Hydroa vacciniformelike lymphoma hvll is an epsteinbarr virus ebvpositive tcell. To report the clinical presentation systemic and ocular of hydroa vacciniforme in a black south african child. Awareness of this occasional manifestation may help to prevent severe complications. A 6year old girl presented with complaint of 3year history of recurrent skin lesions, 3 months of fever accompanied by cough for 8 days. Effective treatment options are scarce and mainly constitute of strict sun protection. Hydroa vacciniforme definition of hydroa vacciniforme by. Deciphering hydroa vacciniforme blood american society of. Clinicopathological categorization of hydroa vacciniformelike. To the internet archive community, time is running out. The lesions mainly presented in the sunexposed area, especially the face. An 18 year old female student reported that since the age of 5 she has been. Pathogenic link between hydroa vacciniforme and epstein. In current study, we report an adult patient with the tumor.
Hydroa vacciniforme and hydroa vacciniforme like tcell lymphoma. First described by bazin in 1855, and later by hutchinson and others, a sufficient number of cases have been reported to. Results of blood and urine porphyrin studies were normal, and no systemic abnormalities were noted. Photosensitivity papulovesicular eruption typical varioliform scarring significantly elevated epsteinbarr viral load in the blood positive phototest responses to uva clinical diagnosis must be made by an experienced photodermatologist able to. Hydroa vacciniforme an overview sciencedirect topics.
Hydroa vacciniforme and hydroa vacciniformelike tcell. Hydroa vacciniformelike lymphoma is an epsteinbarr virus positive peripheral tcell lymphoma predominantly affecting asian and hispanic children and young adults. Early diagnosis and treatment of this disease which is characterized with recurrent vesicles and bullae on areas which are exposed to sun including the face and distal parts of the extremities is important, since it recovers by leaving scar. We present the clinicopathological features of 20 mexican children with hvll with a median age of 8 years at diagnosis range, 115. Multiple exposures to uva reproduced the symptoms and the vesicular and scarring lesions typical of the suninduced disease in our patient. First described by bazin in 1855, and later by hutchinson and others, a sufficient number of cases have been reported to make the disease a well recognized entity. Hydroa vacciniforme is a rare acquired photosensitivity disorder that manifests in childhood.
Hydroa vacciniforme hv is an unusual photosensitivity disease of unknown aetiology which starts in childhood and is characterised by vesiculopapular eruptions on the exposed area of the body. Recurrent vesicles on sunexposed skin that heal with vacciniform or. Hydroa vacciniforme genetic and rare diseases information. Hydroa vacciniforme is one of the rarest forms of photosensitivity disorders of the skin.
May 01, 20 hydroa vacciniforme like lymphoma hvl is a rare type of epsteinbarr virus ebvpositive lymphoma of cytotoxic tcell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme hv. We report a case of hydroa vacciniforme recurred after 3 years period of quiescence in a 20 yearold man who had had history of the disease from the age of. Eligibility statement for hydroa vacciniforme 15083. Pdf virtual slides the virtual slides for this article can be found here.
Hydroa vacciniformelike cutaneous tcell lymphoma in a chil. Though the pathogenesis is poorly understood, epstein barr virus ebv can be detected in the dermal infiltrate. Hydroa vacciniforme like lymphoma hvll is an extremely rare lymphoma described in children that occurs mainly in asia and latin american countries. Nineteen surveys were included in the data analysis. It is currently unclear whether hydroa vacciniforme like t cell lymphoma is a discrete entity or lies at the severe end of the spectrum of hydroa vacciniforme.
In its classic form, hydroa vacciniforme hv is characterized by lightinduced vesicles that. The word vacciniforme is derived from the word vaccinum which means vaccine in latin expresses healing by leaving a scar like the scar of a vaccine 1, 2. Systemic lymphoma arising from hydroa vacciniformelike. Hydroa vacciniforme hv is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. Hydroa vacciniformelike lymphoma is one of two major types of epsteinbarr associated tcell lymphoproliferative disorders that have been reported in the pediatric age group. Hydroa vacciniformelike lymphoma hvl is a rare type of epsteinbarr virus ebvpositive lymphoma of cytotoxic tcell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme hv. Lately, hydroa vacciniforme has been associated with epsteinbarr virus infection. We herein report two patients with hydroa vacciniforme and concomitant ophthalmic involvement, primarily in the form of keratoconjunctivitis leading to corneal opacity. A survey examination of patients with hydroa vacciniforme. Hydroa vacciniforme is associated with increased numbers. The established lesions of hydroa vacciniforme show intraepidermal vesiculation with.
