A Case of Generalized Pustular Psoriasis Associated with Turner Syndrome

Generalized pustular psoriasis (GPP) is a rare disease characterized by recurrent fever and systemic flushing accompanied by extensive sterile pustules. The committee of the guidelines was founded as a collaborative project between the Japanese Dermatological Association and the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labour, and Welfare Research Project on Overcoming Intractable Diseases. The aim of the guidelines was to provide current information to aid in the treatment of patients with GPP in Japan. Its contents include the diagnostic and severity classification criteria for GPP, its pathogenesis, and recommendations for the treatment of GPP. Since there are few clinical trial data with high levels of evidence for this rare disease, recommendations by the committee are described in the present guidelines.

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“…In addition, GPP occasionally develops as a complication of Turner syndrome. 23,24 Severity criteria for GPP ( Table 2)…”

Section: Clinical Statistics On Gpp and Reference Itemsmentioning

confidence: 99%

Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP

Fujita

Terada

Hayama

et al. 2018

The Journal of Dermatology

198

456

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“…There have been two previous reports of pustular psoriasis in TS patients in the English‐language literature 3,5 . Despite long‐lasting stability in her condition, our patient experienced significant exacerbation soon after the discontinuation of HRT for TS.…”

Section: Measurement Of Serum Cytokines and Chemokines And Comparisonmentioning

confidence: 62%

Pustular psoriasis in a patient with Turner syndrome: profile of serum cytokine levels

2012

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“…Cyclosporine has been efficacious for generalized pustular psoriasis in its juvenile form [53], in pregnancy [54], and in adults (grade D evidence) [55, 56]. It has a rapid onset of action.…”

Section: Resultsmentioning

confidence: 99%

A Delphi Consensus Approach to Challenging Case Scenarios in Moderate-to-Severe Psoriasis: Part 2

Strober

Cather

Cohen

et al. 2012

Dermatol Ther (Heidelb)

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IntroductionClinicians may be confronted with difficult-to-treat psoriasis cases for which there are scant data to rely upon for guidance. To assist in managing such patients, who are typically excluded from clinical trials, a consensus panel of 14 experts in the field of psoriasis was formed to conduct a Delphi method exercise.MethodsThe exercise consisted of both survey questionnaires and a live meeting to review and discuss current data (as of 2009, when the exercise was conducted) and arrive at a consensus for optimal treatment options. Seventy difficult treatment scenarios were identified, and the top 24 were selected for discussion at the live meeting.ResultsFive of the 24 discussed case scenarios are presented in this article: (1) moderate-to-severe psoriasis that has failed to respond to all currently approved therapies for psoriasis; (2) palmoplantar psoriasis that is unresponsive to topical therapy and phototherapy; (3) erythrodermic psoriasis; (4) pustular psoriasis; and (5) the preferred therapeutic choice to combine with low-dose methotrexate. A previous article (part 1) presented six other scenarios.ConclusionThe Delphi exercise resulted in guidelines for practicing physicians to utilize when confronted with patients with challenging cases of psoriasis.

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A Case of Generalized Pustular Psoriasis Associated with Turner Syndrome

Abstract: TS: Turner syndrome, GPP: generalized pustular psoriasis

Cited by 9 publications

References 6 publications

Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP

Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP

Pustular psoriasis in a patient with Turner syndrome: profile of serum cytokine levels

A Delphi Consensus Approach to Challenging Case Scenarios in Moderate-to-Severe Psoriasis: Part 2

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