| H01663 | |
| H number | H01663 |
| Name | Pustular psoriasis |
| Description | Pustular psoriasis is a form of psoriasis [DS:H01656] characterized by multiple tender sterile pustules with an underlying, blotchy, erythematous base. It has been classified into localized and generalized forms. Generalized pustular psoriasis (GPP) is the development of extensive sterile pustules with widespread erythema. The von Zumbusch type often starts abruptly and can be associated with painful skin, fever, and chills. The disease course varies from a benign, chronic process to an acute life-threatening episode, and as such optimal treatment depends on severity. Diseases considered within the spectrum of GPP include: impetigo herpetiformis and childhood GPP. Acrodermatitis continua of Hallopeau and palmoplantar pustulosis are two distinct forms of localized pustular psoriasis. Treatment should be governed by the extent of involvement and severity of disease. Acitretin, cyclosporine, methotrexate, and infliximab are considered to be first-line therapies for those with GPP. Adalimumab, etanercept, and psoralen plus ultraviolet A are second-line modalities in this setting. Pustular psoriasis in children, in pregnant women, and in localized forms alter which agents are first-line modalities as concerns such as teratogenicity need to be factored into the decisionmaking for the individual patient. |
| Category | Immune system disease; Skin disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Spesolimab [DR:D12066] |
| Comment | - |
| Other DBs | ICD-11: EA90.4 ICD-10: L40.1 L40.3 MeSH: D011565 |
| Reference | PMID:22609220 AUTHORS Robinson A, Van Voorhees AS, Hsu S, Korman NJ, Lebwohl MG, Bebo BF Jr, Kalb RE TITLE Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. JOURNAL J Am Acad Dermatol 67:279-88 (2012) DOI:10.1016/j.jaad.2011.01.032 PMID:24434359 AUTHORS Raychaudhuri SK, Maverakis E, Raychaudhuri SP TITLE Diagnosis and classification of psoriasis. JOURNAL Autoimmun Rev 13:490-5 (2014) DOI:10.1016/j.autrev.2014.01.008 |