Indian Dermatology Online Journal

SKINDIA QUIZ
Year
: 2016  |  Volume : 7  |  Issue : 4  |  Page : 348--349

Sk India Quiz 26: Hypopigmented papules in a human immunodeficiency virus-positive patient


Lalit Kumar Gupta, Manisha Balai, Ashok Kumar Khare, Asit Mittal 
 Department of Dermatology, Venereology and Leprology, R.N.T. Medical College, Udaipur, Rajasthan, India

Correspondence Address:
Lalit Kumar Gupta
Department of Dermatology, Venereology and Leprology, R.N.T. Medical College, Udaipur - 313 001, Rajasthan
India




How to cite this article:
Gupta LK, Balai M, Khare AK, Mittal A. Sk India Quiz 26: Hypopigmented papules in a human immunodeficiency virus-positive patient.Indian Dermatol Online J 2016;7:348-349


How to cite this URL:
Gupta LK, Balai M, Khare AK, Mittal A. Sk India Quiz 26: Hypopigmented papules in a human immunodeficiency virus-positive patient. Indian Dermatol Online J [serial online] 2016 [cited 2020 May 30 ];7:348-349
Available from: http://www.idoj.in/text.asp?2016/7/4/348/182402


Full Text

A 40-year-old human immunodeficiency virus. (HIV)-positive male presented with one year history of multiple, asymptomatic, hypopigmented lesions on upper limb and trunk. The patient was on zidovudine, lamivudine, and nevirapine for 9. months. His CD4 count was 92. cells/mm3. Physical examination showed scaly, discrete to confluent papules of 1-3. mm size and distributed symmetrically on shoulder, arms and trunk [Figure 1]a. Koebnerization was evident at some places. Some of the confluent lesions had figurate morphology [Figure 1]b. None of the family members had similar lesions. Potassium hydroxide examination of scraped hypopigmented skin lesions did not show fragmented hyphae. Histopathology showed broad plate like epidermal hyperplasia with focal hypergranulosis and several vacuolated cells (koilocytes) with abundant pale cytoplasm in the upper spinous layer [Figure 2]a,[Figure 2]b.

 QUESTION



What is your diagnosis?

 View Answer

 ANSWER



Epidermodysplasia verruciformis (EV).

 DISCUSSION



Epidermodysplasia verruciformis, first described in 1922 by Lewandowksi and Lutz, is a very rare, chronic disease, characterized by a unique susceptibility to cutaneous infections by a group of phylogenetically related human papilloma virus (HPV) types, referred as EV types.[1] In most EV-affected families, inheritance follows an autosomal recessive pattern and recently, truncating mutations in two genes, TMC6 (EVER 1) and TMC8 (EVER 2) have been identified as a cause of EV. About 2 dozen EV-specific HPV types have been described, a subset of which (mainly 5 and 8) is detected in EV-associated skin cancers.[2] Squamous cell carcinomas usually arise in pityriasis versicolor like EV-lesions on sun exposed areas. It usually manifests in childhood with persistent and widespread warts. Individual lesions typically have either the appearance of flat warts or flat, scaly red-brown or hypopigmented macules that resemble lesions of pityriasis versicolor.[3] Histopathologically EV is characterized by hyperkeratosis, acanthosis and prominent vacuolation in the Malphigian layer. Biopsies of EV lesions from HIV-positive patients report higher rates of dysplasia (63%) compared to the patients of EV without immunosuppression (20%).[4] However, our case did not reveal features of dysplasia. Treatment is usually less successful in HIV-associated EV than classical EV. In various series[5],[6],[7] of HIV patients with EV, different treatment modalities including systemic retinoids, interferon, imiquimod, and combination of systemic and topical therapies have been attempted and found to be either unsuccessful, inconsistent or associated with rapid recurrence after treatment. The diagnosis of EV should be considered in patients with HIV who present with pityriasis versicolor like disseminated hypopigmented lesions resistant to treatment with antifungal medications.[8]

References

1Majewski S, Jabłońska S. Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin. Arch Dermatol 1995;131:1312-8.
2Kirnbauer R, Lenz P. Human papillomaviruses. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology. 3rd ed. China: Elsevier Saunders; 2012. p. 1303-19.
3Androphy EJ, Kirnbauer R. Human papilloma virus infections. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Lefell DJ, Wolff K, editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw Hill; 2012. p. 2421-33.
4Morrison C, Eliezri Y, Magro C, Nuovo GJ. The histologic spectrum of epidermodysplasia verruciformis in transplant and AIDS patients. J Cutan Pathol 2002;29:480-9.
5Berthelot C, Dickerson MC, Rady P, He Q, Niroomand F, Tyring SK, et al. Treatment of a patient with epidermodysplasia verruciformis carrying a novel EVER 2 mutation with imiquimod. J Am Acad Dermatol 2007;56:882-6.
6Janssen K, Lucker GP, Houwing RH, van Rijssel R. Epidermodysplasia verruciformis: Unsuccessful therapeutic approach with imiquimod. Int J Dermatol 2007;46 Suppl 3:45-7.
7Rallis E, Paparizos V, Kyriakis K, Katsambas A. Treatment of epidermodysplasia verruciformis in human immunodeficiency virus-positive patients. J Eur Acad Dermatol Venereol 2009;23:195-6.
8Hultgren TL, Srinivasan SK, DiMaio DJ. Epidermodysplasia verruciformis occurring in a patient with human immunodeficiency virus: A case report. Cutis 2007;79:307-11.