Pustular Psoriasis


Article Author:
Muneeb Shah
Daifallah Al Aboud
Jonathan Crane


Article Editor:
Sandeep Kumar


Editors In Chief:
Ahmad Al Aboud
Jayakar Thomas
Pramod Nigam


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
7/5/2019 2:04:57 PM

Introduction

Pustular psoriasis is a rare, immune-mediated systemic skin disorder characterized by yellowish pustules on an erythematous base with a variety of clinical presentations and distribution patterns.[1] Pustular psoriasis is considered a variant of psoriasis vulgaris. The pustules can be widespread or localized and are characterized by a sterile, predominantly neutrophilic infiltrate. Unlike chronic plaque psoriasis (the most common variant of psoriasis vulgaris), lesions of pustular psoriasis are often tender to palpation.[2] Pustular psoriasis can be further subdivided based on the clinical presentation and the location of pustules. 

Subtypes of pustular psoriasis include:

  • Generalized:
    • von Zumbusch subtype: Diffuse generalized pustular eruption with associated systemic symptoms (fevers, arthralgias, etc.)
    • Annular subtype: Annular lesions with pustules along the advancing edge.
    • Exanthematic subtype: Acute pustular eruption that disappears after a few days without systemic symptoms. 
    • Impetigo herpetiformis: Pustular psoriasis occurring during pregnancy.
  • Localized:
    • Acrodermatitis continua of Hallopeau: Pustules affecting the fingers, toes, and nail beds. 
    • Palmoplantar psoriasis: Pustules affecting the palms and soles

Etiology

Pustular psoriasis occurs in genetically susceptible individuals and can be triggered by certain risk factors leading to neutrophil chemotaxis and accumulation in the epidermis.[3] Patients with pustular psoriasis have an increased frequency of the HLA-B27 allele compared to the general population. Most cases of pustular psoriasis are idiopathic, however, risk factors that may have a role in the etiology of pustular psoriasis include [4][5][4]:

  1. Sudden withdrawal of systemic steroids
  2. Skin infections (Staphylococcus aureus)
  3. Electrolytes imbalance; hypocalcemia (von Zumbusch subtype)
  4. Medications (lithium, iodine, penicillin, interferon-alpha, etc.)
  5. Pregnancy (impetigo herpetiformis)
  6. Phototherapy
  7. Vaccinations (BCG and H1N1)

Epidemiology

Pustular psoriasis is a rare disease. It usually affects both genders equally and has no sexual predilection. It has a bimodal presentation; affecting adults and children. In the adult age group, the disorder appears between ages 40 to 50, whereas in the pediatric age group, it tends to present in infancy. Asians tend to be more affected than Caucasians.[6] The pustular variants of psoriasis represent approximately 1% of all clinical cases of psoriasis vulgaris.[5]

Pathophysiology

The exact pathogenesis of pustular psoriasis has not been fully elucidated. However, based on the expression of certain cytokines and responses to specific medications, several mechanisms have been proposed. A combination of genetic susceptibility and exposure to certain inciting factors (e.g., withdrawal of systemic corticosteroids) lead to an upregulation of specific cytokines and accumulation of neutrophils in the epidermis.[5] Additionally, dermal dendritic cells release elastase, which may play a role in the formation of pustules.[7] Both pustular psoriasis and chronic plaque psoriasis exhibit overexpression of IL-1, IL-17, IL-23, IL-36, TNF-alpha, and IFN-gamma. However, the expression of IL-1 and IL-36 are more prominent in pustular psoriasis. Studies have also identified an IL-36 receptor antagonist deficiency in patients with pustular psoriasis and patients with pustular psoriasis have also been successfully treated with a novel monoclonal antibody against the IL-36 receptor.[8] Therefore, IL-36 likely plays a critical role in the pathogenesis of pustular psoriasis. 

