PMID- 29707779
OWN - NLM
STAT- MEDLINE
DCOM- 20181115
LR  - 20181115
IS  - 1365-4632 (Electronic)
IS  - 0011-9059 (Linking)
VI  - 57
IP  - 8
DP  - 2018 Aug
TI  - Alpha-1 antitrypsin deficiency panniculitis: clinical and pathologic 
      characteristics of 10 cases.
PG  - 952-958
LID - 10.1111/ijd.14012 [doi]
AB  - BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) may be associated with liver 
      and lung disease and rarely causes panniculitis. OBJECTIVE: We evaluated the 
      clinicopathologic and laboratory findings of AATD panniculitis in 10 patients. 
      METHODS: We conducted a retrospective review of all cases of AATD panniculitis at 
      Mayo Clinic, Rochester, MN, from 1989 to 2016. RESULTS: Ten patients with AATD 
      panniculitis were included. Seven of ten were women. Clinical lesions were most 
      commonly nodular (100%), erythematous (90%), ulcerated (90%), and painful (90%) 
      subcutaneous nodules and plaques. Extracutaneous associations were rare. PiZZ 
      phenotype was most commonly identified (50%). Histopathologically, lobular 
      panniculitis (80%) with associated septal involvement (60%) and a predominant 
      neutrophilic infiltrate (100%) were most common. Treatments varied; dapsone and 
      alpha-1 proteinase inhibitor infusions were each used in five (50%) patients, 
      respectively. In patients with greater than 6-month follow-up (n = 4), one 
      patient continued to have disease activity despite treatment. CONCLUSION: AATD 
      panniculitis should be considered in the differential for a painful, ulcerative 
      panniculitis with a predominantly neutrophilic histopathologic infiltrate. 
      Diagnosis can be made with clinicopathologic correlation and genetic and 
      laboratory evaluations. Serum AAT level and phenotype assists in diagnosing 
      patients with suspected AATD panniculitis.
CI  - (c) 2018 The International Society of Dermatology.
FAU - Johnson, Emma F
AU  - Johnson EF
AUID- ORCID: 0000-0003-3962-1771
AD  - Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
FAU - Tolkachjov, Stanislav N
AU  - Tolkachjov SN
AUID- ORCID: 0000-0003-0725-1649
AD  - Surgical Dermatology Group, Birmingham, AL, USA.
FAU - Gibson, Lawrence E
AU  - Gibson LE
AD  - Department of Dermatology, Mayo Clinic, Rochester, MN, USA.
AD  - Division of Dermatopathology, Mayo Clinic, Rochester, MN, USA.
LA  - eng
PT  - Journal Article
DEP - 20180429
PL  - England
TA  - Int J Dermatol
JT  - International journal of dermatology
JID - 0243704
RN  - 0 (Anti-Infective Agents)
RN  - 0 (Trypsin Inhibitors)
RN  - 0 (alpha 1-Antitrypsin)
RN  - 8W5C518302 (Dapsone)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Anti-Infective Agents/therapeutic use
MH  - Dapsone/therapeutic use
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Neutrophils/*pathology
MH  - Panniculitis/drug therapy/*etiology/*pathology
MH  - Phenotype
MH  - Retrospective Studies
MH  - Skin Ulcer/etiology
MH  - Treatment Outcome
MH  - Trypsin Inhibitors/therapeutic use
MH  - Young Adult
MH  - alpha 1-Antitrypsin/therapeutic use
MH  - alpha 1-Antitrypsin Deficiency/*complications/drug therapy/pathology
EDAT- 2018/05/01 06:00
MHDA- 2018/11/16 06:00
CRDT- 2018/05/01 06:00
PHST- 2017/08/30 00:00 [received]
PHST- 2018/03/14 00:00 [revised]
PHST- 2018/04/02 00:00 [accepted]
PHST- 2018/05/01 06:00 [pubmed]
PHST- 2018/11/16 06:00 [medline]
PHST- 2018/05/01 06:00 [entrez]
AID - 10.1111/ijd.14012 [doi]
PST - ppublish
SO  - Int J Dermatol. 2018 Aug;57(8):952-958. doi: 10.1111/ijd.14012. Epub 2018 Apr 29.