Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype).
The association of alpha-1 antitrypsin deficiency (PiZ phenotype) with systemic nodular panniculitis has been well documented. Despite reports of cases of systemic nodular panniculitis associated with other alpha-1 antitrypsin deficiency phenotypes, it is still not known if this association is fortuitous rather than causal. We report a case of systemic nodular panniculitis associated with alpha-1 antitrypsin deficiency (PiSS phenotype), with clinico-pathological features similar to those reported in cases associated with the PiZ phenotype.
Pinto
A R
AR
Department of Medicine 2, Hospital de S. João, Porto, Portugal.
Maciel
L S
LS
Carneiro
F
F
Resende
C
C
Chaves
F C
FC
Freitas
A F
AF
D000328:Adult; D006801:Humans; D008297:Male; D015434:Panniculitis / Q000150:complications* / Q000473:pathology; D010641:Phenotype; D019896:alpha 1-Antitrypsin Deficiency