Skin-nontumor / Clinical Dermatology - Alpha-1-antitrypsin deficiency panniculitis

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Other dermatoses

Alpha-1-antitrypsin deficiency panniculitis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 18 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Clinical features
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● 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

Clinical images
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Raised red spots on skin

Ulcer on upper extremity

Case report
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47 year old woman with with ulcerating, painful skin lesions (Cleve Clin J Med 2008;75:414)

Micro description
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● 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

Micro images
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Lobular panniculitis with neutrophils

Positive stains
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● Immunoglobulin IgM and C3 may be found in blood vessel walls

Differential diagnosis
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● Bacterial cellulitis: infectious etiology

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Alpha-1-antitrypsin deficiency panniculitis

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