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1/46. epidermodysplasia verruciformis treated using topical 5-aminolaevulinic acid photodynamic therapy.

    We describe a 65-year-old woman who had had wart-like lesions on the hands, lower arms and forehead for about 45 years. She had already had several basal cell carcinomas excised. Histological study, electron microscopy and in situ hybridization [human papilloma virus (HPV)-types 5/8/12/14/19-23/25/36] of skin biopsies confirmed a diagnosis of epidermodysplasia verruciformis (EV). Photodynamic therapy (PDT) was performed using a 20% 5-aminolaevulinic acid ointment applied for 6 h to the lesions and irradiating using an incoherent light source (lambda = 580-740 nm, 160 mW/cm2, 160 J/cm2). Following PDT, blistering and crusting of the lesions occurred, but these healed completely within 2-3 weeks without scarring, and the cosmetic result was excellent. Six months after PDT a skin biopsy was taken. in situ hybridization was positive for HPV type 8 in skin which was clinically and histologically normal. Twelve months after PDT a few lesions had recurred on the hands. Although permanent cure of EV cannot be achieved by any therapy at present and single lesions continue to appear in this patient, topical PDT might result in better control of HPV-induced lesions. ( info)

2/46. epidermodysplasia verruciformis: association with isolated IgM deficiency and response to treatment with acitretin.

    We describe a 25-year-old woman, who had extensive, large viral warts consistent with epidermodysplasia verruciformis (EV) since she was 6-year-old. Laboratory studies revealed an isolated IgM-deficiency, but the patient demonstrated no other abnormalities. She was treated with oral acitretin (0.5-1 mg/kg/day) for six months and her skin lesions improved slightly. However, after discontinuing the treatment, the lesions came back but she declined further treatment. ( info)

3/46. No apparent effect of cidofovir in epidermodysplasia verruciformis.

    This paper reports the failure of a patient suffering from epidermodysplasia verruciformis, characterised by widespread infection of the skin with human papillomaviruses, to respond to topical and systemic treatment with the antiviral agent, Cidofovir, despite its previously demonstrated effectiveness against a range of different papillomavirus-associated conditions. ( info)

4/46. Distinctive distribution of human papillomavirus type 16 and type 20 dna in the tonsillar and the skin carcinomas of a patient with epidermodysplasia verruciformis.

    BACKGROUND: epidermodysplasia verruciformis (EV) is a rare skin disease characterized by disseminated pityriasis versicolor-like or flat wart-like lesions and by the development of skin carcinomas. It is well established that specific cutaneous human papillomaviruses (EV-HPVs) are associated with both benign and malignant skin lesions in EV patients. However, little is known of the relationship between HPV and the mucosal lesions of EV patients. OBJECTIVES: To detect and identify HPV types associated with skin and mucosal lesions of an EV patient. PATIENT/methods: We investigated the skin carcinoma and the coexisting tonsillar carcinoma of a 41-year-old man with EV. Histopathologically, both lesions were squamous cell carcinomas. We analysed these two lesions by immunohistochemistry, in situ hybridization, and by molecular virology. RESULTS: Neither skin nor tonsillar lesions exhibited positivity for HPV capsid antigen by immunohistochemistry. By Southern blot hybridization, however, the skin carcinoma harboured 'EV-specific' HPV20 dna, while the tonsillar carcinoma harboured 'genital' HPV16 dna. In addition, in situ hybridization localized the respective viral dna in the corresponding lesion. CONCLUSIONS: The results indicate that EV-HPV could be responsible for the development of the skin carcinoma, but not the mucosal carcinoma in this patient. ( info)

5/46. Treatment of epidermodysplasia verruciformis with a combination of acitretin and interferon alfa-2a.

    epidermodysplasia verruciformis (EV) is an autosomal recessive disease characterized by the lifelong eruption of disseminated verrucae-like lesions. Numerous treatment modalities have been used to treat EV without benefit. Recently, retinoid and interferon therapies have been found to be of value in the treatment of EV. We present a case of EV that was treated with a combination of acitretin and interferon alfa-2a. ( info)

