Skin nontumor

Panniculitis

Alpha-1-antitrypsin deficiency panniculitis



Last author update: 1 July 2011
Last staff update: 28 August 2020

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PubMed Search: Alpha-1-antitrypsin deficiency panniculitis

Mowafak Hamodat, M.B.Ch.B., M.Sc.
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Cite this page: Hamodat M. Alpha-1-antitrypsin deficiency panniculitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumora1antitrypsin.html. Accessed March 28th, 2024.
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
Clinical images

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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
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