The patient improved with use of high protection sunscreens and per os hydroxychloroquine 200 mgday. A rare skin disorder characterized the development of crusting skin eruptions following exposure to the sun. The histopathology and immunohistochemistry were consistent with the diagnosis of hydroa vacciniforme like tcell lymphoma. Hydroa vacciniforme hv, also termed as bazins hydroa vacciniforme, refers to an occasional yet chronic skin condition of photodermatosis that begins hydroa vacciniforme it normally is found in children below 10 years of age, characterized by recurrent vesicles on sunexposed areas of the skin that can be treated with vacciniform or. System ic spread and mosquito bite allergy hypersensitivity result in. We herein report two patients with hydroa vacciniforme and concomitant ophthalmic involvement, primarily in the form of keratoconjunctivitis leading to corneal opacity.
Ocular findings as a presenting sign of hydroa vacciniforme. Others are the debilitating actinic prurigo, a prolonged ple, and hydroa vacciniforme, very possibly a scarring ple, chronic actinic dermatitis, a severe contact dermatitis. Jun 17, 2011 va005 clinical outcomes of vigiv treatment of smallpox vaccination complications or vaccinia infection the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. It affects sun exposed skin and is characterised by recurrent fluidfilled blisters hydroa that heal with pox like vacciniform scars. The infiltrate in the dermis may be denser than seen in hydroa vacciniforme and. Click on the link to view a sample search on this topic.
More detailed information about the symptoms, causes, and treatments of hydroa vacciniforme is available below. Long wavelength ultraviolet radiation uva is most often implicated. Pathology outlines hydroa vacciniformelike lymphoma hvl. The lesions are seen more commonly on surfaces of the skin exposed to light. Hydroa vacciniformelike lymphoma is an epsteinbarr virus positive peripheral tcell lymphoma predominantly affecting asian and hispanic children and young adults. Hydroa vacciniforme financial definition of hydroa vacciniforme. Pdf an unusual case of hydroa vacciniforme with ocular. Hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood that exists within the spectrum of epsteinbarr virusrelated lymphoproliferative disorders. Hydroa vacciniforme is a rare photodermatosis that typically presents in childhood as vesicles which heal with scarring. Hydroa vacciniforme is one of the rarest forms of photosensitivity dermatoses. An 8yearold boy presented with hydroa vacciniforme with recurrent oral mucosal ulcers and treatmentresistant gingivitisperiodontitis. Results of blood and urine porphyrin studies were normal, and no systemic abnormalities were noted. Hydroa vacciniforme hv is a very rare, chronic photodermatitistype skin condition with usual onset in childhood. A rare case of hydroa vacciniformelike cutaneous tcell lym.
Hydroa vacciniforme like lymphoma hvll is an extremely rare lymphoma described in children that occurs mainly in asia and latin american countries. T cells article pdf available in journal of investigative dermatology 25. Quality of life assessment and disease experience of patient members of a webbased hydroa vacciniforme support group. Pdf hydroa vacciniforme is associated with increased. Clinicopathological categorization of hydroa vacciniforme. We present the clinicopathological features of 20 mexican children with hvll with a median age of 8 years at diagnosis range, 115. Hydroa vacciniforme is a rare, chronic photodermatosis with onset in childhood that is characterized by recurrent vesiculation, necrosis, and vacciniform scarring limited to sunexposed skin. Hydroa vacciniforme hv is a very rare, idiopathic photodermatosis that mainly starts in childhood, frequently resolving by adolescence or young adulthood. Though the pathogenesis is poorly understood, epstein barr virus ebv can be detected in the dermal infiltrate. Staybred vl5 is for vaccination of healthy cows and heifers as an aid in preventing campylobacteriosis vibriosis caused by campylobacter fetus. The hydroa vacciniforme is an entity with clinical and histological characteristics that are very specific and which are useful to develop the diagnosis of the condition. Hydroa vacciniformelike lymphoma is one of two major types of epsteinbarr ebv associated tcell lymphoproliferative disorder s that have been reported in the pediatric age group.
Hydroa vacciniformelike t cell lymphoma it is currently unclear whether this is a discrete entity or lies within the spectrum of hydroa vacciniforme. Va005 clinical outcomes of vigiv treatment of smallpox. The rash progresses to papules, which undergo vesiculation. Cutaneous photobiology rooks textbook of dermatology. The disease which was described by bazin in 1862 for the first time is characterized with bullae and vesicles recurring on the areas which are exposed to sun and its typical characteristic is to leave scars similar to varicella. Hydroa vacciniforme like lymphoma is one of two major types of epsteinbarr associated tcell lymphoproliferative disorders that have been reported in the pediatric age group. Lesions are symmetrically localized in photoexposed areas. 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. Hydroa vacciniforme is a very rare chronic, scarring, photo induced disorder initially seen in childhood and resolves during puberty. Blisters, bullae and papules healing with scarring appeared on light. Hydroa vacciniforme like lymphoma hvll is an ebvpositive peripheral tcell lymphoma nonhodgkins lymphoma, that affects primarily pediatric patients and has a bad prognosis. Atypical hydroa vacciniforme like epsteinbarr virus associated tnkcell 48 lymphoproliferative disorder.
