Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Dermatol. Feb 2, 2018; 7(1): 1-8
Published online Feb 2, 2018. doi: 10.5314/wjd.v7.i1.1
Managing panniculitis in alpha-1 antitrypsin deficiency: Systematic review of evidence behind treatment
Donah K Sabbagh, Behrad Barmayehvar, Thanh Nguyen, Ross G Edgar, Alice M Turner
Donah K Sabbagh, the Medical Research Institute of New Zealand (MRINZ), CSB Building, Wellington Hospital, Newtown, Wellington 6021, New Zealand
Behrad Barmayehvar, Department of Colorectal Surgery, the Royal Wolverhampton Trust, Wolverhampton Road, West Midlands WV10 0QP, United Kingdom
Thanh Nguyen, Department of Intensive Care, United Lincolnshire Hospitals NHS Trust, Lincolnshire LN2 4AX, United Kingdom
Ross G Edgar, Therapy Services, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2WG, United Kingdom
Alice M Turner, Institute of Applied Health Research, University of Birmingham, Birmingham B15 2TT, United Kingdom
Author contributions: Sabbagh DK, Barmayehvar B and Nguyen T contributed equally to this work; Turner AM and Edgar RG designed the study; Edgar RG conducted the literature search; Barmayehvar B and Nguyen T screened the titles and abstracts of retrieved articles, involving a third reviewer (Edgar RG or Turner AM) where required; Turner AM and Edgar RG conducted quality assessment; Sabbagh DK extracted the data, and was checked by another (Barmayehvar B or Nguyen T); Sabbagh DK wrote the majority of the original draft of the paper in consultation with Turner AM; Barmayehvar B and Nguyen T participated in writing the paper; all authors discussed the results and commented on the manuscript; Turner AM was in charge of overall direction and planning.
Conflict-of-interest statement: Edgar RG has received non-commercial research funds from Grifols Biotherapeutics. Edgar RG reports grants from Health Education England (HEE) and the National Institute for Health Research (NIHR), outside the scope of this study.
Data sharing statement: This is not applicable to this review as only data from published works were processed and reviewed.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Alice M Turner, MBChB, MRCP, PhD, Doctor, Senior Lecturer, Honorary Consultant Respiratory Physician, Institute of Applied Health Research, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom.
Telephone: +44-121-3713885 Fax: +44-121-3713887
Received: November 4, 2017
Peer-review started: November 5, 2017
First decision: November 20, 2017
Revised: November 27, 2017
Accepted: December 6, 2017
Article in press: December 6, 2017
Published online: February 2, 2018

To systematically review literature for management of alpha-1 antitrypsin deficiency (AATD) panniculitis.


Multiple databases were searched using combinations of pertinent terms. Articles were selected describing panniculitis treatment in patients with AAT < 11 μmol and/or PiZZ genotype, with no language limitation. All relevant articles were accessed in full text. Independent review of abstracts and full manuscripts was conducted by 2 reviewers, and quality assessment by one reviewer (checked by a second). Data extraction was conducted by one reviewer (checked by a second). Narrative synthesis only was conducted, as data were unsuitable for meta-analysis.


Thirty-two case reports and 4 case series were found. Augmentation therapy (infusions of plasma-derived AAT) was the most successful, with complete resolution of symptoms in all patients. Dapsone is a less expensive option, and it achieved clinical resolution in 62% of patients, but it is very poorly tolerated. Among other single-agent antibiotics, doxycycline was the most successful with complete clinical resolution seen in 33% of patients. Immunosuppressants were largely unsuccessful; 80% of patients exhibited no response. Liver transplantation and therapeutic plasma exchange displayed complete resolution in 66% of patients. Other strategies, such as non-steroidal anti-inflammatory drugs or antibiotics other than dapsone did not show sufficient response rates to recommend their use. Authors note the risk of bias imposed by the type of evidence (case reports, case series) available in this field.


Dapsone is the recommended first line therapy for AATD panniculitis, followed by augmentation therapy. Plasma exchange may be an alternative in the setting of rapidly progressive disease.

Keywords: Alpha-1 antitrypsin deficiency, Dermatological treatment, Panniculitis, Dapsone, Augmentation therapy

Core tip: There is limited evidence regarding treatment of alpha-1 antitrypsin deficiency (AATD)-related panniculitis. This study aims to systematically review literature for management of AATD-related panniculitis. The most effective treatment is augmentation therapy. Dapsone shows moderate success, but is significantly cheaper and easier to administer. Other treatments have shown limited benefit. Recommended first line therapy for AATD-related panniculitis is dapsone, followed by augmentation therapy. Plasma exchange may be an alternative in the setting of rapidly progressive disease, if augmentation were not available.