Atypical hydroa vacciniformelike epsteinbarr virus associated tnkcell 48 lymphoproliferative disorder. 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 an overview sciencedirect topics. Hydroa vacciniformelike lymphoma is one of two major types of epsteinbarr associated tcell lymphoproliferative disorders that have been reported in the pediatric age group. Dermatology articles diagnosis, dermatologic surgery. Hydroa vacciniforme may also be known as hydroa aestivale or recurrent summer eruption. Barrionuevo c, anderson vm, zevallosgiampietri e, et al. System ic ebvpositive tcell lymph oproliferative disease of childhood system ic spread and mosquito bite allergy hypersensitivity result in a much. Pathogenic link between hydroa vacciniforme and epsteinbarr virusassociated hematologic disorders. Hydroa vacciniforme with mucosal involvement and recalcitrant periodontitis and multiple virus reactivators after sunexposure.
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. The lesions mainly presented in the sunexposed area. In these cases, the hydroa vacciniforme lesions may spread to. 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. It affects the nasal mucous membrane and is composed of tall columnshaped cells, mucous cells, which have small hairlike structures called cilia. The rash progresses to papules, which undergo vesiculation.
Hydroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Photoaging or photoageing also known as dermatoheliosis is a term used for the characteristic changes to skin induced by chronic uva and uvb exposure 29 tretinoin is the best studied retinoid in the treatment of photoaging the deterioration of biological functions and ability to manage metabolic stress is one of the major consequences of the aging process. The child responded remarkably to oral steroids but relapsed on tapering. Hydroa vacciniforme financial definition of hydroa vacciniforme. Clinicopathological analysis of the hydroa vacciniforme. However, prognostic factors and treatment remain unclear. 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 vacciniforme like lymphoma should be distinguished from a group of tcell and nk cell lymphoproliferative disorders presenting in the skin. 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. Mantle cell lymphoma presenting with exaggerated skin.
They are traditionally divided into hodgkins lymphoma which accounts for about 10% of all lymphomas and nonhodgkin lymphoma, which is the topic of this seminar. Nov 10, 2016 hydroa vacciniforme and hydroa vacciniforme. Hydroa vacciniforme is a rare photodermatosis that typically presents in childhood as vesicles which heal with scarring. 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. The histopathology was consistent with a cellmediated rejection reaction. Hydroa vacciniformelike lymphoproliferative disorder. Because these lymphomas are rare they can be challenging to diagnose for clinicians and pathologists. Ebvassociated tnkcell lymphoproliferative diseases in. Hydroa vacciniforme financial definition of hydroa. 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. The 2008 who classification of lymphoid neoplasms and. Hydroa vacciniforme with unusually severe scar formation. 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. Long wavelength ultraviolet radiation uva is most often implicated.
Aug 05, 2019 hydroa vacciniforme hv is a rare, chronic photodermatosis of unknown origin occurring in childhood. 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. The histopathology and immunohistochemistry were consistent with the diagnosis of hydroa vacciniformelike tcell lymphoma. 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. Systemic ebvpositive tcell lymphoproliferative disease of childhood. Hydroa vacciniformelike t cell lymphoma it is currently unclear whether this is a discrete entity or lies within the spectrum of hydroa vacciniforme. Lymphomas can affect any organ in the body, present with a wide range of symptoms, and be seen by primary care physicians and physicians from most specialties. 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. A number of nk and tcell lymphomas with diverse clinical, histologic, and immunophenotypic features express one or more cytotoxic markers. Here, we present 2 pediatric patients from tibet of china, which is the first report from this area. In some cases, hydroa vacciniforme has been associated with epsteinbarr viral ebv infection, which normally results in glandular fever infectious mononucleosis. Review article epstein barr virusassociated tnatural killer. Goodlad, jonathan said, yasodha natkunam, t and nkcell lymphomas and systemic lymphoproliferative disorders and the immunodeficiency setting. Edited by ieming shih, md, phd division of gynecologic pathology department of pathology johns hopkins medical institutions email.
