Case Report: Successful Treatment of Acute Generalized Exanthematous Pustulosis With Secukinumab

Acute generalized exanthematous pustulosis is a severe, usually drug-related reaction, characterized by an acute onset of mainly small non-follicular pustules on an erythematous base. Most cases of acute generalized exanthematous pustulosis (AGEP) clear quickly with a systemic corticosteroid, but severe or recalcitrant cases may need other systemic therapies. In this case, a man in his 40 s with a history of psoriasis consulted a physician about widespread erythema, pustules, target lesions, and fever after the administration of a quadruple antituberculosis drug. Routine laboratory testing revealed elevated white blood cell count and C-reactive protein. The histopathology showed subcorneal pustules, spongiosis as well as lymphocyte and eosinophils infiltration in the dermis. The patient was diagnosed with definitive AGEP according to the diagnostic score from the EuroSCAR study. Cutaneous lesions especially pustules and erythema multiforme-like lesions on the upper arms and palms are crucial for distinguishing AGEP from Generalized pustular psoriasis. The patient was treated with secukinumab as a result of his failure to respond to topical corticosteroids and constrain of systemic steroids. Remission with secukinumab therapy was safe without increased risks of infections. This case indicates that secukinumab is a potential therapy that can rapidly improve the clinical symptoms of AGEP.

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A Case of Ceftriaxone-Induced Acute Generalized Exanthematous Pustulosis/Generalized Pustular Psoriasis Overlap

Case Reports in Dermatology ◽

10.1159/000488076 ◽

2018 ◽

Vol 10 (1) ◽

pp. 69-75 ◽

Cited By ~ 2

Author(s):

Jianfeng Zheng ◽

Yunlu Gao ◽

Xuemei Yi ◽

Yangfeng Ding

Keyword(s):

Pustular Psoriasis ◽

Whole Body ◽

Complete Regression ◽

Clinical Form ◽

Acute Generalized Exanthematous Pustulosis ◽

Plaque Type ◽

Generalized Pustular Psoriasis ◽

History Of ◽

Exanthematous Pustulosis ◽

Missense Substitution

Acute generalized exanthematous pustulosis, characterized by subcorneal or superficial intraepidermal pustules, is induced by drugs in more than 90% of cases. Psoriasis is an autoimmune disease triggered by different conditions in genetically susceptible people. Generalized pustular psoriasis is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this report the authors have reported a 40-year-old male patient with primary syphilis who developed generalized pustular dermatosis after the use of ceftriaxone. On the third day after ceftriaxone treatment, complete regression of the syphilis lesions was reached. While on the sixth day, erythematous pustular lesions accompanied with fever were observed on the whole body. A personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. After discontinuation of ceftriaxone, the patient’s condition slowly improved until he had plaque-type psoriasis 3 weeks later. A heterozygous c.115 + 6T>C missense substitution of IL36RN related to the pathogenesis of acute generalized exanthematous pustulosis/generalized pustular psoriasis was identified.

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Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease): A Rare Case

International Journal of PharmTech Research ◽

10.20902/ijptr.2019.130401 ◽

2020 ◽

Vol 13 (4) ◽

pp. 309-315

Author(s):

Rina Gustia ◽

Yosse Rizal ◽

Dwi Sepfourteen

Keyword(s):

Skin Lesion ◽

Physical Examination ◽

Rare Case ◽

Disease Patient ◽

Acute Generalized Exanthematous Pustulosis ◽

Generalized Pustular Psoriasis ◽

Subcorneal Pustular Dermatosis ◽

History Of ◽

Exanthematous Pustulosis ◽

Histopathology Examination

Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent, pustular eruption. It may be difficult to differentiate SPD from Acute Generalized Exanthematous Pustulosis (AGEP) and Generalized Pustular Psoriasis (GPP)just from clinical appeareance. A 48 years old woman with subcorneal pustular dermatosis was reported. She was complained there were tiny blisters containing pus on the reddish patches that felt burning and became widespread to neck, chest, abdomen, arms, groin and legs since 4 days ago. Patient had no history of skin lesion before. From physical examination revealed no other pathologies and on the skin showed erythematous patches with annular lesion, pustules formation that tended to coalesce to form desquamative plaques, erosions, on chest, abdomen, back, arm, buttock and leg. Based on histopathology showed there are subcorneal pustules. The subcorneal pustule is filled with neutrophils and on the dermis, there are mild infiltration of neutrophil cells. This feature revealed to SPD.Clinically it may be difficult to differentiate SPD from AGEP and GPP. Histopathology examination can distinguished these disease,patient was treated with prednisone 40 mg and dapson 100 mg and had improvement after 4 weeks.

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Faculty Opinions recommendation of The histopathological spectrum of acute generalized exanthematous pustulosis (AGEP) and its differentiation from generalized pustular psoriasis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.5161958.5100058 ◽

2010 ◽

Author(s):

Bruce Smoller ◽

Cornelia Müller

Keyword(s):

Pustular Psoriasis ◽

Acute Generalized Exanthematous Pustulosis ◽

Generalized Pustular Psoriasis ◽

Exanthematous Pustulosis

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Acute generalized exanthematous pustulosis (AGEP). Case report

Revista do Instituto de Medicina Tropical de São Paulo ◽

10.1590/s0036-46652005000300011 ◽

2005 ◽

Vol 47 (3) ◽

pp. 171-176 ◽

Cited By ~ 15

Author(s):

Walter Belda Junior ◽

Ana Carolina Junqueira Ferolla

Keyword(s):

Antidepressant Drugs ◽

Corticosteroid Treatment ◽

Beta Lactam ◽

Drug Induced ◽

Acute Generalized Exanthematous Pustulosis ◽

Outpatient Unit ◽

Similar Drug ◽

Generalized Pustular Psoriasis ◽

Inflammatory Drugs ◽

Exanthematous Pustulosis

Acute Generalized Exanthematous Pustulosis (AGEP) is a drug-induced dermatosis characterized by an acute episode of sterile pustules over erythematous-edematous skin. It is accompanied by an episode of fever, which regresses a few days after discontinuation of the drug that caused the condition or as a result of corticosteroid treatment. The main triggering drugs are antibiotics, mainly beta-lactam ones. Other medications, such as antifungal agents, non steroid anti-inflammatory drugs, analgesics, antiarrhythmic, anticonvulsant and antidepressant drugs, may also be responsible. Histologically, it is characterized by the existence of vasculitis, associated with non-follicular subcorneal pustules. A case of a Caucasian female outpatient unit of Dermatology with AGEP, who presented with generalized pustulosis lesions after the use of cephalosporin for urinary infection is related. The diagnosis was confirmed by the clinical and pathological correlations, the resolution of the dermatosis after discontinuation of the drug and use of systemic corticosteroid treatment, and the recurrence of the disorder after the introduction of a similar drug. The importance of the recognition of this drug-induced dermatosis is given by its main differential clinical and histological diagnoses: generalized pustular psoriasis and subcorneal pustulosis.

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Chondrosternal Arthritis in Infant: An Unusual Entity

Case Reports in Orthopedics ◽

10.1155/2014/652539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4

Author(s):

Athina Nikolarakou ◽

Dana Dumitriu ◽

Pierre-Louis Docquier

Keyword(s):

Clinical Symptoms ◽

Therapeutic Option ◽

White Blood Cells ◽

Laboratory Data ◽

C Reactive Protein ◽

Reactive Protein ◽

History Of ◽

Local Tenderness ◽

Wait And See

Primary arthritis of chondrosternal joint is very rare and occurs in infants less than 18 months of age. Presentation is most often subacute but may be acute. Child presents with a parasternal mass with history of fever and/or local signs of infection. Clinical symptoms vary from a painless noninflammatory to a painful mass with local tenderness and swelling, while fever may be absent. Laboratory data show low or marginally raised levels of white blood cells and C-reactive protein, reflecting, respectively, the subacute or acute character of the infection. It is a self-limiting affection due to the adequate immune response of the patient. Evolution is generally good without antibiotherapy with a progressive spontaneous healing. A wait-and-see approach with close follow-up in the first weeks is the best therapeutic option.

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Acute generalized exanthematous pustulosis and generalized pustular psoriasis: Are they distinct pustular disorders?

Journal of Dermatological Science ◽

10.1016/j.jdermsci.2016.08.048 ◽

2016 ◽

Vol 84 (1) ◽

pp. e13

Author(s):

Hyo Sang Song ◽

Sang Jin Kim ◽

Yong Hyun Jang ◽

Eun-So Lee

Keyword(s):

Pustular Psoriasis ◽

Acute Generalized Exanthematous Pustulosis ◽

Generalized Pustular Psoriasis ◽

Exanthematous Pustulosis

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Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis

Medicina ◽

10.3390/medicina57101004 ◽

2021 ◽

Vol 57 (10) ◽

pp. 1004

Author(s):

Morgan Sussman ◽

Anthony Napodano ◽

Simo Huang ◽

Abhirup Are ◽

Sylvia Hsu ◽

...

Keyword(s):

Adverse Reaction ◽

Clinical Course ◽

Pustular Psoriasis ◽

Topical Steroids ◽

Inflammatory Disorder ◽

Acute Generalized Exanthematous Pustulosis ◽

Cutaneous Adverse Reaction ◽

History Of ◽

Exanthematous Pustulosis ◽

Crucial Part

The similarity between pustular psoriasis (PP) and acute generalized exanthematous pustulosis (AGEP) poses problems in the diagnosis and treatment of these two conditions. Significant clinical and histopathologic overlap exists between PP and AGEP. PP is an inflammatory disorder that has numerous clinical subtypes, but all with sterile pustules composed of neutrophils. AGEP is a severe cutaneous adverse reaction that is also characterized by non-follicular sterile pustules. Clinical features that suggest a diagnosis of PP over AGEP include a history of psoriasis and the presence of scaling plaques. Histologically, eosinophilic spongiosis, vacuolar interface dermatitis, and dermal eosinophilia favor a diagnosis of AGEP over PP. Importantly, PP and AGEP vary in clinical course and treatment. PP treatment involves topical steroids, oral retinoids, and systemic immunosuppressants. Newer therapies targeting IL-36, IL-23, IL-1, and PDE-4 have been investigated. The removal of the offending agent is a crucial part of the treatment of AGEP.

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