Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
Images in Dermatology
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Media and news
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Observation Letter
88 (
6
); 817-819
doi:
10.25259/IJDVL_1044_2021
pmid:
35962496

A diagnostic challenge: Pathergy positivity in papulopustular lesions of Behçet disease mimicking acute localised exanthematous pustulosis

Cihanbeyli Public Hospital, Dermatology and Venereology Clinic, Cihanbeyli, Konya, Turkey
Department of Dermatology, Hacettepe University, Ankara, Turkey
Department of Pathology, Hacettepe University, Ankara, Turkey
Division of Rheumatology, Department of Internal Diseases, Hacettepe University, Ankara, Turkey

Corresponding author: Dr. Ecem Bostan, Cihanbeyli Public Hospital, Dermatology and Venereology Clinic, Cihanbeyli, Konya, Turkey. bostanecem@gmail.com

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Bostan E, Gulseren D, Akdogan N, Ozdemir DA, Karadag O. A diagnostic challenge: Pathergy positivity in papulopustular lesions of Behçet disease mimicking acute localised exanthematous pustulosis. Indian J Dermatol Venereol Leprol 2022;88:817-19.

Sir,

Behçet disease is characterised by uveitis, recurrent oral/genital aphthae, erythema nodosum, papulopustular skin lesions, pathergy positivity besides neurologic, gastrointestinal and cardiovascular manifestations.1 Superficial folliculitis, pityrosporum folliculitis, eosinophilic pustular folliculitis, pustular psoriasis, subcorneal pustular dermatosis and acute localised exanthematous pustulosis are some other cutaneous diseases which present with pustular eruption.1 We present a case of pathergy positivity mimicking acute localised exanthematous pustulosis in a patient diagnosed with Behçet disease.

A 38-year-old woman was referred to the dermatology clinic for a pustular eruption on her left inner thigh which started one week ago. She had recurrent attacks of folliculitis involving both lower and upper extremities in the past 16 years. Her current lesions appeared after an epilation session as a few grouped pustules on her left inner thigh. She was started on oral ampicillin-sulbactam with a provisional diagnosis of folliculitis four days ago. However, the pustular eruption continued to progress despite the oral antibiotic treatment. There was no history of any other drug intake which could have provoked the development of papulopustular lesions. She reported having recurrent oral aphthae over three times within a year. She did not have any history of genital ulceration, pain or swelling in the joints, abdominal/chest pain or dyspnoea. There was no history suggestive of uveitis, erythema nodosum, photosensitivity, malar rash, alopecia, deep vein thrombosis, periodic fever, neurologic, gastrointestinal, genitourinary and cardiovascular symptoms which could be associated with Behçet disease or other rheumatologic disorders. Ophthalmologic examination was normal. Neurologic, gastrointestinal and cardiovascular examination findings did not reveal any abnormalities. The family history was unremarkable. Dermatological examination revealed a crusted plaque surrounded by discrete and confluent non-follicular pustules on erythematous base on medial aspect of left thigh [Figure 1a]. Our provisional diagnoses included acute localised exanthematous pustulosis, pathergy positivity of Behçet disease, pustular pyoderma gangrenosum, and infective folliculitis. Fungal and aerobic cultures taken from the pustules were negative. Anti-nuclear antibody, rheumatoid factor, anti-double-stranded DNA antibody, anti-neutrophilic cytoplasmic antibodies, complement C3, complement C and extractable nuclear antibodies were negative. A pathergy test was done and a biopsy was taken from the pustular eruption. Pathergy test was positive [Figure 1b]. One week after the initiation of treatment with systemic and topical corticosteroid, regression of pustules leaving desquamation and pale erythematous patches were noted [Figure 1c]. Skin biopsy revealed subcorneal/intraepidermal spongiform pustule formation expanding into the dermis [Figure 2]. According to the international criteria for Behçet disease point score system, a patient having 4 points or above was classified as having Behçet disease.2 In this point score system, ocular lesions, oral aphthae and genital aphthae are each assigned two points, while skin manifestations, central nervous system involvement and vascular manifestations are given one point each.2 The pathergy test, when positive, is given one point.2 Our patient had recurrent oral aphthae (two points), skin lesions (papulopustular eruption - one point) and a positive pathergy test (one point). Thus she scored four points which fulfilled the diagnostic criteria for Behçet disease. Additionally, she had HLA-B51 positivity which is a risk allele for Behçet disease. A partial improvement of cutaneous eruption is reported on treatment with oral colchicine3 and this was noticed in our patient as well. Even though the patient performed the epilation procedure upon the entire lower extremity, we believe the friction between the upper legs might be the contributing factor to the emergence of papulopustular eruption and pathergy positivity. Therefore, a final diagnosis of the papulopustular eruption of Behçet disease with pathergy positivity, mimicking acute localised exanthematous pustulosis was made.