Histopathology

The histopathological features of pustular psoriasis mimic that for classical psoriasis vulgaris in having retention of the nuclei in the stratum corneum (parakeratosis), a thick stratum spinosum (hyperkeratosis), elongation of rete ridges, reduced stratum granulosum, and thinning of the suprapapillary epidermis.[5] In addition to the classic histologic features of psoriasis vulgaris, there is also a prominent neutrophilic infiltrate in the papillary dermis and epidermis, causing basal keratinocytes to herniate into the papillary dermis disrupting desmosomal junctions of keratinocytes (spongiosis), and leading to the development of superficial micro-abscesses.[9] Neutrophilic infiltration of the epidermis is more prominent in pustular psoriasis than other variants of psoriasis vulgaris. 

History and Physical

Pustular psoriasis appears as numerous discrete and confluent superficial, yellowish pustules on a background of erythema. Pustular psoriasis can be diffuse or localized. A full body skin examination should be performed, including careful evaluation of the mucous membranes and the nails for signs of psoriasis and to exclude other causes of pustulosis. 

Pustular psoriasis starts as red papules or plaques that quickly evolves into yellowish, superficial pustules on a background of erythema. The generalized von Zumbusch subtype usually presents with systemic symptoms like fever, joint pain, headaches, and leukocytosis. In contrast, the exanthematic subtype presents as an acute pustular eruption without systemic symptoms. The annular subtype is seen more frequently in children as annular lesions with pustules along the advancing edge.[10] Acrodermatitis continua of Hallopeau affects the finger, toes, and nailbeds. Palmoplantar psoriasis, which can be seen as part of SAPHO syndrome, affects the palms and soles.

The clinician should inquire about the trigger factors in patients who present with pustular psoriasis, especially the use of medications like corticosteroids. Family history is important to ascertain since there is a genetic element responsible for the disease.

Evaluation

Patients with suspected pustular psoriasis require careful evaluation, as the von Zumbusch subtype can be life-threatening. Laboratory investigations should include a complete blood count (CBC) to evaluate for leukocytosis, an electrolyte panel to assess for hypocalcemia, and a liver panel to assess for aspartate transaminase, alanine transaminase, and levels of albumin. A pregnancy test should be ordered in women of childbearing age. A punch biopsy can be performed in all equivocal cases. 

Treatment / Management

The first step in managing cases of pustular psoriasis is the identification of the triggering factors. Generalized pustular psoriasis (e.g., von Zumbusch subtype), require admission to the hospital. Systemic symptoms including fever and joint pain will require treatment with antipyretics and anti-inflammatory medications. Disease-specific medications include systemic retinoids (acitretin, isotretinoin), methotrexate, cyclosporine, and infliximab. In the pediatric age group, acitretin, cyclosporine, methotrexate, and etanercept are considered the first line options.[11]

Second line options include systemic treatments like etanercept and adalimumab or topical treatments like corticosteroids, calcipotriene, and tacrolimus. All of these options can be monotherapy choices or in combination with the first line options.[12] Phototherapy is another treatment option.[13] Early delivery is recommended in pregnant patients with impetigo herpetiformis.

Recent reports indicate the successful use of L-1 receptor antagonists (e.g., anakinra) and IL-36 receptor antagonists in treating pustular psoriasis.[8] Tocilizumab, a monoclonal antibody against the IL-6 receptor, has also shown efficacy in some recalcitrant cases of pustular psoriasis.[14] 

Differential Diagnosis

  • Acute generalized exanthematous pustulosis
  • Drug eruption reaction
  • Pemphigus vulgaris
  • Pemphigus foliaceous
  • Impetigo
  • Dermatitis Herpetiformis
  • Infected eczema
  • Erythroderma
  • Acute cutaneous lupus
  • Dyshidrotic eczema
  • Disseminated herpes simplex virus

Prognosis

Pustular psoriasis is a chronic disease marked by recurrent episodes or flares. Treatment is aimed to shorten the duration of relapse, prevent complications, and extend disease-free periods. Compared to other subtypes, the von Zumbusch subtype of pustular psoriasis has a higher risk of mortality because of systemic involvement, especially with underlying comorbidities, such as cancer, renal failure, or liver failure. The localized subtypes of pustular psoriasis have a much lower risk of mortality. 

