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
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter To Editor
2008:74:3;283-285
doi: 10.4103/0378-6323.41395
PMID: 18583816

Familial acne inversa with acne conglobata in three generations

PVS Prasad1 , PK Kaviarasan1 , Joe Mathew Joseph1 , S Madhuri1 , P Viswanathan2
1 Department of Dermatology, Venereology and Leprosy, Rajah Muthiah Medical College and Hospital, Annamalai University, Annamalai Nagar, Tamil Nadu, India
2 Department of Pathology, Rajah Muthiah Medical College and Hospital, Annamalai University, Annamalai Nagar, Tamil Nadu, India

Correspondence Address:
PVS Prasad
88, Auta Nagar, Sivapuri Post, Annamalai Nagar - 608 002
India
How to cite this article:
Prasad P, Kaviarasan P K, Joseph JM, Madhuri S, Viswanathan P. Familial acne inversa with acne conglobata in three generations. Indian J Dermatol Venereol Leprol 2008;74:283-285
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Acne inversa (Hidradenitis suppurativa) is a chronically relapsing inflammatory disease that is characterized by recurrent draining sinuses and abscesses occurring predominantly in skin folds that carry terminal hairs and apocrine glands. [1] Healing occurs with substantial scarring. We report here a family with acne inversa and acne conglobata occurring in three generations.

A 33 year-old male with no history of consanguinity, presented to the outpatient department with multiple painful swellings of eleven years′ duration over the face, chest, axillae and gluteal region. He had taken various courses of antibiotics without much benefit. His younger brother also was suffering from similar complaints, but with less severity. Both his father and grandfather also had similar lesions in the axillae, but had not taken any treatment. Local examination revealed multiple, hyperpigmented, firm nodules of varying sizes measuring one to several centimeters in size. Nodules on the chest wall coalesced to form large plaques with sinus-discharging pus. Larger nodules were seen on the gluteal region [Figure - 1]. The inguinal lymph nodes were enlarged, discrete and nontender. General and systemic examinations were unremarkable. His younger brother aged 30 years also showed similar lesions of ten years′ duration. On examination, he also revealed similar nodules and cystic lesions, but the lesions were less severe. There was no sinus formation at the time of examination [Figure - 2].

Investigations revealed normal blood counts, liver and renal function tests and the ESR was 110 cm in the first hour. Glucose tolerance test was normal. Bacterial culture showed heavy growth of Staphylococcus aureus . Serology for HIV-1 and 2 gave negative results. Biopsy revealed multiple epidermal inclusion cysts and perifolliculitis with neutrophilic and histiocytic infiltrates. He was treated successively with oral doxycycline, minocycline, isotretinoin, acitretin and intralesional corticosteroids. He had some relief with oral acitretin combined with intralesional steroids, but there was no evidence of complete remission.

Investigations revealed similar findings in the younger sibling. He was treated with rifampicin 600 mg per day for eight weeks, and showed good improvement with prolonged remission.

Both our patients suffered from acne conglobata with acne inverse, which was evident from the history and clinical examination findings. There was no evidence of the follicular occlusion triad as they did not suffer from dissecting cellulitis of scalp.

In some families, acne inversa may show a single gene dominant inheritance. Fitzsimmons et al. studied three families with a total of 21 affected members and reported that the pattern of transmission and the number of affected individuals were consistent with autosomal dominant inheritance. Later, they studied the families of 266 subjects with acne inversa, comprising a total of 62 affected individuals. They reported that 34% were first-degree relatives in 11 families. [2],[3] There was a history of disease in three generations in our patients′ family, suggesting an autosomal dominant inheritance.

More recent studies have identified acne inversa as a disorder of follicular, rather than apocrine occlusion. Thus, apocrine gland involvement is only incidental or secondary to the primary developments that involve the terminal hair follicle. Unlike acne vulgaris, acne inversa is localized in nonfacial regions where there are terminal pigmented coarse hairs. Acne inversa is a segmental rupture of the follicular epithelium, followed by spilling of foreign body material, such as corneocytes, bacteria, sebum products and hairs into the dermis. The dumping of foreign products initiates an inflammatory response provoking foreign body granuloma and epithelial strands, which then try to encapsulate the necrotic tissue. The apocrine glands are not involved in the earliest stage of follicular hyperkeratosis. Once rupture of the follicular epithelium has occurred, the disease spreads rapidly. [4] The draining sinus is a late complication of acne inversa.

Acne inversa has to be differentiated from furuncles, carbuncles, vegetating pyoderma, cutaneous tuberculosis, actinomycosis, lymphogranuloma inguinale and Crohn′s disease. [5] Treatment options include antiseptics, antibiotics and corticosteroids. [6] Tetracycline, clindamycin or rifampicin have all been tried with limited success. Isotretinoin is effective in some cases only. Etretinate and acitretin have been more successful. [7] Incision and drainage or exteriorization of individual lesions may be useful in some instances. [8] Radical surgical excision at the earliest recognized stage remains a mainstay of therapy. Inadequate excision is the main reason for recurrence. Carbon dioxide laser excision is another treatment option for acne inversa.

Our patients were treated with adequate courses of antibiotics. The elder sibling did not respond to the various treatments given. Hence, he was given acitretin at a dose of 25 mg per day for four months. Large nodular lesions began decreasing in size. Intralesional steroid therapy was also given to large nodules on the chin and the lip. These lesions also partially responded to therapy. Some of these lesions recurred after stopping the treatment. Large nodules on the gluteal region were treated by surgical intervention earlier but there was recurrence at that site as well. On the other hand, the younger sibling showed an adequate response with rifampicin. There was no evidence of recurrence after stopping the therapy.

References
1.
Jansen T, Plewig G. Acne inversa. Int J Derm 1998;37:96-100.
[Google Scholar]
2.
Fitzsimmons JS, Fitzsimmons EM, Gilbert G. Familial Hidradenitis suppurativa : Evidence in favor of single gene transmission. J Med Genet 1984:21:281-5.
[Google Scholar]
3.
Fitzsimmons JS, Gilbert PR. A family study of Hidradenitis suppurativa . J Med Genet 1985;22:367-73.
[Google Scholar]
4.
Yu CC, Cook MG. Hidradenitis suppurativa : A disease of follicular epithelium rather than apocrine glands. Br J Dermatol 1990;122:767-9.
[Google Scholar]
5.
Church JM, Fazio VW, Lavery IC. The differential diagnosis and co-morbidity of Hidradenitis suppurativa and perianal Crohn's disease. Int J Colorectal Dis 1993;8:117-9.
[Google Scholar]
6.
Newell GB. Treatment of hidradenitis suppurativa. JAMA 1975;223:556-7.
[Google Scholar]
7.
Chow ET, Mortimer PS. Successful treatment of v and retro auricular acne with etretinate. Br J Dermatol 1992;126:415.
[Google Scholar]
8.
Hughes LE, Harrison BJ, Mudge M. Surgical management of Hidradenitis suppurativa -principles and results. In : Marks R, Plewig G, editors. Acne and related disorders. London: Martin Dunitz; 1989. p. 367-70
[Google Scholar]

Fulltext Views
242

PDF downloads
50
Show Sections