Alpha-1-antitrypsin deficiency panniculitis pathology

Introduction

Panniculitis is a recognized, but rare, complication of α1-antitrypsin (A1AT) deficiency. It is mainly diagnosed in homozygous PiZZ variant.

Histology of panniculitis due to alpha-1-antitrypsin deficiency

Histopathological Examination usually reveals lobar panniculitis, but septal involvement may be seen. In the early stages, neutrophils They are arranged interstitially between collagen bunches from the deep lattice dermis, followed by a neutrophilic mixed panniculitis with lobar necrosis (Figures 1, 2, 3). Vasculitis has been described in association with significant neutrophils infiltration.

Special studies for alpha-1-antitrypsin deficiency panniculitis

Special stains and/or cultures for microorganisms may be useful if an infectious etiology It is suspected.

Differential diagnosis of panniculitis due to alpha-1-antitrypsin deficiency

Neutrophils skin disease: Clinical correlation may be necessary to exclude other neutrophilic dermatoses. Patients are usually known to have alpha-1-antitrypsin deficiency when panniculitis occurs.

Factitious panniculitis: Sections should be polarized to exclude foreign material if a factitious cause is suspected.