1/9. 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 = 1keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
2/9. 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 = 0.44384432652132keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
3/9. 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 = 0.44384432652132keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
4/9. 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 = 0.49069173222969keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
5/9. hydroa vacciniforme with unusually severe scar formation: diagnosis by repetitive UVA phototesting.hydroa vacciniforme is a rare, chronic, photosensitive disorder manifested in childhood by recurrent vesicles that heal with scarring. reproduction of vesicles with repetitive UVA phototesting may be an important diagnostic aid. Recurrent eruption resulted in severe scarring of the face and a flexion contracture of the finger in our patient. The clinical features and laboratory evaluation of hydroa vacciniforme are reviewed.- - - - - - - - - - ranking = 0.30247834120907keywords = vacciniforme, hydroa (Clic here for more details about this article) |
6/9. hydroa vacciniforme-like lymphomatoid papulosis in a Japanese child: a new subset.An 8-year-old Japanese girl had a 9-month history of a self-healing papulovesicular eruption on her face, scalp, and neck that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of small lymphocytic cells and scattered large atypical cells expressing CD30. Study of gene rearrangement showed no monoclonality in the infiltrating cells. To our knowledge, this is the second case of lymphomatoid papulosis with clinical features resembling HV. However, we also found descriptions in the literature of two other Japanese children with malignant lymphoma who both initially had clinical features resembling HV. These findings suggest that these cases of HV-like disease constitute a subset of lymphomatoid papulosis that is highly likely to progress to malignant lymphoma.- - - - - - - - - - ranking = 0.44384432652132keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
7/9. hydroa vacciniforme.hydroa vacciniforme is a rare chronic photodermatosis with onset in childhood. It is characterized by recurrent vesicles healing with scars. We report a typical case of a young, 8-year-old boy showing favorable evolution under treatment with chloroquine and a topical sunscreen.- - - - - - - - - - ranking = 0.24230540815164keywords = vacciniforme (Clic here for more details about this article) |
8/9. A case of hydroa vacciniforme with unusual ear mutilation.A 22 year-old man visited our department with a 18-year-history of recurrent vesicular eruption on his skin when exposed to the sun. history revealed that the skin lesions developed as vesicles at first, then over the next several days, they formed crusts and healed with scarring. We were able to induce skin lesions by a repetitive UV-A provocation test. By the clinical and histologic features of the induced lesions, the case was diagnosed as hydroa vacciniforme (HV). However, no vesicular lesions were found on physical examination. Instead, in addition to varioliform scarring, we found various unusual clinical manifestations: burn-like lesions and crusts, flexion contracture of the digitum, and ear lobe mutilation. The ear lobe mutilation, which had not been reported previously in HV, was especially interesting.- - - - - - - - - - ranking = 1.25keywords = hydroa vacciniforme, vacciniforme, hydroa (Clic here for more details about this article) |
9/9. hydroa vacciniforme presenting in an adult successfully treated with cyclosporin A.hydroa vacciniforme (HV) is a rare photodermatosis first described by Bazin in the mid-nineteenth century. It typically presents in childhood and resolves during adolescence. Rarely, however, symptoms may persist into adult life.2,3 We now describe a patient with this condition presenting unusually for the first time at the age of 58 years and responding to treatment with cyclosporin A.- - - - - - - - - - ranking = 0.24230540815164keywords = vacciniforme (Clic here for more details about this article) |