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Psoriasis

MedGen UID:
10997
Concept ID:
C0033860
Disease or Syndrome
Synonym: Psoriases
SNOMED CT: Psoriasis (9014002)
 
HPO: HP:0003765
Monarch Initiative: MONDO:0005083
OMIM® Phenotypic series: PS177900

Definition

A skin abnormality characterized by redness and irritation, with thick, red skin that displays flaky, silver-white patches (scales). [from HPO]

Conditions with this feature

Mycosis fungoides
MedGen UID:
7771
Concept ID:
C0026948
Neoplastic Process
Mycosis fungoides is a malignant T-cell lymphoma of the skin, first reported (and named) by Alibert (1835). Sezary syndrome is a leukemic variant of mycosis fungoides defined by erythroderma with greater than 80% of the skin showing redness, adenopathy and greater than 1,000 circulating Sezary cells/microliter with a CD4+CD26- or CD4+CD7- phenotype. Sezary cells have a type 2 helper T cell cytokine profile. Sezary syndrome has a median overall survival time of only 2.4 years in patients with Sezary cells at a density of greater than 10,000 cells/microliter or 5.4 years in patients with 1,000-10,000 Sezary cells/microliter. Mycosis fungoides and Sezary syndrome are the most common cutaneous T-cell lymphomas. Sezary syndrome can arise de novo or can appear following years of chronic mycosis fungoides. Both are thought to arise from clonal expansion of CD4+ helper T cells responding to chronic antigen stimulation (summary by Wang et al., 2015).
Ankylosing spondylitis
MedGen UID:
11561
Concept ID:
C0038013
Disease or Syndrome
Spondyloarthropathy (SpA), one of the commonest chronic rheumatic diseases, includes a spectrum of related disorders comprising the prototype ankylosing spondylitis (AS), a subset of psoriatic arthritis (PsA), reactive arthritis (ReA), arthritis associated with inflammatory bowel disease, and undifferentiated spondyloarthropathy (Miceli-Richard et al., 2004). These phenotypes are difficult to differentiate because they may occur simultaneously or sequentially in the same patient. Studies have suggested that a predominant shared component, including HLA-B27, predisposes to all phenotypic subsets, and that these subsets should be considered as various phenotypic expressions of the same disease (Said-Nahal et al., 2000, Said-Nahal et al., 2001). Braun and Sieper (2007) provided a detailed review of ankylosing spondylitis, including clinical features, pathogenesis, and management. Genetic Heterogeneity of Susceptibility to Spondyloarthropathy Additional susceptibility loci for spondyloarthropathy have been identified on chromosome 9q31-q34 (SPDA2; 183840) and chromosome 2q36 (SPDA3; 613238).
Pustular psoriasis, generalized
MedGen UID:
473074
Concept ID:
C0343055
Disease or Syndrome
Generalized pustular psoriasis (GPP) is a life-threatening disease characterized by sudden, repeated episodes of high-grade fever, generalized rash, and disseminated pustules, with hyperleukocytosis and elevated serum levels of C-reactive protein (123260) (summary by Marrakchi et al., 2011). GPP often presents in patients with existing or prior psoriasis vulgaris (PV; see 177900); however, GPP can develop without a history of PV (Sugiura et al., 2013). Palmoplantar pustulosis and acrodermatitis continua of Hallopeau represent acral forms of pustular psoriasis that have historically been grouped with GPP (summary by Setta-Kaffetzi et al., 2013). GPP in association with sterile multifocal osteomyelitis and periostitis (612852) is caused by mutation in the IL1RN gene (147679). Capon (2013) noted that the percentage of GPP patients reported to be negative for mutation in IL36RN ranges from 51 to 84%, indicative of genetic heterogeneity in the generalized pustular form of psoriasis. For a discussion of genetic heterogeneity of psoriasis, see PSORS1 (177900).
Lig4 syndrome
MedGen UID:
339855
Concept ID:
C1847827
Disease or Syndrome
A hereditary disorder associated with impaired DNA double-strand break repair mechanisms with characteristics of microcephaly, unusual facial features, growth and developmental delay, skin anomalies, and pancytopenia, which is associated with combined immunodeficiency. Caused by mutations in the LIG4 gene (13q22-q34). The resulting defect of DNA ligase IV, a component of the classical non-homologous end-joining (NHEJ) pathway, affects the major mechanism of DNA double-strand break repair. Transmission is autosomal recessive.
Interleukin 2 receptor, alpha, deficiency of
MedGen UID:
377894
Concept ID:
C1853392
Disease or Syndrome
Immunodeficiency-41 is an autosomal recessive complex disorder of immune dysregulation. Affected individuals present in infancy with recurrent viral, fungal, and bacterial infections, lymphadenopathy, and variable autoimmune features, such as autoimmune enteropathy and eczematous skin lesions. Immunologic studies show a defect in T-cell regulation (summary by Goudy et al., 2013).
