title: Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy.
abstract: BACKGROUND
  Alpha-1-antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha-1-antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant challenge. Previous attempts using immunosuppressive, anti-inflammatory, and cytotoxic drugs have shown variable results.
  
  
  OBJECTIVE
  To report a case of alpha-1-antitrypsin deficiency-associated panniculitis treated with plasma exchange therapy.
  
  
  METHODS
  A 23-year-old patient developed painful red nodules on her thighs and buttocks with spontaneous ulceration and discharge of oily fluid. A skin biopsy specimen showed septal and lobular panniculitis. The serum alpha-1-antitrypsin level was 22 mg/dL. She was treated with plasma exchange therapy.
  
  
  RESULTS
  Treatment of this patient with plasma exchange therapy led to the control of the cutaneous lesions.
  
  
  CONCLUSIONS
  Plasma exchange therapy represents an alternative treatment which restores serum and tissue alpha-1-antitrypsin levels. This method is proposed because of its clinical benefits and greater availability.
authors.affiliation: Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina, University of São Paulo, São Paulo, Brazil. priscila_o@hotmail.com <priscila_o@hotmail.com>
authors.forename: Priscila
authors.initials: P
authors.lastname: de Oliveira
authors.forename: Libania
authors.initials: L
authors.lastname: Paz-Melgar
authors.forename: Maria Denise F
authors.initials: MD
authors.lastname: Takahashi
authors.forename: Marcello Menta S
authors.initials: MM
authors.lastname: Nico
authors.forename: Evandro A
authors.initials: EA
authors.lastname: Rivitti
authors.forename: Alfredo
authors.initials: A
authors.lastname: Mendrone
authors.forename: Nelson H
authors.initials: NH
authors.lastname: Tatsui
mesh_terms: D000328:Adult; D002081:Buttocks; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007866:Leg; D015434:Panniculitis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D010951:Plasma Exchange; D012883:Skin Ulcer / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D019896:alpha 1-Antitrypsin Deficiency / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000628:therapy