Complications

  • Hypocalcemia
  • Septicemia, especially in the generalized form
  • Hyperthermia
  • Liver damage
  • Acute renal failure
  • Malnutrition

Deterrence and Patient Education

Patients with psoriasis should be educated to avoid abrupt steroid withdrawal, which can trigger episodes of pustular psoriasis. Flaccid lesions should be treated with proper wound care to avoid complications and secondary infections. 

Enhancing Healthcare Team Outcomes

Management of pustular psoriasis requires an interprofessional team approach, including providers from primary care, dermatology, and infectious disease. A detailed history and complete physical examination are critical in diagnosing cases of pustular psoriasis. A dermatologist can help in the initial diagnostic workup and to assess for complications. Depending on the surface area involved, admission to the hospital may be required (e.g., von Zumbusch subtype). In equivocal cases, a biopsy can be performed, as the differential diagnosis for pustulosis is rather vast. Treatment is targeted to prevent relapses and shorten the duration of flares. 


  • Image 7226 Not availableImage 7226 Not available
    Contributed by Daifallah Al Aboud, MD
Attributed To: Contributed by Daifallah Al Aboud, MD

Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Pustular Psoriasis - Questions

Take a quiz of the questions on this article.

Take Quiz
A previously healthy 41-year-old man comes to the physician for evaluation of a new rash. He has a family history of pernicious anemia and psoriasis vulgaris. Vital signs are significant for fever and tachycardia. Physical examination reveals multiple pustules on an erythematous base scattered over his trunk, arms, and legs with an associated silvery scale. Laboratory studies a neutrophilic predominant leukocytosis and an elevated erythrocyte sedimentation rate. Which of the following is most likely to be found on histopathological examination?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A previously healthy 48-year-old man comes to the clinic for evaluation of a new rash. He has a family history of psoriasis. Vital signs are significant for fever and tachycardia. Physical examination reveals multiple pustules on an erythematous base scattered over his trunk, arms, and legs with associated scaling. Laboratory studies a neutrophilic predominant leukocytosis and an elevated erythrocyte sedimentation rate. Which of the following is the most important risk factor for this disease?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 22-year-old pregnant woman comes to the physician for evaluation of a new rash. She reports that the rash began suddenly last night. She is 37 weeks pregnant. Vital signs are within normal limits. Physical examination reveals multiple yellowish pustules on an erythematous base scattered over the extremities and torso. There is also a silver scale over the extensor surfaces of both elbows. Which of the following is the next best step in management?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A previously healthy 52-year-old man comes to the physician for evaluation of a new rash. He has a family history of psoriasis. Vital signs are significant for fever and tachycardia. Physical examination reveals multiple pustules on an erythematous base scattered over his trunk, arms, and legs with associated scale. Laboratory studies a neutrophilic predominant leukocytosis and an elevated erythrocyte sedimentation rate. Which of the following cytokinesis most likely implicated in this disease?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 24-year-old pregnant woman comes to the physician for evaluation of a new rash. She reports that the rash began suddenly last night. She is 38 weeks pregnant. Vital signs are within normal limits. Physical examination reveals multiple yellowish pustules on an erythematous base scattered over the extremities and torso. There is also a silver scale over the extensor surfaces of both elbows. Which of the following is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Pustular Psoriasis - References