Spondyloarthropathy, susceptibility to, 1
MedGen UID:
400145
Concept ID:
C1862852
Finding
Ankylosing spondylitis is a form of ongoing joint inflammation (chronic inflammatory arthritis) that primarily affects the spine. This condition is characterized by back pain and stiffness that typically appear in adolescence or early adulthood. Over time, back movement gradually becomes limited as the bones of the spine (vertebrae) fuse together. This progressive bony fusion is called ankylosis.\n\nThe earliest symptoms of ankylosing spondylitis result from inflammation of the joints between the pelvic bones (the ilia) and the base of the spine (the sacrum). These joints are called sacroiliac joints, and inflammation of these joints is known as sacroiliitis. The inflammation gradually spreads to the joints between the vertebrae, causing a condition called spondylitis. Ankylosing spondylitis can involve other joints as well, including the shoulders, hips, and, less often, the knees. As the disease progresses, it can affect the joints between the spine and ribs, restricting movement of the chest and making it difficult to breathe deeply. People with advanced disease are also more prone to fractures of the vertebrae.\n\nAnkylosing spondylitis affects the eyes in up to 40 percent of cases, leading to episodes of eye inflammation called acute iritis. Acute iritis causes eye pain and increased sensitivity to light (photophobia). Rarely, ankylosing spondylitis can also cause serious complications involving the heart, lungs, and nervous system.
Psoriasis susceptibility 2
MedGen UID:
351141
Concept ID:
C1864497
Disease or Syndrome
Psoriasis susceptibility 1
MedGen UID:
357279
Concept ID:
C1867449
Finding
Psoriasis (psoriasis vulgaris; PV) is a chronic inflammatory dermatosis that affects approximately 2% of the population. It is characterized by red, scaly skin patches that are usually found on the scalp, elbows, and knees, and may be associated with severe arthritis. The lesions are caused by abnormal keratinocyte proliferation and infiltration of inflammatory cells into the dermis and epidermis. The usual age of onset of psoriasis is between 15 and 30 years, although it can present at any age (summary by Matthews et al., 1996). Generalized pustular psoriasis (GPP) is a life-threatening disease characterized by sudden, repeated episodes of high-grade fever, generalized rash, and disseminated pustules, with hyperleukocytosis and elevated serum levels of C-reactive protein (123260) (summary by Marrakchi et al., 2011). GPP often presents in patients with existing or prior psoriasis vulgaris; however, GPP can develop without a history of PV (Sugiura et al., 2013). Palmoplantar pustulosis and acrodermatitis continua of Hallopeau represent acral forms of pustular psoriasis that have historically been grouped with GPP (summary by Setta-Kaffetzi et al., 2013). Nestle et al. (2009) provided a detailed review of the pathogenesis and genetics of psoriasis. Genetic Heterogeneity of Psoriasis and Psoriasis Susceptibility PSORS2 (602723) is caused by mutation in the CARD14 gene (607211) on chromosome 17q25, and PSORS14 (614204) is caused by mutation in the IL36RN gene (605507) on chromosome 2q14. Psoriasis susceptibility loci include PSORS1 on 6p21.3; PSORS3 (601454) on 4q; PSORS4 on 1q21; PSORS5 (604316) on 3q21; PSORS6 (605364) on 19p; PSORS7 (605606) on 1p; PSORS8 (610707) on 16q; PSORS9 (607857) on 4q31; PSORS10 (612410) on 18p11; PSORS11 (612599) on 5q31-q33; PSORS12 (612950) on 20q13; PSORS13 (614070), conferred by variation in the TRAF3IP2 gene (607043) on 6q21; and PSORS15 (616106), conferred by variation in the AP1S3 gene (615781) on 2q36. An additional putative psoriasis candidate locus has been reported on 20p (Nair et al., 1997).
Mental retardation and psoriasis
MedGen UID:
477241
Concept ID:
C3275610
Disease or Syndrome
A rare, X-linked syndromic intellectual disability disorder characterized by severe intellectual disability, psychomotor developmental delay, generalized seizures, and psoriasis. Mild craniofacial dysmorphism, such as hypertelorism, broad nasal bridge, anteverted nares, macrostomia, highly arched palate and large ears, is also associated. There have been no further descriptions in the literature since 1988.
Erythroderma, congenital, with palmoplantar keratoderma, hypotrichosis, and hyper-ige
MedGen UID:
816049
Concept ID:
C3809719
Disease or Syndrome
