Generalized pustular psoriasis
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Generalized 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.
Descrizione
Generalized 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.
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 characterised by red, scaly patches of skin (plaques) on parts of the body.
Modelli di ereditarietà
The source lists the following possible inheritance patterns: Autosomal recessive; Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Altri nomi
Acute generalised pustular psoriasis
Deficiency of the interleukin-36 receptor antagonist
DITRA
Generalized pustular psoriasis of von Zumbusch
GPP
Von Zumbusch psoriasis
Related patient information and medicines
Patient information leaflet
Psoriasi
Psoriasis is a long-term skin condition that can also affect the nails and joints. Nail psoriasis may also occur alone without any skin rash. Pustular psoriasis This is the second most common type of psoriasis. Rarely, a form of pustular psoriasis can affect skin apart from the palms and soles. This more widespread form is a more serious form of.
Patient information leaflet
Eruzioni cutanee
There are many different types of eruzioni cutanee and many different causes of skin rashes. Although most skin rashes are harmless, some do need treatment (which may be tablets, creams or ointments) from your doctor or pharmacist. If filled with yellow fluid (pus) then it is called a pustule. Lichen planus. Psoriasi. Folliculitis. Red (erythema) and.
Patient information leaflet
Allergia ai farmaci
Medicines can cause allergic reactions. In this leaflet these reactions are called 'drug allergies'. In this instance the drugs referred to are medicines people take for their health. All sorts of skin rashes are possible. These include: Acneform lesions - acne spots on the upper part of the body. Acute generalised exanthematous pustulosis (AGEP).
Patient information leaflet
Problemi del pene, prurito al pene e dolore al pene
It can also be caused by infections not linked to sexual activity, such as thrush. Our separate leaflet on Balanitis has more information. An itchy foreskin can be caused by posthitis, which means inflammation of the foreskin. Allergies. Irritants. More generalised skin problems such as psoriasis. Other skin conditions that can make your penis.
Patient information leaflet
Steroidi topici
Steroids are medicines that are used to treat a large number of conditions that cause inflammation - for example, eczema, psoriasi, hay fever, allergies and certain conditions of the eyes. They work by reducing inflammation and irritation. They cause a narr... This leaflet gives an overview of topical steroids, including their main possible.
Patient information leaflet
Prurito vulvare
Some sexually transmitted infections , such as trichomoniasis and genital warts. Sensitivity of the vulval skin Sensitivity of the vulval skin is the most common cause of persistent vulval itch. The vulval skin can become sensitive to anything that comes into contact with it, such as: Creams, including treatments for, for example, thrush. Bath.
Ulteriori letture e riferimenti
- Generalized pustular psoriasis; MedlinePlus, National Library of Medicine.
- Hussain S, Berki DM, Choon SE, Burden AD, Allen MH, Arostegui JI, Chaves A, Duckworth M, Irvine AD, Mockenhaupt M, Navarini AA, Seyger MMB, Soler-Palacin P, Prins C, Valeyrie-Allanore L, Vicente MA, Trembath RC, Smith CH, Barker JN, Capon F. IL36RN mutations define a severe autoinflammatory phenotype of generalized pustular psoriasis. J Allergy Clin Immunol. 2015 Apr;135(4):1067-1070.e9. doi: 10.1016/j.jaci.2014.09.043. Epub 2014 Nov 12. No abstract available.
- Jordan CT, Cao L, Roberson ED, Duan S, Helms CA, Nair RP, Duffin KC, Stuart PE, Goldgar D, Hayashi G, Olfson EH, Feng BJ, Pullinger CR, Kane JP, Wise CA, Goldbach-Mansky R, Lowes MA, Peddle L, Chandran V, Liao W, Rahman P, Krueger GG, Gladman D, Elder JT, Menter A, Bowcock AM. Rare and common variants in CARD14, encoding an epidermal regulator of NF-kappaB, in psoriasis. Am J Hum Genet. 2012 May 4;90(5):796-808. doi: 10.1016/j.ajhg.2012.03.013. Epub 2012 Apr 19.
