Alpha-1-antitrypsin deficiency-associated panniculitis: a case report.
Panniculitis is a recognized, but rare complication of α1-antitrypsin (A1AT) deficiency. Less than 60 cases have been reported, mostly in the homozygous PiZZ variant. We report the case of a 55-year old woman with A1AT panniculitis associated with the heterozygous phenotype PiMS and discrete reduction of A1AT serum levels. In addition, the pathophysiology of the disease, clinical and histopathological features, and current treatment possibilities are briefly reviewed.
Hospital de Curry Cabral - Centro Hospitalar de Lisboa Central, Lisboa, Portugal.
André
A
Laureano
Rodrigo
R
Carvalho
Alexandra
A
Chaveiro
Jorge
J
Cardoso
D001706:Biopsy; D003078:Colchicine / Q000627:therapeutic use; D000075202:Contraindications; D003622:Dapsone; D004890:Erythema / Q000209:etiology; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications; D006579:Heterozygote; D006801:Humans; D008875:Middle Aged; D015434:Panniculitis / Q000209:etiology* / Q000473:pathology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology
D003622:Dapsone; D003078:Colchicine