, reduction of inflammatory cells, detachment of lobules of fat from fibrous septa in a background of neutrophils
- Draining lesions have channels lined by squamous epithelium
- Late: extensive scarring of dermis and subcutis
Positive stains
- Immunoglobulin IgM and C3 may be found in blood vessel walls
Differential diagnosis
- Bacterial cellulitis: infectious etiology