However, prognostic factors and treatment remain unclear. Received october 22, 2008, revise d january 22, 2009, accepted for. Hydroa vacciniforme is a recurring vesicular eruption upon the exposed portions of the skin, excited by extremes of temperature, and which, upon its disappearance, leaves scars. Hydroa vacciniforme is commonly associated with reactivation of a latent epsteinbarr virus ebv formerly acquired by an asymptomatic or infectious mononucleosiscausing infection. Hydroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Chop cyclophosphamide, adriamycin, vincristine, and prednisolone chemotherapy was initiated. Hydroa vacciniforme like lymphoma hvll is an epsteinbarr virus ebvpositive tcell lymphoproliferative disorder of childhood that occurs mainly in central and south america and asia. Hydroa vacciniforme with unusually severe scar formation. Hydroa vacciniforme can often be diagnosed from the patients or parents description and by examination of the skin or photographs of the rash. Systemic symptoms are absent, and no abnormalities are found. Hydroa vacciniforme with mucosal involvement and recalcitrant periodontitis and multiple virus reactivators after sunexposure.
Hydroa vacciniformelike lymphoma hvll is an extremely rare lymphoma described in children that occurs mainly in asia and latin american countries. Deciphering hydroa vacciniforme blood american society. Skin biopsy revealed a hvlike lymphoma presentation and positive signals. Hydroa vacciniforme hvlike tcell lymphoma is a rare malignancy in childhood associated with epsteinbarr virus infection. With this lymphoma, the lesions are usually larger, fail to resolve and may also involve nonsunexposed sites. Hydroa vacciniforme may also be known as hydroa aestivale or recurrent summer eruption. Is it easy to diagnose on clinical and photobiologicai data. Recurrent vesicles on sunexposed skin that heal with vacciniform or varioliform scarring characterize hv.
Hydroa vacciniforme like lymphoma should be distinguished from a group of tcell and nk cell lymphoproliferative disorders presenting in the skin. Systemic lymphoma arising from hydroa vacciniformelike. 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. Marked increase of circulating doublenegative t cells. Hvll is associated with photosensitivity and insect bites. We report a case of nonvesicular hydroa vacciniforme in which only extensive crusting associated with hypertrophic scarring on sunexposed skin was present. Treatment with hydroxychloroquine sulfate, but not indomethacin, reduced the. 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. Aug 05, 2019 hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood that exists within the spectrum of epsteinbarr virusrelated lymphoproliferative disorders. The prevalence of this entity is sporadic although there have been reported cases of entire families affected and even of fraternal twins affected by it.
Epsteinbarr virusassociated tnatural killercell lymphoproliferative disorders. The lesions typically heal with vacciniform scarring. Diagnosis by repetitive uva phototesting seung kyung hann, md,a sungbin 1m, md,b yoonkee park, md,b and sungnack lee, mdb kangwondo, korea hydroa vacciniforme is a rare, chronic, photosensitive disorder manifested in childhood by recurrent vesicles that heal with scarring. Learn about aepox vac canada for animal usage including. Multiple exposures to uva reproduced the symptoms and the vesicular and scarring lesions typical of the suninduced disease in our patient. Awareness of this occasional manifestation may help to prevent severe complications.
Jaschke e, honigsmann h 1981 hydroa vacciniformeaktionsspektrum. Eligibility statement for hydroa vacciniforme 15083. Pubmed is a searchable database of medical literature and lists journal articles that discuss hydroa vacciniforme. Hydroa vacciniforme definition of hydroa vacciniforme by. In some cases, hydroa vacciniforme has been associated with epsteinbarr viral ebv infection, which normally results in glandular fever infectious mononucleosis. Hydroa vacciniforme hv is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. Symptoms of oral mucosal involvement and gingivitisperiodontitis mirrored the severity of the skin lesions in sunexposed areas.
If there is uncertainty about the diagnosis, your dermatologist may suggest blood tests and perhaps special tests photo testingskin biopsy. Multiple exposures to uva reproduced the symptoms and the vesicular and scarring lesions typical of the suninduced. Three patients demonstrated low minimal erythema doses meds following monochromatic ultraviolet uva irradiation of back skin. Pathogenic link between hydroa vacciniforme and epstein. Pdf antiviral therapy in children with hydroa vacciniforme. The incidence of hydroa vacciniforme is approximately 0. Hydroa vacciniforme like lymphoproliferative disorder hvlpd is a rare epsteinbarr virus ebvassociated lymphoma that mainly affects children.
