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:

Net letter
2010:76:6;724-724
doi: 10.4103/0378-6323.72478
PMID: 21079335

Linear perforating lesions in dystrophic epidermolysis bullosa: Is it Koebner's phenomenon?

Amiya Kumar Nath, Devinder Mohan Thappa, Surendra Kumar Varma
 Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India

Correspondence Address:
Devinder Mohan Thappa
Department of Dermatology and STD, JIPMER, Pondicherry - 605 006
India
How to cite this article:
Nath AK, Thappa DM, Varma SK. Linear perforating lesions in dystrophic epidermolysis bullosa: Is it Koebner's phenomenon?. Indian J Dermatol Venereol Leprol 2010;76:724
Copyright: (C)2010 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Dystrophic epidermolysis bullosa (DEB) is a rare, inherited, blistering disease caused by mutations in a single gene, COL7A1, which encodes the anchoring fibril protein, type VII collagen. [1] DEB has two main subtypes: the more aggressive autosomal recessive type and much less severe dominant type. Besides the typical skin lesions, the dominant DEB may also have congenital absence of skin (Bart syndrome), atopic dermatitis, prurigo-like nodules, and albo-papuloid lesions. [2] The presence of perforating skin lesions in patients with DEB is unknown in the English literature. We hereby report a case of dominant DEB showing linearly disposed perforating lesions at various body sites and propose a plausible hypothesis for such occurrence.

A 4.5-year-old male child was brought with a history of spontaneous as well as trauma-induced blistering in both hands and feet since birth. Loss of nails due to blistering was also noted from early infancy. There was history of blistering in the oral mucosa and development of abnormal teeth, but no history of blistering elsewhere. Multiple, itchy, hyperpigmented, raised, linear lesions developed in the lower abdomen, inguinal region, groins, both axillae, popliteal fossae, wrists, ankles, dorsa of hands and feet from early infancy. His parents were second degree consanguineous. No one else in the family had similar problem. On examination, multiple bullae (some pus-filled) and crusted ulcers were seen in both palms and palmar aspects of the fingers [Figure - 1]a. Scarring and atrophy were also seen in the palms and fingers, being more prominent in the tips of the fingers. Well-defined areas of hyperkeratosis were present over both the heels and lateral aspects of the forefeet [Figure - 1]a. Fissures were seen on the medial aspect of left foot and toe clefts. An attenuated and dystrophic nail was seen in the right thumb. Remaining digits showed complete absence of the nail plate, with scarring and atrophy of the nail beds [Figure - 1]b. Mild contractures and resorption of the terminal phalanges of the fingers were also observed. Upper incisors were deformed and showed enamel defects. These features were consistent with dominant DEB. There were multiple, 2-5 mm sized, hyperkeratotic papules in the extensor aspects of bilateral elbows, knees, dorsa of hands, ankles, and dorsa of feet [Figure - 1]b. Some of the papules showed a central keratotic plug, removal of which revealed a crater. Linear hyperkeratotic streaks were present in the lower abdomen, inguinal region, groin [Figure - 2], bilateral popliteal fossae, axillae, ankles [Figure - 1]b, dorsa of hands and feet. Other cutaneous, hair and systemic examinations were within normal limits. Histopathology of the skin biopsy from the linear hyperkeratotic lesions showed acanthosis, transepidermal elimination of basophilic, degenerated collagen and elastic tissue materials admixed with inflammatory cell infiltrates [Figure - 3]. These findings were consistent with acquired perforating dermatosis. A final diagnosis of dominant DEB associated with perforating dermatosis was made. Topical tretinoin was prescribed for the perforating skin lesions, and care for epidermolysis bullosa was explained.

