Generalized pustular psoriasis (von Zumbusch)

Romiti, Ricardo; Hirayama, André Luís da Silva; Arnone, Marcelo; Magalhães, Renata Ferreira

doi:10.1016/j.abd.2021.05.011

Ricardo Romiti

Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil

http://orcid.org/0000-0003-0165-3831

André Luís da Silva Hirayama ^** Corresponding author. E-mail: xkrandre1979@yahoo.com.br (A.L. Hirayama).

Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil

http://orcid.org/0000-0002-6276-3551

Marcelo Arnone

Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil

http://orcid.org/0000-0002-7192-5161

Renata Ferreira Magalhães

Discipline of Dermatology, Faculdade de Ciências Médicas, Unicamp, Campinas, SP, Brazil

http://orcid.org/0000-0001-9170-932X

* Corresponding author. E-mail: xkrandre1979@yahoo.com.br (A.L. Hirayama).

*
Study conducted at the Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brazil and at the Discipline of Dermatology, Faculdade de Ciências Médicas da Unicamp, Campinas, SP, Brazil.

Authors’ contributions

Marcelo Arnone: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis and interpretation of data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.

Renata Ferreira Magalhães: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis and interpretation of data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.

Ricardo Romiti: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.

André Luis da Silva Hirayama: Statistical analysis; approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.

Conflicts of interest

Ricardo Romiti has conflicts of interest with the following laboratories, working as a consultant and/or speaker: Teva, Abbvie, Boeringer-Ingelheim, Janssen, Eli-Lilly, Leo Pharma, Novartis, PPfizer, UCB Biopharma. André L. S. Hirayama has conflicts of interest with the following laboratories, working as a speaker, researcher and in others as a consultant: Abbvie, Boeringer-Ingelheim, Novartis, Janssen, Eli-Lilly. Marcelo Arnone has conflicts of interest with the following laboratories, working as a speaker, researcher and in others as a consultant: Abbvie, Boeringer-Ingelheim,Novartis, Janssen, Eli-Lilly, Leo Pharma and UCB Biopharma. Renata F. Magalhães has conflicts of interest with the following institutions, working as a speaker and in others as a researcher: Abbvie, Novartis, Janssen-Cilag, Eli-Lilly and Unicamp.

Terms	Definition
NFkB	It comprises a sequence of 11 base pairs that interacts with the light chain of the immunoglobulin produced by B lymphocytes, a protein complex that performs functions as a transcription factor, i.e., activated in the cell membrane, it goes to the nucleus to order the transcription of specific genes. The Kappa B Nuclear Factor (NFB) is a family of transcription factors vital to the coordination of inflammatory responses of the innate and adaptive immune systems, cell differentiation, proliferation and survival in almost all multicellular organisms.¹⁵15 Mitchell S, Vargas J, Hoffmann A. Signaling via the NFB system. Wiley Interdiscip Rev: Syst Biol Med. 2016;8:227-41.,²⁰20 omim.org [Internet]. Baltimore: Online Mendelian Inheritance in Man; c1966 [cited 2020 Jul 9]. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available from: www.omim.org www.omim.org...
BCL10	The BCL10 gene (B-cell CLL/lymphoma 10) encodes a protein complex member (CBM), which also contains adapters from the CARD family, for the recruiting of caspases such as CARD9 and MALT1. The CBM complex is involved in NFkB activation after stimulation of several receptors on lymphoid, myeloid and epithelial cells. BCL10 forms heterotrimers with different CARD proteins in different cell types.²⁰20 omim.org [Internet]. Baltimore: Online Mendelian Inheritance in Man; c1966 [cited 2020 Jul 9]. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available from: www.omim.org www.omim.org...
MAPK	The MAPK (mitogen-activated protein kinase) gene encodes the enzyme participating in intracellular signaling pathways.²⁰20 omim.org [Internet]. Baltimore: Online Mendelian Inheritance in Man; c1966 [cited 2020 Jul 9]. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available from: www.omim.org www.omim.org...
MALT1	The MALT1 (mucosa-associated lymphoid tissue lymphoma translocation gene 1) gene or paracaspase encodes a caspase-like cysteine protease essential for the activation of kappa-B nuclear factor downstream of cell surface receptors.²⁰20 omim.org [Internet]. Baltimore: Online Mendelian Inheritance in Man; c1966 [cited 2020 Jul 9]. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available from: www.omim.org www.omim.org...
CXCL	Chemokines comprise a group of small molecules (approximately 8 to 14 kD) that regulate several types of leukocytes through interactions with a subset of 7-transmembrane G protein-coupled receptors. They are divided into two main subfamilies, CXC and CC, based on the arrangement of the first two of the four conserved cysteine residues; the two cysteines are separated by a single amino acid in the CXC chemokines and are adjacent in the CC chemokines. CXC chemokines are subdivided into ELR and non-ELR types, based on the presence or absence of an adjacent glu-leu-arg sequence and N-terminal. ELR types are chemotactic for neutrophils, while non-ELR types are chemotactic for lymphocytes.²⁰20 omim.org [Internet]. Baltimore: Online Mendelian Inheritance in Man; c1966 [cited 2020 Jul 9]. McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD). Available from: www.omim.org www.omim.org... For example, IL-8 is known as chemokine (C-X-C motif) ligand 8, CXCL8.
Inflammasome	The innate immune system has pattern recognition receptors (PRRs) for specific molecular sequences present in microorganisms and conserved during evolution, called pathogen-associated molecular patterns (PAMPs). Toll-like receptors (TLRs) and nucleotide-binding receptors containing repeated sequence domain of leucine amino acid residues (NLRs) are considered to be the main PAMP recognition receptors. TLRs also recognize endogenous molecules called damage-associated molecular patterns (DAMPs), which are released during stress and cell damage. The activation of these receptors increases the expression of genes and factors that control the production of type I interferon and pro-inflammatory cytokines. While TLRs are expressed in the cell membrane, NLRs are cytoplasmic sensors that oligomerize to form an activating protein complex, which is the inflammasome.⁴⁶46 Paiva-Oliveira EL, Silva AC, Silva RM, Sevenini LA, Melo HA, Lagrota-Candido JM, et al. Inflamassoma e sua repercussão clínica: revisão da literatura. Revista de Ciências Médicas e Biológicas. 2012;11:96-102.

[1] * Corresponding author. E-mail: xkrandre1979@yahoo.com.br (A.L. Hirayama).

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Generalized pustular psoriasis (von Zumbusch)^* * Study conducted at the Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brazil and at the Discipline of Dermatology, Faculdade de Ciências Médicas da Unicamp, Campinas, SP, Brazil.

Abstract

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Generalized pustular psoriasis (von Zumbusch)* * Study conducted at the Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brazil and at the Discipline of Dermatology, Faculdade de Ciências Médicas da Unicamp, Campinas, SP, Brazil.

Abstract

Generalized pustular psoriasis (von Zumbusch)^* * Study conducted at the Department of Dermatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, SP, Brazil and at the Discipline of Dermatology, Faculdade de Ciências Médicas da Unicamp, Campinas, SP, Brazil.