A rare genetic epidermal disorder with characteristics of congenital erythroderma with severe psoriasiform dermatitis, ichthyosis, severe palmoplantar keratoderma, yellow keratosis on the hands and feet, elevated immunoglobulin E, multiple food allergies, and metabolic wasting. Other variable features may include hypotrichosis, nail dystrophy, recurrent infections, mild global developmental delay, eosinophilia, nystagmus, growth impairment and cardiac defects.
Common variable immunodeficiency 10
MedGen UID:
816321
Concept ID:
C3809991
Disease or Syndrome
Common variable immunodeficiency-10 (CVID10) is an autosomal dominant primary immunodeficiency characterized by childhood-onset of recurrent infections, hypogammaglobulinemia, and decreased numbers of memory and marginal zone B cells. Some patients may develop autoimmune features and have circulating autoantibodies. An unusual feature is central adrenal insufficiency (summary by Chen et al., 2013). For a general description and a discussion of genetic heterogeneity of common variable immunodeficiency, see CVID1 (607594).
Autoimmune lymphoproliferative syndrome type V
MedGen UID:
863651
Concept ID:
C4015214
Disease or Syndrome
Autoimmune lymphoproliferative syndrome type V is an autosomal dominant complex immune disorder characterized by autoimmune thrombocytopenias and abnormal lymphocytic infiltration of nonlymphoid organs, including the lungs, brain, and gastrointestinal tract, resulting in enteropathy. Some patients may show features of an immunodeficiency syndrome with recurrent infections, but immunosuppressive therapy often results in clinical improvement (summary by Kuehn et al., 2014). For a general description and a discussion of genetic heterogeneity of ALPS, see 601859.
Psoriasis 15, pustular, susceptibility to
MedGen UID:
863672
Concept ID:
C4015235
Finding
While many affected individuals have features only of GPP (called GPP alone), some develop features of another skin condition called psoriasis vulgaris (PV), either before or after GPP appears. PV, the most common form of psoriasis, is characterized by red, scaly patches of skin (plaques) on parts of the body.\n\nGeneralized pustular psoriasis (GPP) is a severe form of a skin disorder called psoriasis. GPP and other forms of psoriasis are caused by abnormal inflammation. Inflammation is a normal immune system response to injury and foreign invaders (such as bacteria). However, when inflammation is abnormal and uncontrolled, it can damage the body's tissues and organs. Individuals with GPP have repeated episodes in which large areas of skin become red and inflamed and develop small pus-filled blisters (pustules). The skin problems can be accompanied by fever, extreme tiredness (fatigue), muscle weakness, an increased number of white blood cells, and other signs of inflammation throughout the body (systemic inflammation). The inflammation problems subside and reappear often. Episodes can be triggered by infection, exposure to or withdrawal from certain medications, menstruation, or pregnancy, although the trigger is often unknown. GPP can be life-threatening if not treated.
Microcephaly, congenital cataract, and psoriasiform dermatitis
MedGen UID:
900653
Concept ID:
C4225189
Disease or Syndrome
SC4MOL deficiency represents an inborn error of cholesterol metabolism that is characterized by accumulation of a large amount of methylsterols, particularly dimethylsterols, in affected patients. The associated features of immune dysregulation, skin disease, and growth delay can be at least partially corrected with cholesterol and statin supplements (He et al., 2014).
Bleeding disorder, platelet-type, 21
MedGen UID:
1386863
Concept ID:
C4479515
Disease or Syndrome
BDPLT21 is a hematologic disorder characterized by increased risk of bleeding resulting from a functional platelet defect. Platelets have decreased or even absent dense bodies and abnormally enlarged and fused alpha-granules, and they show defective secretion and aggregation responses to agonists. Platelets are usually enlarged, and some patients may have mild to moderate thrombocytopenia (summary by Saultier et al., 2017).
Immunodeficiency, common variable, 14
MedGen UID:
1614928
Concept ID:
C4540380
Disease or Syndrome
Multiple gastrointestinal atresias
MedGen UID:
1708537
Concept ID:
C5234880
Disease or Syndrome
Gastrointestinal defects and immunodeficiency syndrome (GIDID) is characterized by multiple intestinal atresia, in which atresia occurs at various levels throughout the small and large intestines. Surgical outcomes are poor, and the condition is usually fatal within the first month of life. Some patients exhibit inflammatory bowel disease (IBD), with or without intestinal atresia, and in some cases, the intestinal features are associated with either mild or severe combined immunodeficiency (Samuels et al., 2013; Avitzur et al., 2014; Lemoine et al., 2014).