6/46. HPV5b variant in a neoplastic lesion of an Italian patient affected by epidermodysplasia verruciformis.

    epidermodysplasia verruciformis (EV) can be defined as a genetic disorder that determines a lifelong infection of the skin by human Papillomaviruses (HPV). The benign lesions contain different HPVs; whereas in the tumors HPV types 5, 8, and, much less frequently, types 14, 17, 20 and 47 can be detected. Variants of HPV5 have been recognized on the basis of the genetic heterogeneity of the E6 open reading frame. We report a typical case of EV in which the presence and expression of the HPV type 5 were clearly detectable. Direct sequence analysis demonstrated a perfect homology with the sequence of the HPV5b variant. This variant was first isolated in a Japanese patient and thereafter in a Polish one. Its presence in the Italian patient indicates that the same variant can be detected in different geographic areas and therefore that some cellular genes may exert a selection pressure so strong as to induce the emergence of this stable HPV5b infectious variant. ( info)

7/46. epidermodysplasia verruciformis associated with neurofibromatosis type 1: coincidental association or model for understanding the underlying mechanism of the disease?

    We describe a 25-year-old man with epidermodysplasia verruciformis (EV) associated with neurofibromatosis type 1 (NF1). The lesions, persisting for more than 15 years, consisted of widespread planar warts on the backs of the hands and wrists, and reddish-brown macules on the trunk, neck and face. During the last 5 years, our patient developed several epithelial tumours, namely solar keratoses, plaques of bowen's disease and squamous cell carcinomas (SCCs). He also presented with NF1 lesions with neurofibromas, cafe-au-lait macules, axillary freckling and Lisch nodules. He had left tibial bowing. polymerase chain reaction analysis of the skin lesions demonstrated the presence of human papillomavirus (HPV) 15 in a flat wart, HPV 20 in a plaque of bowen's disease, and HPV 15 and HPV 20 in an SCC lesion. Both EV and NF1 show an inherited predisposition to malignancy but the molecular mechanism underlying tumour development is not fully understood. The appearance of both diseases in our patient may be a coincidental association but may also contribute to the identification of loci for susceptibility to NF1 and EV on chromosome 17. ( info)

8/46. CD4 T lymphocytopenia with disseminated HPV.

    BACKGROUND: There have been several reports of HIV-negative patients with chronic idiopathic CD4 T lymphocytopenia, the diagnostic criteria for which are: depressed numbers of circulating T lymphocytes (less than 300/ micro l or less than 20%) on more than one occasion; no laboratory evidence of hiv-1 or hiv-2 infection; and the absence of any defined immunodeficiency or therapy associated with depressed levels of CD4 T lymphocytes. methods: We report a patient with disseminated human papillomavirus infection associated with idiopathic CD4 T-cell lymphocytopenia. A 50-year-old woman presented to the dermatology clinic with a 10-year history of widespread verrucae involving the skin and the cervix. RESULTS: biopsy from the arm revealed a common wart. PCR analysis performed from the paraffin-embedded block was strongly positive for HPV type 2. Other HPV types (including EV-associated HPV 5, 8, 14, 15, 17) were not found. Further laboratory work up revealed T-cell lymphocytopenia, with an absolute CD4 count of 21. HIV tests were repeatedly negative. She was treated with interferon A 8 million units SQ three times per week with partial improvement. The patient underwent a hysterectomy for cervical dysplasia and a vulvectomy for vulvar intraepithelial neoplasia. She developed small-cell lung carcinoma and died. CONCLUSIONS: The diagnosis of idiopathic CD4 T-cell lymphocytopenia should be considered in any patient with widespread viral, fungal, or mycobacterial infection whose HIV test is negative, and appropriate evaluation of the absolute CD4 counts should be performed. ( info)

9/46. epidermodysplasia verruciformis: 14 members of a pedigree with an intriguing squamous cell carcinoma transformation.

