• Users Online: 533
  • Print this page
  • Email this page
  • Email this page
  • Email this page
  • Email this page

Table of Contents
Year : 2022  |  Volume : 40  |  Issue : 4  |  Page : 195-196

Recent developments in our understanding of autoinflammatory keratinization diseases

Department of Dermatology, Kaohsiung Medical University Hospital; Department of Dermatology, College of Medicine, Kaohsiung Medical University; Department of Dermatology, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan

Date of Submission08-Dec-2022
Date of Acceptance08-Dec-2022
Date of Web Publication27-Dec-2022

Correspondence Address:
Dr. Stephen Chu-Sung Hu
Department of Dermatology, Kaohsiung Medical University Hospital, No. 100, Tzyou 1st Road, Kaohsiung 807
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1027-8117.365589

Rights and Permissions

How to cite this article:
Hu SC. Recent developments in our understanding of autoinflammatory keratinization diseases. Dermatol Sin 2022;40:195-6

How to cite this URL:
Hu SC. Recent developments in our understanding of autoinflammatory keratinization diseases. Dermatol Sin [serial online] 2022 [cited 2023 Jun 6];40:195-6. Available from: https://www.dermsinica.org/text.asp?2022/40/4/195/365589

Autoinflammatory keratinization disease (AiKD) is an emerging disease concept and is a group of keratinizing skin conditions characterized by increased activation of the innate immune system.[1] These diseases include generalized pustular psoriasis,[2],[3] pityriasis rubra pilaris,[4] hidradenitis suppurativa,[5] and porokeratosis.[6] Recently, the underlying etiologies and molecular mechanisms associated with the development of AiKDs have been gradually elucidated. In this issue of Dermatologica Sinica, a comprehensive review by Akiyama described the clinical features and pathogenesis of various AiKDs.[7] In particular, recent advances in elucidation of underlying signaling pathways and novel treatment strategies were emphasized. It is expected that more skin disorders will be included as AiKDs in the near future, and our increasing understanding regarding their pathogenic mechanisms will lead to the development of new therapeutic modalities.

This issue of Dermatologica Sinica

Recently, with increasing emphasis on the application of evidence-based medicine in clinical practice, there has been a growing number of publications based on systemic reviews and meta-analyses. In order to improve the quality of systemic reviews, various international guidelines have been established in the research community.[8] In this issue, Kuo et al. described the essential steps involved in performing a high-quality systemic review.[9] Understanding these important steps will ensure the quality of systemic reviews and enable the provision of current best evidence for clinical decision-making.

Alopecia areata is a common autoimmune disease characterized by circular patches of hair loss on the scalp and other body regions. Huang and Tsai used a retrospective approach to investigate the therapeutic effect of diphenylcyclopropenone (a topical contact allergen) for alopecia areata in a Taiwanese medical center. They found that topical diphenylcyclopropenone is an effective form of treatment for alopecia areata, with >90% sustainable hair regrowth in 32.6% (28 out of 86) of patients.[10] However, possible side effects associated with this topical treatment modality should be monitored.

Extramammary Paget's disease is a malignant skin condition with high recurrence rate and poor prognosis.[11],[12] In this issue of Dermatologica Sinica, Hou et al. performed a retrospective study to investigate the clinical and pathological characteristics of perianal Paget's disease in a Taiwanese medical center.[13] They found that out of 8 patients with perianal Paget's disease, four cases were associated with underlying rectal or anal adenocarcinoma (secondary extramammary Paget's disease). In addition, they found differential expression of CK7 in the epidermal and dermal cancer cells for two cases with secondary perianal Paget's disease. These findings may have implications for the diagnosis of this skin malignancy.

