Details of Disease

General Information of Disease (ID: DISTSNLR)

Disease Name	Generalized pustular psoriasis
Disease Class	EA90: Psoriasis
Definition	A rare and extreme form of psoriasis characterized by the appearance of sterile pustules which can take many patterns. All the main pathological features of the disease are accentuated. Generalized pustular psoriasis is clinically heterogeneous in its age at onset, precipitants, severity, and natural history. Many overlapping clinical entities are recognized. There is a relationship between these entities and plaque psoriasis, as some individuals may have episodes of plaque psoriasis preceding or following the generalized pustular psoriasis, but in others generalized pustular psoriasis occurs as the sole phenotype without plaque psoriasis at any time.
Disease Hierarchy	DISXOG13: Pustular psoriasis DISTSNLR: Generalized pustular psoriasis
ICD Code	ICD-11 ICD-11: EA90.40
Disease Identifiers	MONDO ID MONDO_0100491 UMLS CUI C0343055 MedGen ID 473074 Orphanet ID 247353 SNOMED CT ID 238612002

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Imsidolimab	DMC3FOW	Phase 3	Monoclonal antibody	[1]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
IL1RL2	TTUS18T	Strong	Genetic Variation	[2]
IL37	TTQTX98	Strong	Biomarker	[3]
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This Disease Is Related to 1 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
MMEL1	DEYCUQ2	Limited	Biomarker	[4]
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This Disease Is Related to 5 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
PIPOX	OTWTCOTN	Limited	Biomarker	[5]
PLXNB1	OTCA7JIT	Limited	Biomarker	[4]
AP1S3	OTPCMV24	Strong	Genetic Variation	[6]
IL17RA	OTVVI8ER	Strong	Biomarker	[7]
IL36RN	OT5CO95A	Strong	Genetic Variation	[8]
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References

1	ClinicalTrials.gov (NCT05366855) A Phase 3, Long-Term Extension Study to Evaluate the Safety and Efficacy of Imsidolimab (ANB019) in the Treatment of Adult Subjects With Generalized Pustular Psoriasis. U.S.National Institutes of Health.
2	Pharmacotherapeutic approaches for treating psoriasis in difficult-to-treat areas.Expert Opin Pharmacother. 2018 Apr;19(6):561-575. doi: 10.1080/14656566.2018.1448788. Epub 2018 Mar 22.
3	Exposure-Response Relationships for the Efficacy and Safety of Risankizumab in Japanese Subjects with Psoriasis.Clin Pharmacokinet. 2020 May;59(5):575-589. doi: 10.1007/s40262-019-00829-2.
4	Serum Procalcitonin and Presepsin Levels in Patients with Generalized Pustular Psoriasis.Dis Markers. 2018 Dec 16;2018:9758473. doi: 10.1155/2018/9758473. eCollection 2018.
5	Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background.Acta Derm Venereol. 1996 Jan;76(1):68-71. doi: 10.2340/00015555766871.
6	Generalized Pustular Psoriasis: Clinical Management and Update on Autoinflammatory Aspects.Am J Clin Dermatol. 2020 Apr;21(2):227-236. doi: 10.1007/s40257-019-00492-0.
7	Biologic therapy for acrodermatitis continua of Hallopeau: Successful treatment with secukinumab and review of the literature.Dermatol Ther. 2019 May;32(3):e12899. doi: 10.1111/dth.12899. Epub 2019 Apr 29.
8	Lack of association between mutation in IL36RN and palmoplantar pustular psoriasis in Chinese patients.An Bras Dermatol. 2019 Nov-Dec;94(6):658-663. doi: 10.1016/j.abd.2019.01.008. Epub 2019 Oct 26.