1/27. Familial hydroa vacciniforme.hydroa vacciniforme is a rare, idiopathic photodermatosis with an onset in childhood and characterized by acute vesiculation, crusting and scarring following sun exposure. Familial cases are extremely rare with only one previous report. We report a brother and sister, both of whom have developed hydroa vacciniforme.- - - - - - - - - - ranking = 1keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
2/27. The association of latent Epstein-Barr virus infection with hydroa vacciniforme.patients with hydroa vacciniforme (HV)-like eruptions and malignant potential have been reported from asia and mexico, and those patients frequently had an associated latent Epstein-Barr virus (EBV) infection. In order to elucidate the association of latent EBV infection with HV, we studied six children with typical manifestations of HV by detection of EBV genes and EBV-related RNAs in biopsy specimens from cutaneous lesions. Cutaneous lesions of all six children with typical HV contained EBV-encoded small nuclear rna (EBER) cells in 3-10% of the dermal infiltrates, whereas no Bam HI-H, l-fragment (BHLF) mRNA, or transcripts encoding EA-D antigen, were detected. No EBER cells were detected in other inflammatory or benign lymphoproliferative skin disorders tested. polymerase chain reaction amplification confirmed the presence of EBV dna sequences in five of six biopsy specimens from the patients. Latent EBV infection is associated with the development of cutaneous lesions of HV.- - - - - - - - - - ranking = 0.95770090765399keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
3/27. hydroa vacciniforme persistent in a 60-year-old man.hydroa vacciniforme (HV) is an idiopathic photodermatosis with onset in childhood, a chronic-recurrent course, and spontaneous resolution in adolescence or early adulthood. We present a patient with typical HV in whom lesions began in childhood and continued until 60 years of age.- - - - - - - - - - ranking = 0.21149546173006keywords = vacciniforme (Clic here for more details about this article) |
4/27. Late-onset hydroa vacciniforme: two case reports.hydroa vacciniforme (HV) is a rare blistering photodermatosis that heals with vacciniform scarring, with onset usually in childhood and spontaneous resolution by early adulthood. We report two cases of HV seen at the National Skin Centre, singapore, both with a late onset at 20 years of age, during compulsory military service, and one with a less typical non-vesicular presentation, that initially caused some diagnostic difficulties.- - - - - - - - - - ranking = 0.8084598184692keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
5/27. Epstein-Barr virus-associated peripheral T-cell lymphoma in adults with hydroa vacciniforme-like lesions.We describe two Korean adult patients who had necrotizing papulovesicles mainly on their faces. Skin biopsy specimens showed perivascular and periadnexal infiltrate of atypical lymphoid cells with vasculitis in the dermis and subcutaneous tissue. in situ hybridization demonstrated a latent infection of Epstein-Barr virus in the majority of lymphoid cells in the dermis. These patients were diagnosed as having T-cell lymphoma. Interestingly, large granular lymphocytosis was found in the peripheral blood of Case 2.- - - - - - - - - - ranking = 0.76616072612319keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
6/27. hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma.An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the dna extracted from the specimen. A diagnosis of CD8 cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8 primary CTCL with clinical features resembling HV.- - - - - - - - - - ranking = 0.36073655091485keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
7/27. hydroa vacciniforme-like eruptions in a patient with chronic active EB virus infection.We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV dna (PCR) 8 x 10(4). EBV-encoded small nuclear rna (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g dna in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.- - - - - - - - - - ranking = 0.36073655091485keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
8/27. Artificial reproduction of atypical hydroa vacciniforme caused by latent EpStein-Barr virus infection.BACKGROUND: Cases of hydroa vacciniforme-like eruptions caused by latent Epstein-Barr virus (EBV) infection have been reported in asia and mexico, some of which progressed to lymphoproliferative disorders. The precise clinical features of the disease are not, however, clear. methods: We performed an artificial provocation test with repeated exposures of ultraviolet A (UVA) and UVB in an 8-year-old Korean girl who had severe hydroa vacciniforme-like skin eruptions on exposed areas. RESULTS: The patient showed features of latent EBV infection serologically, and an in situ hybridization study indicated that most infiltrating mononuclear cells in the dermis were positive for EBV. After three exposures to UVA irradiation with a total dose of 90 J/cm2, erythema and vesicles appeared on the UVA-irradiated site but not on the UVB-irradiated site. CONCLUSIONS: Atypical hydroa vacciniforme caused by latent EBV infection could be reproduced by repeated UVA irradiation. Further study on the photo-protection would be necessary to prevent its progression to lymphoproliferative malignancies.- - - - - - - - - - ranking = 0.74381691397644keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
9/27. Epstein-Barr virus-associated lymphoproliferative lesions presenting as a hydroa vacciniforme-like eruption: an analysis of six cases.BACKGROUND: There are many reports of patients with a severe hydroa vacciniforme (HV)-like eruption in which cutaneous lesions occur in both sun-exposed and non-exposed areas, unlike in true HV. Several patients have died from a malignant haematological neoplasm. In most cases, a latent Epstein-Barr virus (EBV) infection has been detected in the skin lesions. OBJECTIVES: To describe the clinical and laboratory features of six additional patients with an EBV-associated HV-like eruption. methods: The clinical, histological and immunohistochemical features were reviewed. T-cell receptor gamma gene rearrangements were studied using polymerase chain reaction (PCR) and heteroduplex analysis. In-situ hybridization was performed to detect mRNA for EBV in skin biopsy specimens. PCR was performed to screen for EBV infection in the skin lesions of three patients and blood of two patients. Photoprovocation with repeated ultraviolet (UV) A exposure was performed in three patients. RESULTS: The severity of the skin lesions and the clinical course varied among the patients. Skin lesions were induced by repeated UVA exposure in three patients and a latent EBV infection was demonstrated in the photoprovoked lesions. CONCLUSIONS: Three different clinical courses were found in six patients with an HV-like eruption associated with chronic EBV infection: (i) spontaneous remission; (ii) clearing after photoprotection; and (iii) continuous recurrence irrespective of sun exposure. It is possible that there are two patterns of HV-like eruption associated with chronic EBV infection. One is characterized by recurrent necrotic papulovesicles of the face and the other by nodules and facial swelling. It was demonstrated that the skin lesions could be triggered by repeated UVA exposure in the patients showing recurrent necrotic papulovesicles of the face.- - - - - - - - - - ranking = 0.36074416871623keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
10/27. hydroa vacciniforme with oral mucosal involvement.We report the occurrence of oral ulcers in a 6-year-old girl with hydroa vacciniforme. This finding has not hitherto been reported in the literature.- - - - - - - - - - ranking = 0.36073655091485keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
| | Next -> |