Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis

Sussman, Morgan; Napodano, Anthony; Huang, Simo; Are, Abhirup; Hsu, Sylvia; Motaparthi, Kiran

doi:10.3390/medicina57101004

Cited by 16 publications

(13 citation statements)

References 44 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The diagnosis is supported by histopathological findings such as vacuolar interface dermatitis, eosinophilia, and keratinocyte apoptosis. [22][23] Fever is a common symptom of GPP, with 54.7% of patients developing a fever during the acute stage in our study, and 25.0%-68.6% in other studies [11][12]14,17 .…”

Section: Discussionsupporting

confidence: 44%

See 1 more Smart Citation

Characterization of Generalized Pustular Psoriasis in Northwest China: A Single-Center Retrospective Study

Li¹,

Peng²,

Geng³

2022

International Journal of Dermatology and Venereology

View full text Add to dashboard Cite

Objective: This study was performed to investigate the clinical characteristics of patients with generalized pustular psoriasis (GPP) in Northwest China. Methods: The clinical data of patients with GPP were retrospectively collected in the Second Affiliated Hospital of Xi’an Jiaotong University from January 1, 2017, to December 31, 2021 and analyzed using the chi-square test and Mann-Whitney U test. Results: In total, 179 hospitalized patients were included. The male:female ratio was 1.16:1.00, and the mean age at onset was 35.05 ± 19.11 years. Psoriasis vulgaris was also present in 76.0% of patients, and a family history of psoriasis was present in 15.6%. The mean duration of hospitalization was 10.67 ± 4.31 days, and the mean duration of flares was 29.58 ± 24.32 days. Infections and suspected drugs were predisposing factors. A fever developed in 54.7% of patients, and pruritus developed in 70.9%. Some patients had involvement of the nails (38.0%), scalp (46.9%), and tongue (4.5%). Cardiovascular disease, hypertension, and gallbladder-related disease were common comorbidities. The efficacy of acitretin was 84.7%, that of methotrexate was 66.7%, and that of cyclosporine was 100%. Fifteen patients were treated with secukinumab or adalimumab and responded well. The mean response time was 6.34 ± 2.91 days based on the combination treatments. The mean duration of the treatment regimen was 111.35 ± 94.25 days, and approximately 46.6% (n = 131) of patients developed recurrence. Conclusion: Our retrospective study showed that most cases of GPP were accompanied by psoriasis vulgaris and associated with fever or pruritus. Acitretin had good therapeutic efficacy, but recurrence should be noted. Biologics are increasingly becoming effective treatments, but their superiority and safety need further research.

show abstract

Section: Discussionsupporting

confidence: 44%

“…The diagnosis is supported by histopathological findings such as vacuolar interface dermatitis, eosinophilia, and keratinocyte apoptosis. 22-23…”

Section: Discussionmentioning

confidence: 99%

Characterization of Generalized Pustular Psoriasis in Northwest China: A Single-Center Retrospective Study

Li¹,

Peng²,

Geng³

2022

International Journal of Dermatology and Venereology

View full text Add to dashboard Cite

show abstract

“…The unusual nature of GPP can make diagnosis problematic, and the existence of numerous different pustular diseases further complicates it. For example, the similarity between GPP and AGEP, a severe cutaneous drug reaction, poses diagnostic and therapeutic problems [ 3 – 7 , 49 , 50 ].…”

Section: Discussionmentioning

confidence: 99%

Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Díaz

Daudén

Carrascosa

et al. 2023

Dermatol Ther (Heidelb)

View full text Add to dashboard Cite

Generalized pustular psoriasis (GPP) is a rare, chronic, and severe inflammatory skin disorder characterized by sudden eruption of sterile pustules, often accompanied by systemic inflammation. GPP flares can be life-threatening if untreated, owing to potential serious complications such as sepsis and cardiovascular failure. Diagnosis and clinical measurement of disease severity in GPP are often difficult. Lack of standardized criteria in the international guidelines and the heterogeneity of cutaneous and extracutaneous symptoms make the diagnosis of GPP difficult. Clinical criteria for description and diagnosis of pustular conditions, including GPP, are variable and there is no specific agreement on commonly sustained concepts. Differentiation of GPP from other similar conditions/diseases is important and requires careful assessments. The evidence that supports current topical or systemic therapies is largely based on case reports and small studies. Some biologic agents that target key cytokines involved in the activation of inflammatory pathways have been used as treatments for GPP. Recently, spesolimab, an IL-36R antagonist, has been approved in the USA and Japan for the treatment of GPP flares in adults, but there are no currently approved treatments for GPP in Europe. The IL-36 pathway has recently emerged as a central axis driving the pathogenic inflammatory mechanisms of GPP. Biologic agents that inhibit the IL-36 pathway have shown efficacy and safety in patients with GPP, addressing a generally considered unmet medical need.

show abstract

“…Although GPP may show tortuous dilated vessels and psoriasiform hyperplasia, neither of which were observed in our patient’s histopathological image. 7 , 8 The absence of epidermal sloughing and negative Nikolsky sign differentiates it from Toxic epidermal necrolysis(TEN). 9 The EuroSCAR study group scoring system for AGEP 10 and the Naranjo Scale for the Adverse Drug Reaction(ADR) 11 probability score resulted in the definite diagnosis of drug-related AGEP.…”

Section: Discussionmentioning

confidence: 99%

Terbinafine Induced Acute Generalized Exanthematous Pustulosis Treated with Adalimumab: Recalcitrant to Systemic Corticosteroid Therapy

Deng

Ali

et al. 2023

CCID

View full text Add to dashboard Cite

Acute generalized exanthematous pustulosis (AGEP) is generally caused by drugs and is characterized by the rapid development of numerous non-follicular sterile pustules on an erythematous base, fever, and neutrophilia. We report an association between terbinafine, with AGEP, and adalimumab treatment. A 24-year-old teenage female patient with a history of onychomycosis was treated with terbinafine. On the second day of the first dose, multiple edematous and erythematous lesions appear with pinhead pustules. Neutrophilia was observed in the blood report. The clinical history, lesions, and laboratory evaluations were consistent with AGEP. We discontinued the terbinafine therapy, and the systemic corticosteroid was initiated; however, the patient’s condition worsened. Adalimumab subcutaneously was initiated, and the symptoms cleared up in weeks. The European Study of Severe Cutaneous Adverse Reactions (EuroSCAR) scoring system and Naranjo’s algorithm scale were used to check the possibility of a drug-induced adverse reaction. The Association of AGEP with the terbinafine drug is not rare. However, there are no reports or literature of drug-related rash or exanthematous eruptions unresponsive to corticosteroids and treated with adalimumab.

show abstract

Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis

Cited by 16 publications

References 44 publications

Characterization of Generalized Pustular Psoriasis in Northwest China: A Single-Center Retrospective Study

Characterization of Generalized Pustular Psoriasis in Northwest China: A Single-Center Retrospective Study

Generalized Pustular Psoriasis: A Review on Clinical Characteristics, Diagnosis, and Treatment

Terbinafine Induced Acute Generalized Exanthematous Pustulosis Treated with Adalimumab: Recalcitrant to Systemic Corticosteroid Therapy

Contact Info

Product

Resources

About