Recent clinical studies

Etiology

Zheng J, Chen W, Gao Y, Chen F, Yu N, Ding Y, Liu N
J Dermatol 2021 Sep;48(9):1336-1342. Epub 2021 May 21 doi: 10.1111/1346-8138.15958. PMID: 34018629
Graetz C, Woeste S, Mrowietz U, Ehrenthal JC
BMC Oral Health 2021 Mar 4;21(1):95. doi: 10.1186/s12903-021-01457-8. PMID: 33663457Free PMC Article
Beytout Q, Pepiot J, Maruani A, Devulder D, Aubert R, Beylot-Barry M, Amici JM, Jullien D, Mahé E; Association France Psoriasis.; Research Group on Psoriasis of the French Society of Dermatology (GrPso).; Research Group of the French Society of Pediatric Dermatology (SFPD).
Ann Dermatol Venereol 2021 Jun;148(2):106-111. Epub 2021 Feb 23 doi: 10.1016/j.annder.2021.01.005. PMID: 33637347Free PMC Article
Mahil SK, Dand N, Mason KJ, Yiu ZZN, Tsakok T, Meynell F, Coker B, McAteer H, Moorhead L, Mackenzie T, Rossi MT, Rivera R, Mahe E, Carugno A, Magnano M, Rech G, Balogh EA, Feldman SR, De La Cruz C, Choon SE, Naldi L, Lambert J, Spuls P, Jullien D, Bachelez H, McMahon DE, Freeman EE, Gisondi P, Puig L, Warren RB, Di Meglio P, Langan SM, Capon F, Griffiths CEM, Barker JN, Smith CH; PsoProtect study group.
J Allergy Clin Immunol 2021 Jan;147(1):60-71. Epub 2020 Oct 16 doi: 10.1016/j.jaci.2020.10.007. PMID: 33075408Free PMC Article
Elmets CA, Korman NJ, Prater EF, Wong EB, Rupani RN, Kivelevitch D, Armstrong AW, Connor C, Cordoro KM, Davis DMR, Elewski BE, Gelfand JM, Gordon KB, Gottlieb AB, Kaplan DH, Kavanaugh A, Kiselica M, Kroshinsky D, Lebwohl M, Leonardi CL, Lichten J, Lim HW, Mehta NN, Paller AS, Parra SL, Pathy AL, Siegel M, Stoff B, Strober B, Wu JJ, Hariharan V, Menter A
J Am Acad Dermatol 2021 Feb;84(2):432-470. Epub 2020 Jul 30 doi: 10.1016/j.jaad.2020.07.087. PMID: 32738429

