Generic selectors
Exact matches only
Search in title
Search in content
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 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
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
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
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
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
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
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Quiz
2019:85:4;407-409
doi: 10.4103/ijdvl.IJDVL_896_16
PMID: 31172980

Persistent perioral papules in a young man

Piyush Kumar1 , Anupam Das2 , Rizwana Barkat1
1 Department of Dermatology, Katihar Medical College, Katihar, Bihar, India
2 Department of Dermatology , KPC Medical College, Kolkata, West Bengal, India

Correspondence Address:
Piyush Kumar
Katihar Medical College, Katihar, Bihar
India
How to cite this article:
Kumar P, Das A, Barkat R. Persistent perioral papules in a young man. Indian J Dermatol Venereol Leprol 2019;85:407-409
Copyright: (C)2019 Indian Journal of Dermatology, Venereology, and Leprology

Case History

A 22-year-old otherwise healthy male presented with asymptomatic persistent 2-4mm sized papules distributed over the perioral region for the preceding 2 years. The lesions were asymptomatic but the patient reported a tendency to pick at the lesions. His medical as well as surgical history was non-contributory. On examination, multiple, soft-to-firm, flesh-colored to hyperpigmented papules were noted in the perioral area [Figure - 1]. Rest of the mucocutaneous and systemic examination was unremarkable. The lesions had not responded to anti-acne therapy, including benzoyl peroxide, tretinoin and adapalene.

Figure 1: Multiple flesh-colored to hyperpigmented papules in the perioral location

Routine blood investigations including complete blood count, biochemical profile and serum angiotensin converting enzyme estimation were normal. Serology for human immunodeficiency virus and Venereal Disease Research Laboratory test were negative. A 3-mm punch biopsy was taken from a representative papule.

Histopathological findings showed moderately dense superficial and deep perivascular and periappendageal infiltrate of lymphocytes and eosinophils. Hair follicles showed enlargement of infundibulum with deposition of abundant mucin within the infundibular keratinocytes. The infundibulum also showed mild spongiosis and lymphocytic infiltration. Overlying epidermis was unaffected. Alcian blue stain showed blue staining areas confirming mucinous degeneration [Figure - 2]a, [Figure - 2]b and [Figure - 3]. Of note, infiltrating lymphocytes did not show any features of atypia. Immunohistochemistry could not be done for lack of facilities at our institute.

Figure 2:
Figure 3: Mucinous degeneration of follicle (Alcian blue, ×400)

What Is Your Diagnosis?

Diagnosis

Histopathologic findings were consistent with follicular mucinosis. Considering clinical findings, a diagnosis of acneiform follicular mucinosis was made.

Discussion

Follicular mucinosis is a disorder characterized by mucinous degeneration of follicle on histopathology and clinically presents as follicular prominences, alopecia and/or comedone-like plaques.[1] The condition was first described as “alopecia mucinosa” by Pinkus in 1957, and since then, both terms have been used interchangeably.[2]

Follicular mucinosis has been documented in all races and ages and affects both sexes equally.[3] It has been described mainly in two clinical settings – an idiopathic or primary form which usually presents in children and young adults and may show spontaneous remission, and the other lymphoma-associated form which presents in older population and runs a chronic course. Most lymphoma-associated cases are seen in association with mycosis fungoides.[3]

Primary and lymphoma-associated forms have some distinguishing clinical and histopathological features. The lesion in primary follicular mucinosis is solitary in a majority of cases (65%) and is usually restricted to the head and neck region. It is commoner in younger population and often resolves spontaneously. On the other hand, lesions in lymphoma-associated forms are often multiple and widespread on the trunk and extremities. It is seen in older population and does not show spontaneous regression.[3] However, these clinical findings are not absolute.

Deposition of mucin, beginning in the outer root sheath and sebaceous apparatus of hair follicles and perifollicular infiltrate, are the histopathological hallmarks of follicular mucinosis. The inflammatory cell infiltrate in the primary form is predominantly perifollicular and perivascular, whereas infiltrate in lymphoma-associated form is more dispersed, nodular and consists of more plasma cells and fewer eosinophils. Moreover, atypical lymphocytes (enlarged hyperchromatic nuclei with irregular contours) with epidermotropism are frequently seen in lymphoma-associated forms. Of note, mucin deposition is abundant in primary follicular mucinosis in comparison to lymphoma-associated mucinosis. However, overlap of histological features has been described in the literature.[3],[4]

Acneiform follicular mucinosis has also been described as a rare variant of primary follicular mucinosis which presents with erythematous-to-skin-colored papules on the face. The disease runs a chronic course and the lesions may persist for months to years. The differential diagnoses include perioral dermatitis (reddish papules, vesicles and pustules on an erythematous base with a tendency to coalesce), facial demodicosis (facial itching, erythema, seborrheic dermatitis-like eruption mimicking perioral dermatitis), granulomatous periorificial dermatitis (yellow-to-brown papular lesions over perioral, perinasal and periocular regions, common in children), sarcoidosis (histology shows noncaseating granulomas), etc., Various treatment options have been tried effectively including tetracycline, minocycline, isotretinoin, topical tacrolimus and pimecrolimus.[5] Our patient did not report much improvement after a 1 month course of oral minocycline 100mg once daily and was later lost to follow up. The prognosis of such cases is uncertain, and hence, long-term follow-up and monitoring is essential.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References
1.
Yaman B, Gerçeker Türk B, Öztürk G, Ertam I, Kandıloǧlu G, Akalin T, et al. Follicular mucinosis and follicular mycosis fungoides: Clinicopathological evaluation of seven cases. Turk Patoloji Derg 2013;29:108-16.
[Google Scholar]
2.
Böer A, Ackerman AB. Alopecia mucinosa or follicular mucinosis – The problem is terminology! J Cutan Pathol 2004;31:210-1.
[Google Scholar]
3.
Rongioletti F, De Lucchi S, Meyes D, Mora M, Rebora A, Zupo S, et al. Follicular mucinosis: A clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol 2010;37:15-9.
[Google Scholar]
4.
Mehregan DA, Gibson LE, Muller SA. Follicular mucinosis: Histopathologic review of 33 cases. Mayo Clin Proc 1991;66:387-90.
[Google Scholar]
5.
Brau-Javier CN, Santos-Arroyo AE, De Sanctis-González IM, Sánchez JL. Follicular mucinosis presenting as an acneiform eruption: A follow-up study. Am J Dermatopathol 2013;35:792-6.
[Google Scholar]

Fulltext Views
591

PDF downloads
118
Show Sections