References

Tsuchida Y,Hayashi R,Ansai O,Nakajima M,Oginezawa M,Kawai T,Yokoyama R,Deguchi T,Hama N,Shinkuma S,Abe R, Generalized pustular psoriasis complicated with bullous pemphigoid. International journal of dermatology. 2018 Dec 5     [PubMed]
Negrotto L,Correale J, Palmar pustular psoriasis associated with teriflunomide treatment. Multiple sclerosis and related disorders. 2018 Nov 19     [PubMed]
Epple A,Paffhausen JE,Fink C,Enk A,Sedlaczek O,Haenssle HA, Chronic recurrent multifocal osteomyelitis with psoriatic skin manifestations in a 12-year-old female. Dermatology practical     [PubMed]
Madanagobalane S, Secukinumab in Generalized Pustular Psoriasis. Indian dermatology online journal. 2018 Nov-Dec     [PubMed]
Komatsuda S,Kamata M,Chijiwa C,Namiki K,Fukaya S,Hayashi K,Fukuyasu A,Tanaka T,Ishikawa T,Ohnishi T,Abe K,Yamamoto T,Aozasa N,Sugiura K,Tada Y, Gastrointestinal bleeding with severe mucosal involvement in a patient with generalized pustular psoriasis without IL36RN mutation. The Journal of dermatology. 2018 Nov 26     [PubMed]
Zhang Z,Xu JH, Investigation of Psoriasis Susceptibility Loci in Psoriatic Arthritis and a Generalized Pustular Psoriasis Cohort. The journal of investigative dermatology. Symposium proceedings. 2018 Dec     [PubMed]
Gabeff R,Safar R,Leducq S,Maruani A,Sarrabay G,Touitou I,Samimi M, Successful therapy with secukinumab in a patient with generalized pustular psoriasis carrying homozygous IL36RN p.His32Arg mutation. International journal of dermatology. 2018 Nov 14     [PubMed]
Su Z,Paulsboe S,Wetter J,Salte K,Kannan A,Mathew S,Horowitz A,Gerstein C,Namovic M,Todorović V,Seagal J,Edelmayer RM,Viner M,Rinaldi L,Zhou L,Leys L,Huang S,Wang L,Sadhukhan R,Honore P,McGaraughty S,Scott VE, IL-36 receptor antagonistic antibodies inhibit inflammatory responses in preclinical models of psoriasiform dermatitis. Experimental dermatology. 2018 Nov 11     [PubMed]
Meier-Schiesser B,Feldmeyer L,Jankovic D,Mellett M,Satoh TK,Yerly D,Navarini A,Abe R,Yawalkar N,Chung WH,French LE,Contassot E, Culprit drugs induce specific IL-36 overexpression in Acute Generalized Exanthematous Pustulosis. The Journal of investigative dermatology. 2018 Nov 2     [PubMed]
Li ZT,Wang S, [Genetic Polymorphism of IL36RN in Han Patients with Generalized Pustular Psoriasis Alone in Sichuan Region]. Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition. 2018 Jul     [PubMed]
Okubo Y,Mabuchi T,Iwatsuki K,Elmaraghy H,Torisu-Itakura H,Morisaki Y,Nakajo K, Long-term efficacy and safety of ixekizumab in Japanese patients with erythrodermic or generalized pustular psoriasis: subgroup analyses of an open-label, phase 3 study (UNCOVER-J). Journal of the European Academy of Dermatology and Venereology : JEADV. 2018 Oct 14     [PubMed]
Cro S,Smith C,Wilson R,Cornelius V, Treatment of pustular psoriasis with anakinra: a statistical analysis plan for stage 1 of an adaptive two-staged randomised placebo-controlled trial. Trials. 2018 Oct 3     [PubMed]
Boehner A,Navarini AA,Eyerich K, Generalized pustular psoriasis - a model disease for specific targeted immunotherapy, systematic review. Experimental dermatology. 2018 Oct     [PubMed]
Bachelez H,Choon SE,Marrakchi S,Burden AD,Tsai TF,Morita A,Turki H,Hall DB,Shear M,Baum P,Padula SJ,Thoma C, Inhibition of the Interleukin-36 Pathway for the Treatment of Generalized Pustular Psoriasis. The New England journal of medicine. 2019 Mar 7     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Dermatology-Pediatric. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Dermatology-Pediatric, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Dermatology-Pediatric, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Dermatology-Pediatric. When it is time for the Dermatology-Pediatric board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Dermatology-Pediatric.