Crusted plaque surrounded by pustules on erythematous base on medial aspect of left thigh
Figure 1a:
Crusted plaque surrounded by pustules on erythematous base on medial aspect of left thigh
Pathergy test positivity showing erythematous papules and pustules
Figure 1b:
Pathergy test positivity showing erythematous papules and pustules
Regression of the pustules and desquamation after treatment
Figure 1c:
Regression of the pustules and desquamation after treatment
Subcorneal intraepidermal spongiotic pustule, expanding into dermis, erythrocyte extravasation and oedema in the adjacent dermis, infiltrate consisting of mainly neutrophils and eosinophils (Hematoxylin and eosin, ×100).
Figure 2:
Subcorneal intraepidermal spongiotic pustule, expanding into dermis, erythrocyte extravasation and oedema in the adjacent dermis, infiltrate consisting of mainly neutrophils and eosinophils (Hematoxylin and eosin, ×100).

Acute localised exanthematous pustulosis is an adverse cutaneous drug eruption characterised by non-follicular, sterile pustule formation in a localised area of the body. The most common incriminated drugs are beta-lactam antibiotics such as cephalosporins, amoxicillin, ampicillin and macrolides.4 Pustular eruption starts within a few days after the drug initiation and systemic symptoms such as fever and fatigue may also accompany.4 Pustular eruption on the left inner thigh in our patient had started after an epilation session similar to her previous attacks of folliculitis underlined the fact that her lesions were triggered by traumatic procedures which in a way resembles the pathergy positivity. Continuous friction between the upper inner thighs might have created a trauma which further provoked the expansion of the pustular eruption. We diagnosed her lesions as the papulopustular eruption of Behçet disease with pathergy positivity with the help of histopathological confirmation which showed subcorneal spongiform pustule formation infiltrated predominantly by neutrophils and eosinophils and no follicular based inflammation. The papulopustular lesions of Behçet disease may show various histopathological findings such as leukocytoclastic vasculitis, perifollicular/perivascular infiltration, suppurative folliculitis or intrafollicular abscess formation.5,6 Ergun et al.7 reported that intraepidermal pustule formation, polymorphonuclear and lymphocytic aggregates were the histopathological hallmarks of the pustular lesions induced by pathergy test in patients with Behçet disease, similar to our case which showed subcorneal pustule formation with neutrophilic, interstitial and lymphoplasmacytic infiltrate.

We would like to emphasize that patients with Behçet disease might be prone to develop papulopustular eruptions exacerbated by trauma, which may mimic other pustular skin diseases including acute localised exanthematous pustulosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflict of interest

There are no conflicts of interest.

References

  1. . Clinical and pathological manifestations with differential diagnosis in Behçet’s Disease. Patholog Res Int. 2012;2012:690390.
    [CrossRef] [PubMed] [Google Scholar]
  2. . The International Criteria for Behçet’s Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28:338-47.
    [CrossRef] [PubMed] [Google Scholar]
  3. , . Management of Behcet’s syndrome. Rheumatology (Oxford). 2020;59:iii108-17.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , , , , . Two cases of amoxycillin-induced follicular acute localised exanthematous pustulosis. Australas Dermatol. 2017;58:e23-e25.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , , . Can specific vessel-based papulopustular lesions of Behçet’s disease be differentiated from nonspecific follicular-based lesions clinically? Int J Dermatol. 2006;45:814-8.
    [CrossRef] [PubMed] [Google Scholar]
  6. , , , , , , et al. Histopathological and clinical evaluation of papulopustular lesions in Behçet’s disease. Clin Exp Rheumatol. 2015;33:S101-6.
    [PubMed] [Google Scholar]
  7. , , , , . The histopathology of pathergy: A chronologic study of skin hyperreactivity in Behçet’s disease. Int J Dermatol. 1998;37:929-33.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
4,309

PDF downloads
3,236
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections