Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
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
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
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 - 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
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
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
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
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
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Report
doi: 10.4103/0378-6323.37064
PMID: 18032865

Epidermodysplasia verruciformis associated with isolated IgM deficiency

Ulker Gul1 , Secil Soylu1 , Rana Yavuzer2
1 Department of Dermatology, Ankara Numune Education and Research Hospital, Ankara, Turkey
2 Department of Dermatology, Medical Faculty of Ankara University; Ankara, Turkey

Correspondence Address:
Secil Soylu
Cemal Grsel Cad. 66/10, Cebeci, Ankara PA 06590
How to cite this article:
Gul U, Soylu S, Yavuzer R. Epidermodysplasia verruciformis associated with isolated IgM deficiency. Indian J Dermatol Venereol Leprol 2007;73:420-422
Copyright: (C)2007 Indian Journal of Dermatology, Venereology, and Leprology


A 20 year-old man presented to our clinic with multiple warts on both hands and tumoral lesions on his face but otherwise healthy. On dermatological examination, numerous brown-black papular lesions, changing from 2 to 5 cm in diameter were found on his face along with multiple, flesh-coloured, flat-topped papules on the dorsa of his hands. A tumoral lesion, approximately 3 cm in diameter on the right side of his forehead and desquamated erythematous macules were also observed on the trunk. Laboratory investigations showed that serum immunoglobulin M (IgM) level was decreased. The histopathological examination of verrucous lesions on the hands was consistent with epidermodysplasia verruciformis and the histopathological diagnosis of the tumoral lesion was squamous cell carcinoma.
Keywords: Epidermodysplasia verruciformis, IgM deficiency, Immune system
Figure 2: The actinic keratoses, verrucous lesions and tumoral lesion on the forehead
Figure 2: The actinic keratoses, verrucous lesions and tumoral lesion on the forehead
Figure 1: The flesh-colored, flat-topped papules on the dorsa of the hands
Figure 1: The flesh-colored, flat-topped papules on the dorsa of the hands

Epidermodysplasia verruciformis (EV) is a rare genodermatosis caused by human papillomavirus (HPV) types 3, 5, 8-10, 12, 14, 15, 17 and 19-25. [1],[2] Although most EV patients have been found to have an impaired cell-mediated immunity, their humoral immunities have so far been known to be preserved. We report only the second instance reported case of epidermodysplasia verruciformis with IgM deficiency.

Case Report

A 20 year-old man presented to our clinic with multiple warts on both hands and tumoral lesions on his face. His history revealed a skin cancer on his scalp which was excised at another center three years ago. His sister had similar lesions.

On physical examination, there was no pathology except the skin lesions. On dermatological examination, numerous, brown-black papular lesions, ranging from 2 to 5 cm in diameter were found on his forehead and chin along with multiple, flesh-coloured, flat-topped papules on the dorsa of his hands [Figure - 1]. A tumoral lesion, approximately 3 cm in diameter covered with hemorrhagic crusts was seen on the right side of his forehead along with desquamated erythematous macules on the trunk [Figure - 2].

Laboratory findings including complete blood count, urinalysis and serum glucose level were within normal limits. Hepatitis, tumor markers and anti-human immunodeficiency virus (anti-HIV) antibodies were absent. Serum IgA, IgG, C3 and C4 levels, measured by nephelometric technique were within normal limits, whereas the serum IgM level was found to be decreased [IgM: 0.336 g/L (age-related normal values = 0.50-3.20 g/L)]. No pathologic finding was found in the chest roentgenogram, cranial tomography and abdominal ultrasonography. Multiple biopsies were taken from the tumoral lesions on the forehead, pigmented lesions on the chin and verrucous lesions on the hands.

The histopathological examination of the pigmented lesion on the chin was consistent with actinic keratosis. The histopathological examination of verrucous lesions on the hands revealed irregular epidermal hyperplasia, hypergranulosis and keratinocytes including viral inclusion bodies. These findings confirmed the diagnosis of "EV". An immunohistochemical examination revealed keratinocyte nuclei stained brown with polyclonal HPV antibodies. The tumoral lesion was histopathologically diagnosed as squamous cell carcinoma (SCC). The case was appraised as an autosomal recessive trait on genetic consultation. The tumoral lesion was totally excised and the patient is still being followed up.


Epidermodysplasia verruciformis is a rare genodermatosis characterized by disseminated infection by HPV. [1],[3] The lesions of EV usually begin in childhood and continue throughout the patient′s lifetime. Skin lesions on the dorsal surfaces of the hands, extremities and face include flat, wart-like papular lesions, which are usually caused by the same HPV types found in flat warts in the general population. On the trunk, there are red-brown, slightly scaly macules resembling tinea versicolour that are caused by EV-HPV types. [1],[3],[4]

About 50% of all EV cases are inherited, usually with an autosomal recessive pattern. X-linked inheritance has also been reported. [1],[3],[5] The sister of our patient had similar lesions on this skin. The case was appraised as autosomal recessive trait after a genetic consultation.