Diagnosis

Luo Y, Qu K, Kuai L, Ru Y, Huang K, Yan X, Xing M
Mol Genet Genomics 2021 Sep;296(5):1027-1040. Epub 2021 Jun 17 doi: 10.1007/s00438-021-01804-y. PMID: 34137900
Zheng J, Chen W, Gao Y, Chen F, Yu N, Ding Y, Liu N
J Dermatol 2021 Sep;48(9):1336-1342. Epub 2021 May 21 doi: 10.1111/1346-8138.15958. PMID: 34018629
Li YJ, Zhou T, Zhang J, Zhang L, Ke H, Zhang C, Li P
J Ethnopharmacol 2021 Mar 25;268:113551. Epub 2020 Nov 3 doi: 10.1016/j.jep.2020.113551. PMID: 33152434
Elmets CA, Korman NJ, Prater EF, Wong EB, Rupani RN, Kivelevitch D, Armstrong AW, Connor C, Cordoro KM, Davis DMR, Elewski BE, Gelfand JM, Gordon KB, Gottlieb AB, Kaplan DH, Kavanaugh A, Kiselica M, Kroshinsky D, Lebwohl M, Leonardi CL, Lichten J, Lim HW, Mehta NN, Paller AS, Parra SL, Pathy AL, Siegel M, Stoff B, Strober B, Wu JJ, Hariharan V, Menter A
J Am Acad Dermatol 2021 Feb;84(2):432-470. Epub 2020 Jul 30 doi: 10.1016/j.jaad.2020.07.087. PMID: 32738429
Cao D, Shen M, Chen X, Xiao Y, Lu W, Luo Y, Zhu W, Kuang Y
Eur J Dermatol 2020 Dec 1;30(6):674-679. doi: 10.1684/ejd.2020.3914. PMID: 33459259

Therapy

Zheng J, Chen W, Gao Y, Chen F, Yu N, Ding Y, Liu N
J Dermatol 2021 Sep;48(9):1336-1342. Epub 2021 May 21 doi: 10.1111/1346-8138.15958. PMID: 34018629
Beytout Q, Pepiot J, Maruani A, Devulder D, Aubert R, Beylot-Barry M, Amici JM, Jullien D, Mahé E; Association France Psoriasis.; Research Group on Psoriasis of the French Society of Dermatology (GrPso).; Research Group of the French Society of Pediatric Dermatology (SFPD).
Ann Dermatol Venereol 2021 Jun;148(2):106-111. Epub 2021 Feb 23 doi: 10.1016/j.annder.2021.01.005. PMID: 33637347Free PMC Article
Li YJ, Zhou T, Zhang J, Zhang L, Ke H, Zhang C, Li P
J Ethnopharmacol 2021 Mar 25;268:113551. Epub 2020 Nov 3 doi: 10.1016/j.jep.2020.113551. PMID: 33152434
Mahil SK, Dand N, Mason KJ, Yiu ZZN, Tsakok T, Meynell F, Coker B, McAteer H, Moorhead L, Mackenzie T, Rossi MT, Rivera R, Mahe E, Carugno A, Magnano M, Rech G, Balogh EA, Feldman SR, De La Cruz C, Choon SE, Naldi L, Lambert J, Spuls P, Jullien D, Bachelez H, McMahon DE, Freeman EE, Gisondi P, Puig L, Warren RB, Di Meglio P, Langan SM, Capon F, Griffiths CEM, Barker JN, Smith CH; PsoProtect study group.
J Allergy Clin Immunol 2021 Jan;147(1):60-71. Epub 2020 Oct 16 doi: 10.1016/j.jaci.2020.10.007. PMID: 33075408Free PMC Article
Elmets CA, Korman NJ, Prater EF, Wong EB, Rupani RN, Kivelevitch D, Armstrong AW, Connor C, Cordoro KM, Davis DMR, Elewski BE, Gelfand JM, Gordon KB, Gottlieb AB, Kaplan DH, Kavanaugh A, Kiselica M, Kroshinsky D, Lebwohl M, Leonardi CL, Lichten J, Lim HW, Mehta NN, Paller AS, Parra SL, Pathy AL, Siegel M, Stoff B, Strober B, Wu JJ, Hariharan V, Menter A
J Am Acad Dermatol 2021 Feb;84(2):432-470. Epub 2020 Jul 30 doi: 10.1016/j.jaad.2020.07.087. PMID: 32738429

Prognosis

Zheng J, Chen W, Gao Y, Chen F, Yu N, Ding Y, Liu N
J Dermatol 2021 Sep;48(9):1336-1342. Epub 2021 May 21 doi: 10.1111/1346-8138.15958. PMID: 34018629
Vandikas MS, Landin-Wilhelmsen K, Holmäng A, Gillstedt M, Osmancevic A
J Steroid Biochem Mol Biol 2021 Jul;211:105895. Epub 2021 Apr 2 doi: 10.1016/j.jsbmb.2021.105895. PMID: 33819632
Schwade MJ, Tien L, Waller JL, Davis LS, Baer SL, Mohammed A, Young L, Kheda MF, Bollag WB
Am J Med Sci 2021 Jul;362(1):24-33. Epub 2021 Mar 30 doi: 10.1016/j.amjms.2021.03.009. PMID: 33798461
Mahil SK, Dand N, Mason KJ, Yiu ZZN, Tsakok T, Meynell F, Coker B, McAteer H, Moorhead L, Mackenzie T, Rossi MT, Rivera R, Mahe E, Carugno A, Magnano M, Rech G, Balogh EA, Feldman SR, De La Cruz C, Choon SE, Naldi L, Lambert J, Spuls P, Jullien D, Bachelez H, McMahon DE, Freeman EE, Gisondi P, Puig L, Warren RB, Di Meglio P, Langan SM, Capon F, Griffiths CEM, Barker JN, Smith CH; PsoProtect study group.
J Allergy Clin Immunol 2021 Jan;147(1):60-71. Epub 2020 Oct 16 doi: 10.1016/j.jaci.2020.10.007. PMID: 33075408Free PMC Article
Chen W, Ding Y, Lu J, Shi Y, Gao Y, Peng C
Dermatol Ther 2020 Nov;33(6):e14227. Epub 2020 Sep 11 doi: 10.1111/dth.14227. PMID: 32844504

Clinical prediction guides

Malara G, Politi C, Trifirò C, Verduci C, D'Arrigo G, Testa A, Tripepi G
Acta Derm Venereol 2021 Sep 15;101(9):adv00545. doi: 10.2340/00015555-3846. PMID: 34043021
El-Komy MHM, Abdelnaby A, El-Kalioby M
J Cosmet Dermatol 2021 Jun;20(6):1573-1579. Epub 2021 Apr 3 doi: 10.1111/jocd.14104. PMID: 33811728Free PMC Article
Graetz C, Woeste S, Mrowietz U, Ehrenthal JC
BMC Oral Health 2021 Mar 4;21(1):95. doi: 10.1186/s12903-021-01457-8. PMID: 33663457Free PMC Article
Chen C, Hou G, Zeng C, Ren Y, Chen X, Peng C
Theranostics 2021;11(2):754-767. Epub 2021 Jan 1 doi: 10.7150/thno.51154. PMID: 33391503Free PMC Article
Li YJ, Zhou T, Zhang J, Zhang L, Ke H, Zhang C, Li P
J Ethnopharmacol 2021 Mar 25;268:113551. Epub 2020 Nov 3 doi: 10.1016/j.jep.2020.113551. PMID: 33152434

Recent systematic reviews

Bellinato F, Gisondi P, Maurelli M, Girolomoni G
Dermatol Ther 2021 Mar;34(2):e14889. Epub 2021 Feb 25 doi: 10.1111/dth.14889. PMID: 33595861
Camela E, Ocampo-Garza SS, Cinelli E, Villani A, Fabbrocini G, Megna M
Dermatol Ther 2021 Mar;34(2):e14857. Epub 2021 Feb 17 doi: 10.1111/dth.14857. PMID: 33559275
Huang IH, Wu PC, Yang TH, Li H, Huang YT, Cheng YC, Kuo PH, Lee YH, Huang YC, Tu YK
J Am Acad Dermatol 2021 Jul;85(1):135-143. Epub 2021 Jan 19 doi: 10.1016/j.jaad.2021.01.024. PMID: 33482253
Pompili M, Bonanni L, Gualtieri F, Trovini G, Persechino S, Baldessarini RJ
J Psychosom Res 2021 Feb;141:110347. Epub 2020 Dec 26 doi: 10.1016/j.jpsychores.2020.110347. PMID: 33453551
Iskandar IYK, Parisi R, Griffiths CEM, Ashcroft DM; Global Psoriasis Atlas.
Br J Dermatol 2021 Feb;184(2):243-258. Epub 2020 Jun 21 doi: 10.1111/bjd.19169. PMID: 32358790

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