Hydroa vacciniformelike lymphoma is an epsteinbarr virus positive peripheral tcell lymphoma predominantly affecting asian and hispanic children and young adults. The last two issues of diagnostic histopathology concentrated on neoplasms at sites often deemed to be the territory of the haematopathologist see editorials in the february and march 2010 issues. Hidroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Hydroa vacciniformelike lymphoproliferative disorder hvlpd is a rare epsteinbarr virus ebvassociated lymphoma that mainly affects children. Histopathology showed eosinophilic spongiosis and direct immunofluorescence dif was. 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 vaciniforme hv is a rare photodermatosis that is mainly seen in childhood. 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. Though the pathogenesis is poorly understood, epstein barr virus ebv can be detected in the dermal infiltrate. Hydroa vacciniformelike lymphoma is a proliferation of clonal t cells or less frequently nk cells infected by ebv with a latency type 1. Nov 23, 2010 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 a rare cutaneous manifestation of ebvassociated tnk lpd characterized by blistering photodermatosis in childhood that heals with vacciniforme scarring. Dermatology articles covering symptoms, diagnosis, staging, treatment, prognosis, and followup. Hydroa vacciniformelike lymphoproliferative disorder hvllpd is an epsteinbarr virus ebvassociated disease, usually occurring in east asia 1 x 1 iwatsuki, k. 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 disease has an indolent clinical course with long periods of recurrent skin lesions in sunexposed areas that tend to regress spontaneously. Hydroa vacciniformelike lymphoma in tibetan children. Hydroa vacciniformelike lymphoma shows a marked predilection for the pediatric population, especially in latin america and asia. Hydroa vacciniforme hv is a rare chronic photosensitivity disorder mainly affecting children and characterized by erythema, vesicular papules, bullae, ulcers. Hydroa vacciniforme hv is a rare and chronic pediatric disorder that is characterized by photosensitivity and recurrent vesicles that heal with vacciniforme scarring.
Clinicopathological categorization of hydroa vacciniforme. In canadian women, the upper chest is commonly affected. The 2008 world health organization who classification defines hydroa vacciniformelike lymphoma hvll as a clinicopathological type of cutaneous lymphoma with epsteinbarr virus ebv infection. Most nodal tcell lymphomas belong to the adaptive immune system, whereas many extranodal tcell. 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. Epsteinbarr virusassociated tnatural killercell lymphoproliferative disorders. Peripheral tcell and nkcell lymphomas are functionally, pathologically, and clinically complex. Quality of life assessment and disease experience of patient members of a webbased hydroa vacciniforme support group. The current issue deals with haematopoietic neoplasms that may be seen by a wider range of pathologists, including cutaneous lymphomas and lymphoproliferative disorders. The incidence of hydroa vacciniforme is approximately 0. The cause of hydroa vacciniforme is usually unknown. Epithelial papilloma is one of the more common benign nasal tumours. The current issue deals with haematopoietic neoplasms that may be seen by a wider range of pathologists, including cutaneous lymphomas and lymphoproliferative disorders of the immunosuppressed.
Recurrent vesicles on sunexposed skin that heal with vacciniform or varioliform scarring characterize hv. The histopathology of hydroa vacciniformelike lymphoma can mimic other aggressive cutaneous tcell lymphomas. The 2008 world health organization who classification defines hydroa vacciniforme like lymphoma hvll as a clinicopathological type of cutaneous lymphoma with epsteinbarr virus ebv infection. The 2008 who classification of lymphoid neoplasms and beyond. This is a lymphoproliferative disorder of nk cells and rarely t cells which has overlap features with nktcell lymphoma, nasal type, and is considered by some a variant of that condition. Typical hv is a selflimited skin disease characterized by vesiculopapular eruptions on the sunexposed regions with vacciniforme scarring. Hydroa vacciniforme like lymphoma is a proliferation of clonal t cells or less frequently nk cells infected by ebv with a latency type 1. The infiltrate in the dermis may be denser than seen in hydroa vacciniforme and. Atypical hydroa vacciniforme like epsteinbarr virus associated tnkcell 48 lymphoproliferative disorder.
Geoffrey strutton, in weedons skin pathology third edition, 2010. In current study, we report an adult patient with the tumor. The median overall survival os is less than 2 months. Radiation related disorders of the skin and subcutaneous tissue. Spectrum of t and nk lymphoproliferative disorders in.
Aggressive nkcell leukemia ankl is a rare form of nk cell neoplasm sporadically affecting people from asia and central and south america. Hydroa vacciniforme hv is a very rare, chronic photodermatitistype skin condition with usual onset in childhood. What are the histologic findings of mycosis fungoides in. Pathology outlines hydroa vacciniformelike lymphoma hvl. Histopathology of radiationrelated disorders of the skin and subcutaneous tissue. Nonhodgkin lymphoma represents a wide spectrum of illnesses that vary. Hydroa vacciniformelike lymphoma should be distinguished from a group of tcell and nk cell lymphoproliferative disorders presenting in the skin. We present the case of a 62yearold africanamerican woman with medical history of hypertension and hyperlipidaemia who presented to dermatology clinic for bug bites. Review article epstein barr virusassociated tnatural. The histopathology and immunohistochemistry were consistent with the diagnosis of hydroa vacciniforme like tcell lymphoma. 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. Dyspigmentation, the formation of wrinkles and other symptoms appear around regions of skin commonly exposed to sun, mostly the eyes, mouth and forehead.
Jun 21, 2018 a number of nk and tcell lymphomas with diverse clinical, histologic, and immunophenotypic features express one or more cytotoxic markers. Hydroa vacciniforme definition of hydroa vacciniforme by. With this lymphoma, the lesions are usually larger, fail to resolve and may also involve nonsunexposed sites. Skin examination showed resolving bullae on the shins and postinflammatory pigment changes.
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