The pathogenesis of EV may be explained by three different factors: genetic factors, immunological factors and HPV infection. Although most EV patients have been found to have an impaired cell-mediated immunity, it is still not known whether the impairment of the immune response is primary or secondary to massive HPV infection. [6] There are subtle changes in lymphocyte function such as lymphopenia with a reduced B cell/T cell ratio but a normal T helper/T suppressor ratio in some patients. Sometimes, reduced spontaneous migration and chemotaxis of neutrophils can be observed. Despite these findings, the patients are not usually susceptible to other viral and bacterial infections. [7] Majewski et al . reported that natural cell-mediated cytotoxicity was in the normal range in EV patients. [8] It has been thought that the basic immunological defect might be the inability to recognize EV-specific HPVs in EV patients. [6]

There are also case reports regarding the association of idiopathic CD4 lymphocytopenia with epidermodysplasia verruciformis-like eruptions. [9],[10] Idiopathic CD4 lymphocytopenia possibly caused by an as-yet unidentified virus or viruses varies from minimal abnormalities to fatal opportunistic infections. In these cases, the absence of evidence of HIV-1 infection should be noted. [11] Our patient did not have any findings of opportunistic infections. Therefore, the CD4 lymphocyte count was not examined.

Epidermodysplasia verruciformis patients are not prone to bacterial, fungal or viral infections with the exceptions of HPVs associated with plane warts. [1] Their humoral immunities are known to be preserved. However, Iraji and Faghihi reported a case of EV associated with decreased serum IgM levels. The serum IgM level of their patient was as low as 60 mg/dL (normal range: 73-267 mg/dL). In selective IgM deficiency, eczema and persistent, large, viral warts may be present but the main clinical problems usually consist of meningecoccal and pneumococcal infections. Their case had no other pathology except EV. [7] Similarly, our case had EDV associated with selective IgM deficiency but was otherwise healty.

Multiple skin cancers such as Bowen′s disease, squamous cell carcimonas (SCC), actinic keratoses, basal cell carcinomas and sweat apparatus carcinomas develop in one-third of EV patients in sun-exposed areas. [6] Although it is still unclear which EV-HPVs are responsible for the development of malignant lesions, HPV-5 and -8 are considered the biggest culprits. [1],[2],[8] Our case developed SCC.

This case is the second reported case of EV associated with IgM deficiency although this finding may be just coincidental. However, the case of EV with an isolated IgM deficiency previously reported by Iraji and Faghihi and our case raise the question as to whether selective IgM deficiency can play a role in the pathogenesis of EV or whether there can be a genetic association between these disorders. Studies with a large number of EV patients can clarify whether there is an actual relationship between EV and IgM deficiency.

Lowy DR, Androphy EJ. Warts. In : Freedberg IM, Eisen AZ, Wolff K, editors. Fitzpatrick's dermatology in general medicine. 6th ed. Mc-Graw Hill: New York; 2003. p. 2119-31.
[Google Scholar]
Pfister H. Chapter 8: Human papillomavirus and skin cancer. J Natl Cancer Inst Monogr 2003;31:52-6.
[Google Scholar]
Pereira de Oliveira WR, Carrasco S, Neto CF, Rady P, Tyring SK. Nonspecific cell-mediated immunity in patients with epidermodysplasia verruciformis. J Dermatol 2003;30:203-9.
[Google Scholar]
Kawashima M. Epidermodysplasia verruciformis. J Dermatol 1992;19:707-9.
[Google Scholar]
Androphy EJ, Dvoretzky I, Lowy DR. X-linked inheritance of epidermodysplasia verruciformis: Genetic and virologic studies of a kindred. Arch Dermatol 1985;121:864-8.
[Google Scholar]
Ishiji T, Kawase M, Honda M, Niimura M, Yoshimura E, Sata T, et al . Distinctive distribution of human papillomavirus type 16 and type 20 DNA in the tonsillar and the skin carcinomas of a patient with epidermodysplasia verruciformis. Br J Dermatol 2000;143:1005-10.
[Google Scholar]
Iraji F, Faghihi G. Epidermodysplasia verruciformis: Association with isolated IgM deficiency and response to treatment with acitretin. Clin Exp Dermatol 2000;25:41-3.
[Google Scholar]
Majewski S, Skopinska-Rozewska E, Jablonska S, Wasik M, Misicwicz J, Orth G. Partial defects of cell-mediated immunity in patients with epidermodysplasia verruciformis. J Am Acad Dermatol 1986;15:966-73.
[Google Scholar]
Yinnon AM, Rudensky B, Sagi E, Breuer G, Brautbar C, Polacheck I, et al . Invasive cryptococcosis in a family with epidermodysplasia verruciformis and idiopathic CD4 cell depletion. Clin Infect Dis 1997;25:1252-3.
[Google Scholar]
Tobin E, Rohwedder A, Holland SM, Philips B, Carlson JA. Recurrent 'sterile' verrucous cyst abscesses and epidermodysplasia verruciformis-like eruption associated with idiopathic CD4 lymphopenia. Br J Dermatol 2003;149:627-33.
[Google Scholar]
Roberts RL, Stiehm ER. T-cell immunodeficiency disorders. In : Parslow TG, Stites DP, Terr AI, editors. Lange Medical Immunology. 10th ed. Mc-Graw Hill: New-York; 2001. p. 313-9.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections