Case for diagnosis

Ribas, Jonas; Corrêa, Clarisse de Albuquerque; Cavalcante, Melissa de Souza Melo

doi:10.1590/S0365-05962011000200035

Abstracts

A 27-year-old mixed-raced (pardo) female patient presented with flat ovate hypochromic plaques with a rough surface on the back and upper limbs, with an aspect resembling pityriasis versicolor. She reports family history involving a brother with similar lesions. Lab tests, including anti-HIV, showed no alterations and a histopathological examination showed enlarged keratinocytes with basophilic and microvacuolated cytoplasm occupying the upper portion of the spinous layer and the granulous layer. After anatomic-clinical correlation, the diagnosis of epidermodysplasia verruciformis pityriasis versicolor like was confirmed.

Epidermodysplasia verruciformis; Papillomavirus infections; Pityriasis

Paciente do sexo feminino, parda, de 27 anos de idade, apresentando há três anos, placas hipocrômicas planas, ovaladas, de superfície áspera, no dorso e membros superiores com aspecto que lembrava pitiríase versicolor. Refere história familiar de um irmão cujas lesões são semelhantes. Exames laboratoriais, incluindo anti-hiv, sem alterações e com histopatológico evidenciando queratinócitos, aumentados de volume, com citoplasma basofílico e microvacuolado ocupando a porção superior da camada espinhosa e a granulosa. Após correlação anátomo-clínica, confirmou-se o diagnóstico de epidermodisplasia verruciforme pitiríase versicolor like.

Epidermodisplasia verruciforme; Infecções por papillomavirus; Pitiríase

WHAT IS YOUR DIAGNOSIS?

^IMSc - Head of the Dermatoly Service and Professor of Dermatology, Federal University of Amazonas (UFAM) - Manaus (AM), Brazil

^IIPhysician - Physician at the Dermatology Department, VII COMAR - Regional Air Command of the Brazilian Air Force - Amazonas (AM), Brazil

Mailing address

ABSTRACT

A 27-year-old mixed-raced (pardo) female patient presented with flat ovate hypochromic plaques with a rough surface on the back and upper limbs, with an aspect resembling pityriasis versicolor. She reports family history involving a brother with similar lesions. Lab tests, including anti-HIV, showed no alterations and a histopathological examination showed enlarged keratinocytes with basophilic and microvacuolated cytoplasm occupying the upper portion of the spinous layer and granular layer. After anatomo-clinical correlation, the diagnosis of epidermodysplasia verruciformis pityriasis versicolor like was confirmed.

Keywords: Epidermodysplasia verruciformis; Papillomavirus infections; Pityriasis

CASE REPORT

A 27-year-old mixed-raced (pardo) female patient presented with flat ovate hypochromic plaques with a rough surface on the back and upper limbs, with an aspect resembling pityriasis versicolor, which she reported having had for three years (Figure 1). She reports family history involving a brother with similar lesions.

The material for histopathology was obtained by punch biopsy, fixed in 10% formaldehyde and stained with hematoxylin-eosin (HE). The test results showed hyperkeratosis without papillomatosis, hypergranulosis and enlarged keratinocytes with basophilic and microvacuolated cytoplasm occupying the upper portion of the spinous layer and granular layer (Figure 2). They also showed mononuclear perivascular infiltrate in the papillary dermis.

After anatomo-clinical correlation, the diagnosis of epidermodysplasia verruciformis pityriasis versicolor like was confirmed. The patient was counseled about the disease, possible treatment and sent for HIV testing.

The patient received treatment with retinoic acid for six months without satisfactory results.

DISCUSSION

Epidermodysplasia verruciformis (EV), originally described by Lewandowsky and Lutz in 1922, is a rare autosomal recessive genodermatosis attributed to infection by specific types of HPV in immunologically deficient individuals. It presents a high familial incidence, especially in children of consanguineous marriages.³

Initial manifestations of the disorder most frequently occur in childhood, and clinical manifestation of EV may present only flat warts, associated with nononcogenic HPV 3 and / or 10, called "benign form". It may also be expressed in a polymorphic way, with a tendency to malignancy associated with multiple Epidermodysplasia Verruciformis Human Papillomavirus (EVHPV), some oncogenic, such as 5 and / or 8, being called "malignant form", which is the most frequently observed. ^4,5

The clinical expression of pityriasis versicolor like, characteristic of the benign form of EV, develops some years after the first lesion. They are initially erythematous macules, but hypochromic in later stages of the disease. ^5.6

When EV is associated with a state of immunosuppression, with HIV being the most prevalent, the main manifestations are hypopigmented lesions of pityriasis versicolor like, with history of antifungal therapy not being rare. Given the rare occurrence of EV in HIV patients, some authors support the hypothesis that EV arises only in those who also have a genetic susceptibility which has not been defined yet. ⁷

A totally effective treatment has not been described yet, and EV is considered an extremely resistant disease. Acitretin (oral, 0.5-1 mg / kg / day for 6 months) is the substance that brings the best results. However, the treatment is less successful when associated with HIV, and anti-viral therapy does not appear to significantly affect the course of the disease. ^7,8,9

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Jablonska S, Fabjanska L, Formas I. On the viral etiology of epidermodysplasia verruciformis. Dermatologica.1966;132:369-85.
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Hammar H, Hammar L, Lambert L, Ringborg U. A case report including EM and DNA Repair investigations in a dermatosis associated with multiple skin cancers: epidermodysplasia verruciformis. Acta Med Scand.1976;200:441-6.
⁴
Orth G, Jablonska S, Jarzabek-Chorzelska M, Obalek S, Rzesa G, Favre M, et al. Characteristics of the lesions and risk of malignant conversion associated with the type of human papillomavirus involved in epidermodysplasia verruciformis. Cancer Res. 1979;39:1074-82.
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⁶
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⁹
Claudy AL, Touraine JL, Mittanne D. Epidermodysplasia verruciformis induced by a new human papillomavirus (HPV-8). Report of a case without immune dysfunction. Effect of treatment with an aromatic retinoid. Arch Dermatol Res.1982;274:213-9.