- Korber A, Mossner R, Renner R, Sticht H, Wilsmann-Theis D, Schulz P, Sticherling M, Traupe H, Huffmeier U. Mutations in IL36RN in patients with generalized pustular psoriasis. J Invest Dermatol. 2013 Nov;133(11):2634-2637. doi: 10.1038/jid.2013.214. Epub 2013 May 6. No abstract available.
- Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S, Zribi J, Bal E, Cluzeau C, Chrabieh M, Towne JE, Douangpanya J, Pons C, Mansour S, Serre V, Makni H, Mahfoudh N, Fakhfakh F, Bodemer C, Feingold J, Hadj-Rabia S, Favre M, Genin E, Sahbatou M, Munnich A, Casanova JL, Sims JE, Turki H, Bachelez H, Smahi A. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011 Aug 18;365(7):620-8. doi: 10.1056/NEJMoa1013068.
- Onoufriadis A, Simpson MA, Pink AE, Di Meglio P, Smith CH, Pullabhatla V, Knight J, Spain SL, Nestle FO, Burden AD, Capon F, Trembath RC, Barker JN. Mutations in IL36RN/IL1F5 are associated with the severe episodic inflammatory skin disease known as generalized pustular psoriasis. Am J Hum Genet. 2011 Sep 9;89(3):432-7. doi: 10.1016/j.ajhg.2011.07.022. Epub 2011 Aug 11.
- Setta-Kaffetzi N, Simpson MA, Navarini AA, Patel VM, Lu HC, Allen MH, Duckworth M, Bachelez H, Burden AD, Choon SE, Griffiths CE, Kirby B, Kolios A, Seyger MM, Prins C, Smahi A, Trembath RC, Fraternali F, Smith CH, Barker JN, Capon F. AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. Am J Hum Genet. 2014 May 1;94(5):790-7. doi: 10.1016/j.ajhg.2014.04.005.
- Sugiura K, Takemoto A, Yamaguchi M, Takahashi H, Shoda Y, Mitsuma T, Tsuda K, Nishida E, Togawa Y, Nakajima K, Sakakibara A, Kawachi S, Shimizu M, Ito Y, Takeichi T, Kono M, Ogawa Y, Muro Y, Ishida-Yamamoto A, Sano S, Matsue H, Morita A, Mizutani H, Iizuka H, Muto M, Akiyama M. The majority of generalized pustular psoriasis without psoriasis vulgaris is caused by deficiency of interleukin-36 receptor antagonist. J Invest Dermatol. 2013 Nov;133(11):2514-2521. doi: 10.1038/jid.2013.230. Epub 2013 May 22.
- Sugiura K. The genetic background of generalized pustular psoriasis: IL36RN mutations and CARD14 gain-of-function variants. J Dermatol Sci. 2014 Jun;74(3):187-92. doi: 10.1016/j.jdermsci.2014.02.006. Epub 2014 Mar 5.
- Takeichi T, Kobayashi A, Ogawa E, Okuno Y, Kataoka S, Kono M, Sugiura K, Okuyama R, Akiyama M. Autosomal dominant familial generalized pustular psoriasis caused by a CARD14 mutation. Br J Dermatol. 2017 Oct;177(4):e133-e135. doi: 10.1111/bjd.15442. Epub 2017 Sep 10. No abstract available.
- Tauber M, Bal E, Pei XY, Madrange M, Khelil A, Sahel H, Zenati A, Makrelouf M, Boubridaa K, Chiali A, Smahi N, Otsmane F, Bouajar B, Marrakchi S, Turki H, Bourrat E, Viguier M, Hamel Y, Bachelez H, Smahi A. IL36RN Mutations Affect Protein Expression and Function: A Basis for Genotype-Phenotype Correlation in Pustular Diseases. J Invest Dermatol. 2016 Sep;136(9):1811-1819. doi: 10.1016/j.jid.2016.04.038. Epub 2016 May 21.
- Wang Y, Cheng R, Lu Z, Guo Y, Yan M, Liang J, Huang P, Li M, Yao Z. Clinical profiles of pediatric patients with GPP alone and with different IL36RN genotypes. J Dermatol Sci. 2017 Mar;85(3):235-240. doi: 10.1016/j.jdermsci.2016.11.008. Epub 2016 Dec 13.
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