Hydroa vacciniforme symptoms, diagnosis, treatments and. 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. The characteristic clinical sign of hvll is papulovesicular eruption and subsequent ulcerationscarring primarily on the face and upper extremities 1. It is characterized by necrohemorrhagic lesions that appear on uncovered areas of skin, which became crusted then gradually heal leaving varioliform scars, hence the denomination. Hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood. Due to its many clinical appearances, it is named polymorphic or. Il virus di epsteinbarr potrebbe essere implicato nella patogenesi della malattia dal momento che e stato riscontrato in forma latente nellinfiltrato infiammatorio del derma. Hydroa vacciniforme genetic and rare diseases information. Case report article pdf available in european journal of pediatric dermatology 194. Pdf hidroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Hydroa vacciniforme hv is a rare photodermatosis first described by bazin 1 in 1862.
The child responded remarkably to oral steroids but relapsed on tapering doses. Hydroa vacciniforme is a rare, chronic photodermatosis with onset in childhood. It is an epsteinbarr virus ebvpositive lymphoproliferative disease lpd characterized by a monoclonal proliferation of t or nk cells. Hydroa vacciniforme is a rare idiopathic photodermatosis characterized by an itchy and stinging sensation followed by the appearance of an erythematous rash within a few hours after exposure to sun. Hydroa vacciniforme is associated with increased numbers of epsteinbarr virusinfected. Polymorphous light eruption ple, sometimes also called polymorphic light eruption pmle, is a nonlifethreatening and potentially distressing skin condition that is triggered by sunlight and artificial uv exposure in a genetically susceptible person, particularly in temperate climates during the spring and early summer. Hydroa vacciniforme inclusion criteria 29363 all six of the following. The exact cause of this disorder is unknown, but it is thought to be induced by uvb radiation. In all cases of doubt the diagnosis can be con firmed by the examination of fluid from one of the bullaelig or vesicles. An analysis of the number of ebvterminal repeats showed that the snk cell clones had monoclonal ebv genomes identical to the original ebvinfected cells of the respective patients, and snk cells had the type ii latency of ebv infection, suggesting that not only the cell clones isolated from nasal tnkcell. The neoplastic cells of hvll are usually t cells and natural killer nk cells.
Fattori scatenanti sono lesposizione a sorgenti di uva sia naturali luce solare che artificiali lettini solari. Here are links to possibly useful sources of information about hydroa vacciniforme. The cause of hydroa vacciniforme is usually unknown. Hydroa vacciniformelike cutaneous tcell lymphoma in a. To examine the similarities and differences in the clinical pathological features, ebv infection status, and gene rearrangements in adults and children patients with hvlpd. Hydroa vacciniformelike lymphoma hvll is a disorder of childhood that is associated with epsteinbarr virus ebv. 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. Typical hv is a selflimited skin disease characterized by vesiculopapular eruptions on the sunexposed regions with vacciniforme scarring. An unusual case of hydroa vacciniforme with ocular involvement. Hydroa vacciniforme is associated with increased numbers. Two patients with hydroa vacciniforme, a rare photodermatosis of unknown etiology, demonstrated distinctive scarring and vesiculobullous skin lesions on lightexposed body areas. A case of a young female patient with hydroa vacciniforme is reported. Hydroa vacciniforme hv is a rare photosensitivity disorder of childhood characterized by vesiculopapules on sunexposed areas, including the face and dorsal surfaces of the hands, which resolves by early adult life.
System ic ebvpositive tcell lymph oproliferative disease of childhood system ic spread and mosquito bite allergy hypersensitivity result in a much. Hydroa vaciniforme hv is a rare photodermatosis that is mainly seen in childhood. Although the disease is usually sporadic, familial cases have been reported in identical twins and siblings. Treatment was unsatisfactory, although spontaneous improvement in the condition tended to occur and regular application of sunscreens with high protection. Hydroa vacciniforme hv is a rare, acquired and chronic paediatric disorder that is characterized by photosensitivity and recurrent crops of skin lesions on sunexposed skin, such as the face, ears and hands that heal with vacciniforme. 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. Hydroa vacciniforme, photodermatosis, phototest introduction hydroa vacciniforme hv is a very rare photodermatosis of unknown etiology that is principally seen in childhood. It is characterized by recurrent erythema and crops of papulovesicles or vesicles that appear on uncovered areas of skin within 1 to 2 days after sun exposure. A 6year old girl presented with complaint of 3year history of recurrent skin lesions, 3 months of fever accompanied by cough for 8 days. Hydroa vacciniforme hydroa vacciniforme is a rare, chronic photodermatosis, characterized by deepseated vesicles on sunexposed skin, primarily the face and ears, that become necrotic and heal with varioliform scars.
522 1600 848 148 1650 349 1003 1400 1294 70 109 932 377 5 1607 1146 922 1478 1433 1189 193 987 850 753 166 1044 1645 140 962 864 94 294 238 1004 105 339 430 572 640 640