Figure 1 :(a) Multiple bullae, crusted ulcers, scarring, and atrophy on the palms and fingers, along with hyperkeratosis of both soles; (b) absence of finger and toe nails with scarring of the nail beds, linear hyperkeratotic streaks over the ankles and wrists, and hyperkeratotic papules with central keratotic plug over the dorsa of hands and feetd
Figure 2 :Linear hyperkeratotic streaks in the lower abdomen, inguinal region, and groins
Figure 3 :Photomicrograph shows acanthosis, with transepidermal elimination of basophilic, degenerated collagenous and elastic tissue materials admixed with inflammatory cell infiltrates (H and E, ×100)

All forms of DEB are characterized by skin fragility, blistering, scarring, nail changes and milia formation. [3] The essential feature of dominant DEB is that the skin is generally less fragile than the recessive type, and blisters mostly develop over bony prominences (such as the knees and ankles, and dorsa of the hands or feet) following sharp blows. [2] The Cockayne-Touraine variety of dominant DEB is usually localized only to the extremities and is the milder expression of the blistering disease process, while the Pasini variant has more intense blistering and is more generalized. [3] The most consistent findings of DEB are localized scarring with milia formation and dystrophic nails. [2] Nail dystrophy is probably the most important diagnostic feature of the disease. [2]

Perforating disorders are a group of disorders characterized by extrusion of altered dermal components through a transepithelial channel. [4] There are four primary perforating disorders, i.e. Kyrle′s disease, perforating folliculitis, reactive perforating collagenosis, and perforating serpiginous elastosis. [5] However, many cases occur secondary to some underlying disease, such as granuloma annulare or PXE. Similar skin lesions noted in patients with diabetes and renal failure have been called acquired perforating dermatosis. [5] Occasionally, topically applied chemicals or intradermal injection can be eliminated by the transepidermal route to produce a perforating disorder. [5] Koebnerization is known to occur in almost all the perforating disorders with maximum prevalence in reactive perforating collagenosis. [4],[6] The perforating skin lesions in our case were like those of acquired perforating dermatosis. We considered elastosis perforans serpiginosa but ruled it out because lesions were not in typical serpiginous or arcuate in arrangement. [7]

The reason for occurrence of perforating lesions in DEB is at best hypothetical in our case. DEB could have been the underlying factor for abnormal alteration of dermal components followed by subsequent extrusion of the "altered" dermal elements through transepithelial channel. It was an interesting observation in our case that most of the skin areas affected by linear lesions never had blisters of DEB. It raises the possibility that alteration of upper dermal elastic tissue could have occurred in the apparently normal looking skin. The unique features in our patient were the linear arrangement of the perforating lesions, predominantly involving the flexural skin. The linear arrangement of perforating lesions could have been due to Koebnerization as the lesions were itchy. However, no atopic background was noted in our case.

References
1.
Horn HM, Tidman MJ. The clinical spectrum of dystrophic epidermolysis bullosa. Br J Dermatol 2002;146:267-74.
[Google Scholar]
2.
Eady RAJ, Fine JD, Burge SM. Genetic blistering diseases. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7 th ed. Oxford: Blackwell Science; 2004. p. 1-40.
th ed. Oxford: Blackwell Science; 2004. p. 1-40.'>[Google Scholar]
3.
Burkhart CG, Ruppert ES. Dystrophic epidermolysis bullosa. Clin Pediatr (Phila) 1981;20:493-6.
[Google Scholar]
4.
Rapini RP. Perforating Diseases. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 2 nd ed. Spain: Mosby Elsevier; 2008. p. 1461-17.
[Google Scholar]
5.
Burrows NP, Lovell CR. Disorders of Connective Tissue. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7 th ed. Oxford: Blackwell Science; 2004. p. 64-7.
th ed. Oxford: Blackwell Science; 2004. p. 64-7.'>[Google Scholar]
6.
Thappa DM. The isomorphic phenomenon of Koebner. Indian J Dermatol Venereol Leprol 2004;70:187-9.
[Google Scholar]
7.
Mehta RK, Burrows NP, Payne CM, Mendelsohn SS, Pope FM, Rytina E. Elastosis perforans serpiginosa and associated disorders. Clin Exp Dermatol 2001;26:521-4.
[Google Scholar]

Fulltext Views
190

PDF downloads
180
Show Sections