Recently, COVID-19 vaccines have been administered to the worldwide population. However, there have been reports of cutaneous side effects following vaccination in a minority of people.[14],[15] These adverse effects include new onset or exacerbation of urticaria,[16] psoriasis,[17] atopic dermatitis,[18] and lupus erythematosus.[19] In this issue, Chao and Liu described a patient who developed bullous pemphigoid following AstraZeneca COVID-19 vaccination.[20] Therefore, dermatologists should be aware of the possibility of various immune-mediated cutaneous diseases which may occur following the administration of COVID-19 vaccines.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Akiyama M, Takeichi T, McGrath JA, Sugiura K. Autoinflammatory keratinization diseases: An emerging concept encompassing various inflammatory keratinization disorders of the skin. J Dermatol Sci 2018;90:105-11.  Back to cited text no. 1
Takeichi T, Akiyama M. Generalized pustular psoriasis: Clinical management and update on autoinflammatory aspects. Am J Clin Dermatol 2020;21:227-36.  Back to cited text no. 2
Akiyama M. Pustular psoriasis as an autoinflammatory keratinization disease (AiKD): Genetic predisposing factors and promising therapeutic targets. J Dermatol Sci 2022;105:11-7.  Back to cited text no. 3
Akiyama M, Takeichi T, McGrath JA, Sugiura K. Autoinflammatory keratinization diseases. J Allergy Clin Immunol 2017;140:1545-7.  Back to cited text no. 4
Frew JW. Hidradenitis suppurativa is an autoinflammatory keratinization disease: A review of the clinical, histologic, and molecular evidence. JAAD Int 2020;1:62-72.  Back to cited text no. 5
Takeichi T, Akiyama M. Familial or sporadic porokeratosis as an autoinflammatory keratinization disease. J Dermatol 2019;46:e125-6.  Back to cited text no. 6
Akiyama M. Autoinflammatory keratinization diseases: The concept, diseases involved, and pathogeneses. Dermatol Sin 2022;40:197-203.  Back to cited text no. 7
  [Full text]  
Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: An updated guideline for reporting systematic reviews. BMJ 2021;372:n71.  Back to cited text no. 8
Kuo LT, Shao SC, Chi CC. Ten essential steps for performing a systematic review: A quick tutorial. Dermatol Sin 2022;40:204-6.  Back to cited text no. 9
  [Full text]  
Huang CM, Tsai TF. Topical diphenylcyclopropenone in the treatment of alopecia areata: A 10-year follow-up of 86 cases in a single center. Dermatol Sin 2022;40:214-21.  Back to cited text no. 10
  [Full text]  
Ishizuki S, Nakamura Y. Extramammary Paget's disease: Diagnosis, pathogenesis, and treatment with focus on recent developments. Curr Oncol 2021;28:2969-86.  Back to cited text no. 11
Merritt BG, Degesys CA, Brodland DG. Extramammary Paget disease. Dermatol Clin 2019;37:261-7.  Back to cited text no. 12
Hou PC, Lee CN, Wong TW, Hsu TC, Wu CL, Lee JY. A clinicopathological study of perianal Paget disease: A single center-based cohort study and literature review. Dermatol Sin 2022;40:222-30.  Back to cited text no. 13
  [Full text]  
Gambichler T, Boms S, Susok L, Dickel H, Finis C, Abu Rached N, et al. Cutaneous findings following COVID-19 vaccination: Review of world literature and own experience. J Eur Acad Dermatol Venereol 2022;36:172-80.  Back to cited text no. 14
Bellinato F, Maurelli M, Gisondi P, Girolomoni G. Cutaneous adverse reactions associated with SARS-CoV-2 vaccines. J Clin Med 2021;10:5344.  Back to cited text no. 15
Anvari S, Samarakoon U, Fu X, Jaggers J, Gonzalez-Estrada A, Chong HJ, et al. Urticaria and/or angioedema secondary to mRNA COVID-19 vaccines: Updates from a United States case registry. Allergy 2022;10.1111/all.15447. [doi: 10.1111/all.15447].  Back to cited text no. 16
Krajewski PK, Matusiak Ł, Szepietowski JC. Psoriasis flare-up associated with second dose of Pfizer-BioNTech BNT16B2b2 COVID-19 mRNA vaccine. J Eur Acad Dermatol Venereol 2021;35:e632-4.  Back to cited text no. 17
Potestio L, Napolitano M, Bennardo L, Fabbrocini G, Patruno C. Atopic dermatitis exacerbation after Covid-19 vaccination in Dupilumab-treated patients. J Eur Acad Dermatol Venereol 2022;36:e409-11.  Back to cited text no. 18
Kreuter A, Licciardi-Fernandez MJ, Burmann SN, Burkert B, Oellig F, Michalowitz AL. Induction and exacerbation of subacute cutaneous lupus erythematosus following mRNA-based or adenoviral vector-based SARS-CoV-2 vaccination. Clin Exp Dermatol 2022;47:161-3.  Back to cited text no. 19
Chao YC, Liu KL. New-onset bullous pemphigoid triggered by AstraZeneca COVID-19 vaccine. Dermatol Sin 2022;40:245-6.  Back to cited text no. 20
  [Full text]  


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article

 Article Access Statistics
    PDF Downloaded184    
    Comments [Add]    

Recommend this journal