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Skin-nontumor / Clinical Dermatology

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

● 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

Raised red spots on skin

Ulcer on upper extremity

Case report

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

Micro 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

Micro images

Lobular panniculitis with neutrophils

Positive stains

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

Differential diagnosis

Bacterial cellulitis: infectious etiology

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

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