Eb virus infection caebv associated with skin eruptions mimicking hydroa vacciniforme hv in a 4. Hydroa vacciniforme generally begins in childhood and regresses spontaneously after adolescence, but variants that persist into adulthood have been described. The child responded remarkably to oral steroids but relapsed on tapering doses. For language access assistance, contact the ncats public information officer. Hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood. Hydroa vacciniforme is a rare, debilitating photodermatosis of unknown pathogenesis. Recurrent vesicles on sunexposed skin that heal with vacciniform or varioliform scarring characterize hv.
Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase. Clinicopathological analysis of the hydroa vacciniforme. An internati we use cookies to enhance your experience on our website. We analyze clinicopathologic features of seven chinese child patients with hydroa vacciniforme. It is currently unclear whether hydroa vacciniformelike t cell lymphoma is a discrete entity or lies at the severe end of the spectrum of hydroa vacciniforme. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Hydroa vacciniforme is a recurrent vesicular disease that affects lightexposed cutaneous surfaces upon which the ravages of prior episodes are disclosed by scars. Hydroa vacciniforme hv is a very rare, chronic photodermatitistype skin condition with usual onset in childhood. Hydroa vacciniformelike lymphoma hvll is an epsteinbarr virus ebvpositive tcell lymphoproliferative disorder of childhood that occurs mainly in central and south america and asia. Thats right, all we need is the price of a paperback book to sustain a nonprofit library the whole world depends on. Jul 17, 2019 hydroa vacciniforme like lymphoproliferative disorder hvlpd is a cutaneous form of chronic active epsteinbarr virus ebv infection, which occurs mainly in children in latin america and asia.
Oct 31, 20 hydroa vacciniformelike lymphoma hvll is an epsteinbarr virus ebvpositive tcell lymphoproliferative disorder of childhood that occurs mainly in central and south america and asia. Hydroa vacciniformelike lymphoma should be distinguished from a group of tcell and nk cell lymphoproliferative disorders presenting in the skin. Hydroa vacciniforme is a rare, chronic photodermatosis with onset in childhood. Systemic symptoms are absent, and no abnormalities are found on routine laboratory tests. Hydroa vaciniforme is a very rare photodermatosis that is mainly seen in childhood. Barr eb virus infection caebv associated with skin eruptions mimicking hydroa vacciniforme hv in a 4. Hidroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Click on the link to view a sample search on this topic. Hydroa vacciniformelike cutaneous tcell lymphoma in a. Awareness of this occasional manifestation may help to prevent severe. Access to paid content on this site is currently suspended due to excessive activity being detected from your ip address 40. Hydroa vacciniforme hvlike tcell lymphoma is a rare malignancy in childhood associated with epsteinbarr virus infection. The lesions are seen more commonly on surfaces of the skin exposed to light. Coexistence of hydroa vacciniforme and malignant lymphoma.
Table of contents expand submenu for table of contents. Hydroa vacciniforme hv is a rare photodermatosis first described by bazin in the midnineteenth century. The infiltrate in the dermis may be denser than seen in hydroa vacciniforme and. Pdf hydroa vacciniforme hv is a rare and chronic pediatric disorder that is characterized by photosensitivity and. Hydroa vacciniforme like lymphoma should be distinguished from a group of tcell and nk cell lymphoproliferative disorders presenting in the skin. Hydroa vacciniformelike lymphoproliferative disorder hvlpd is a cutaneous form of chronic active epsteinbarr virus ebv infection, which occurs mainly in children in latin america and asia. Clinicopathologic and immunohistochemical study of 12 cases. Hydroa vacciniformelike lymphoma is a rare cutaneous tcell lymphoma associated with latent epsteinbarr virus infection and mainly seen in south american and asian pediatric patients.