    A 38-year-old farmer was apparently well until 5 years of age. At this age, the patient's mother found mildly itchy, raised eruptions over the scalp during combing of the scalp hair. Since then, the eruptions have progressed insidiously to involve the face and other parts of the body, with a preponderance over the face, upper back, and chest, including the palms. The soles, oral cavity, and genital tract were unaffected. The lesions were persistent in nature and showed no remission or exacerbation. Mild to moderate pruritus/discomfort was experienced following sunlight exposure. A prominent, raised eruption appeared on the right side of the forehead 9 years ago, 25 years after the initial eruptions, which in due course ulcerated. It was progressive in nature and acquired a large size. Two years later, it was diagnosed as squamous cell carcinoma, for which liquid nitrogen cryosurgery was performed. There was a recurrence of the lesion at the site of surgery, which was excised 4 months later. Subsequently, there was a sudden flare up at the same site. It was badly infected with maggot infestation. The relentless course of the disease was evident by the appearance of two similar lesions, one on the right half of the nose and the other on the left preauricular region. A short while ago, fatigue and loss of weight were also recorded. Bladder and bowel functions were normal and there was no loss of appetite, hoarseness of voice, or breathlessness. Four of the patient's six children (three sons and one daughter) were reported to have similar lesions all over the body. In addition, nine other relatives were also affected. Accordingly, a total of 14 (12 males and two females) family members were found to be affected from amongst 41 individuals (26 males and 15 females) spread across several generations (Fig. 1). There was a second-degree consanguinity of marriage, with the patient's grandmother and wife's father being brother and sister. skin surface examination was marked by multiple, discrete, flat-topped, scaly, brownish-black papules of diverse morphology, from hypopigmented macules to verrucous lesions, with a few coalescing to form plaques. The scales were brown, dry, and adherent (Fig. 2a). The lesions were conspicuous by their presence over the face, neck, and front and back of the chest. The extremities were also affected. Nevertheless, the soles and genitalia were spared. The oral mucosae, hair, and nails were normal. Koebner's phenomenon was explicit, whereas Auspitz's sign was not demonstrable. In addition, a perceptible nodulo-ulcerative lesion (size, 3 in x 2 in) with indurated, raised, averted margins was encountered on the right side of the forehead. The ulcer was tender and had a fetid discharge. Necrotic slough was prominent over its base. Similar lesions were located on the left preauricular region and right half of the nose. hematoxylin and eosin-stained sections prepared from biopsies taken from representative lesions of epidermodysplasia verruciformis and squamous cell carcinoma revealed their diagnostic features. epidermodysplasia verruciformis showed features of hyperkeratosis and acanthosis with hardly any papillomatosis. Marginal elongation of the rete ridges was present. Extensive vacuolization was a remarkable feature, and was largely confined to the upper stratum Malpighian and the granular cell layer. Some of the vacuolated cells were fairly large; their nuclei were located in the center and had variable pyknosis. The granular cell layer was uniformly thickened, whereas the stratum corneum had a loosely felted (basket-weave) appearance. The dermis was apparently normal (Fig. 2b,c). The other microsection(s) from squamous cell carcinoma were conspicuous by the presence of hyperkeratosis, acanthosis, and irregular masses of epidermal cells, proliferating downwards and invading the dermis. Varying proportions of differentiated squamous cells formed their bulk. These cells were atypical, characterized by variations in size and shape, hyperplasia and hyperchromasia of the nuclei, absence of prickles, chas, characterization of individual cells, and the presence of both mitotic and atypical mitotic figures (Fig. 3b). ( info)

10/46. cimetidine therapy for epidermodysplasia verruciformis.

    A 16-year-old boy with an 8-year history of multiple slightly keratotic papules on his face, trunk, and limbs, is described. On the basis of clinical features, histologic examination of 2 lesions and positivity for HPV 5, a diagnosis of epidermodysplasia verruciformis was made. Treatment with oral cimetidine (40 mg/kg per day) induced a marked improvement after 3 months of therapy, with no relapse at a 6-month follow-up. ( info)
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