The incidence of hydroa vacciniforme is approximately 0. Hydroa vacciniformelike lymphoproliferative disorder hvllpd is an epsteinbarr virus ebvassociated disease, usually occurring in east asia 1 and latin america. Hydroa vacciniforme is an inflammatory summer dermatosis, commencing in early life and characterized by a vesicular, scarring eruption on the exposed parts. This code is effective for cases diagnosed 2010 and later. Hydroa vacciniforme is a disease described in textbooks of dermatology as a recurring vesicular eruption, occurring for the most part in the summer and usually affecting young men. Although this is a scarring photodermatosis, the active lesions often resolve by early adulthood. Hydroa vacciniformelike lymphoproliferative disorder hvlpd is defined as a distinctive clinicopatho logical type of cutaneous lymphoma and a. Hydroa vacciniformelike lymphoma hvll is an extremely rare lymphoma described in children that occurs mainly in asia and latin american countries. Pathogenic link between hydroa vacciniforme and epsteinbarr. In this issue of blood, cohen et al describe a series of hydroa vacciniforme hvlike lymphoproliferative disorder hvlpd cases in whites. Hydroa vacciniforme, bazin, hutchinsons summer eruption.
Hydroa vacciniformelike t cell lymphoma it is currently unclear whether this is a discrete entity or lies within the spectrum of hydroa vacciniforme. We describe 12 additional cases to expand our knowledge of the histopathologic spectrum and the clinical aspects of this rare lymphoma. Ten years after the onset of hydroa vacciniforme hv, a 16yearold boy developed edema and red induration of the face, ears, and dorsa of the hands. The lesions typically heal with vacciniform scarring. The cause of hydroa vacciniforme is usually unknown. Two patients with hydroa vacciniforme, a rare photodermatosis of unknown etiology, demonstrated distinctive scarring and vesiculobullous skin lesions on lightexposed body areas. Typical hv is a selflimited skin disease characterized by vesiculopapular eruptions on the sunexposed regions with vacciniforme scarring. Ocular findings as a presenting sign of hydroa vacciniforme. Hydroa vacciniforme inclusion criteria 29363 all six of the following. The aim of this study was to search for the factors.
In these cases, the hydroa vacciniforme lesions may spread to. We sought to identify the demographics, disease progression, symptoms, risk factors, and treatment efficacy associated with hydroa vacciniforme using a questionnaire. Skin lesions of hydroa vacciniformelike lymphoma on the onset morphology. International agency for research on cancer iarc, 2008. It is an epsteinbarr virus ebvpositive lymphoproliferative disease lpd characterized by a monoclonal proliferation of t or nk cells. Its association with hydroa vacciniforme and pigmentation of the teeth, qjm. However, prognostic factors and treatment remain unclear. Symptoms occurred throughout the year and the most common dermatologic. Hydroa vacciniforme is associated with increased numbers of. Antiviral treatment of a boy with ebvassociated hydroa.
He had freckles on his face and a deep ulcer covered with a crust on his left cheek figure 1. Pubmed is a searchable database of medical literature and lists journal articles that discuss hydroa vacciniforme. We present a patient with hydroa vacciniforme and concomitant previouschronic epsteinbarr virus infection. Hydroa vacciniformelike lymphoproliferative disorder. Haematoporphyria congenita congenital porphyrinuria. By continuing to use our website, you are agreeing to our use of cookies. If you have problems viewing pdf files, download the latest version of adobe reader.
Long wavelength ultraviolet radiation uva is most often implicated in some cases, hydroa vacciniforme has been associated with epsteinbarr viral ebv infection, which normally results in glandular fever infectious mononucleosis. Clinicopathological categorization of hydroa vacciniforme. Chop cyclophosphamide, adriamycin, vincristine, and prednisolone chemotherapy. Aug 05, 2019 hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood. We report a case of hydroa vacciniforme in which an abnormal minimal erythema dose to wavelengths of 322 to 370 nm within the. Deciphering hydroa vacciniforme blood american society. Jun 27, 2019 in this issue of blood, cohen et al describe a series of hydroa vacciniforme hvlike lymphoproliferative disorder hvlpd cases in whites. Hydroa vacciniforme vesicles can be reproduced experimentally with repetitive uva irradiations, but this photoreproduction is not constant. With this lymphoma, the lesions are usually larger, fail to resolve and may also involve nonsunexposed sites. The histopathology and immunohistochemistry were consistent with the diagnosis of hydroa vacciniformelike tcell lymphoma. Systemic ebvpositive tcell lymphoproliferative disease of childhood systemic spread and. The lesions are seen more commonly on surfaces of the skin.
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