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Hydroa Vacciniforme

Hydroa vacciniforme is a rare disorder belonging to the group of photodermatoses and is most often diagnosed in the first decade of life. The recurrent appearance of vesicles and crusts after exposure to the sun and subsequent scarring is the hallmark of this cutaneous disease. The diagnosis rests on clinical criteria and findings on histopathology.


Presentation

Hydroa vacciniforme (HV) is a rare, but potentially debilitating disorder arising exclusively after exposure of the skin to the ultraviolet rays of the sun (termed photodermatoses) [1] [2] [3]. Until recently, the etiology was considered unknown (and thus, HV was classified into the group of idiopathic photodermatoses) [3], but the strong association between Epstein-Barr virus (EBV) described across numerous reports indicates the clear role of this virus in the pathogenesis of hydroa vacciniforme [4] [5] [6] [7]. The prevalence is estimated to be 0.34 per 100,000 individuals, suggesting its rare occurrence in clinical practice [8]. The clinical presentation is distinguished by the development of erythematous lesions, vesicles, papules, and necrotic (sometimes hemorrhagic) crusts in the pediatric population within a few hours after sun exposure [1] [3] [5]. Hence, the most common areas where the typical lesions appear are the face (ears, nose, and cheeks) and the upper extremities (the lower arm and the hands) [3]. The lesions are followed by scarring and depression of the skin at the site of the lesion, as well as hyper and hypopigmentation [3].The course of the disease is chronic, as many patients develop recurrent episodes for a prolonged period of time, although a spontaneous resolution of lesions is usually seen by late teenage years and adolescence[1] [2] [9]. Atypical forms of hydroa vacciniforme have been described in the literature, encompassing similar lesions that develop in non-exposed areas and ocular symptoms (such as conjunctivitis) [4] [7].

Fatigue
  • The children were reported to have less fatigue, fewer eruptions, less scarring, and increased ability to spend time outdoors without provoking new eruptions. This was also in agreement with clinical observations.[ncbi.nlm.nih.gov]
  • After treatment, the patients reported having less fatigue, rashes, and scars. They were able to spend more time outdoors without causing new eruptions.[scielo.br]
Fatigue
  • The children were reported to have less fatigue, fewer eruptions, less scarring, and increased ability to spend time outdoors without provoking new eruptions. This was also in agreement with clinical observations.[ncbi.nlm.nih.gov]
  • After treatment, the patients reported having less fatigue, rashes, and scars. They were able to spend more time outdoors without causing new eruptions.[scielo.br]
High Fever
  • In the same year, he suddenly developed high fever and died from disseminated intravascular coagulation syndrome without any spontaneous regression of the skin lesions.[ncbi.nlm.nih.gov]
  • During acute episodes, systemic findings are present, including high fever, facial swelling, liver damage, and hematologic abnormalities. An association with hypersensitivity to mosquito bites has been described.[visualdx.com]
  • A related cutaneous disorder, mainly described in Japan, is severe mosquito bite allergy. 23 Severe mosquito bite allergy is defined as an EBV NK-cell lymphoproliferation characterized by high fever, ulcers, skin necrosis, and deep scarring after bites[bloodjournal.org]
Intermittent Fever
  • No infectious mononucleosis-like symptoms, such as prolonged or intermittent fever, lymphoadenopathy, or liver damage were evident up till then.[ncbi.nlm.nih.gov]
  • The girl received multiple antibiotics and different kinds of antihistamine drugs in local hospitals but the lesions waxed and waned. 3 months ago she developed intermittent fever (38–39 C).[journals.lww.com]
  • According to the limited reports in the literature, the clinical course may be indolent in patients with the NK-cell phenotype; whereas cases with T-cell phenotype are characterized by intermittent fever and hepatosplenomegaly.[diagnosticpathology.biomedcentral.com]
Chinese Herbal Drugs
  • The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned.[ncbi.nlm.nih.gov]
Saddle Nose
  • Two patients showed saddle nose, and partial absorption of nasal cartilage. One patient displayed cicatricial contracture of lower lip, with incisor extrusion. Four patients showed opacity of cornea.[ncbi.nlm.nih.gov]
Abdominal Pain
  • The most common associated symptoms included oral ulcers, abdominal pain, and eye ulcers. Most participants limited their sun exposure to less than 10 hours weekly and stated sunscreen somewhat helped prevent breakouts.[ncbi.nlm.nih.gov]
  • […] liver (hepatomegaly) There may be associated autoimmune disorders, which can cause joint and muscle pain, heat intolerance, recurrent rashes, abdominal pain, and a general feeling of illness If the brain is involved, then neurological symptoms such as[dovemed.com]
Periodontitis
  • An 8-year-old boy presented with hydroa vacciniforme with recurrent oral mucosal ulcers and treatment-resistant gingivitis/periodontitis.[ncbi.nlm.nih.gov]
  • Hydroa vacciniforme with mucosal involvement and recalcitrant periodontitis and multiple virus re-activators after sun-exposure. Acta Derm Venereol. 2010;90:498-501. [ Links ] 7. Gupta G, Man I, Kemmett D.[scielo.br]
  • "Hydroa vacciniforme with mucosal involvement, recalcitrant periodontitis and multiple virus re-activators after sun exposure". Acta Derm Venereol. vol. 90. 2010. pp. 498-501.[clinicaladvisor.com]
Aphthous Stomatitis
  • Of 63 patients with EBV-related HV and/or hypersensitivity to mosquito bites (HMB), 11 patients (17.5%) presented with mucosal lesions such as aphthous stomatitis and ulcerative gingivitis, 3 patients (4.8%) had ocular symptoms including iritis, conjunctival[ncbi.nlm.nih.gov]
Recurrent Oral Ulceration
  • 1 Adult-onset hydroa vacciniforme-like lymphoma in a long-term resident of the United States. ( 29693057 ) Yu K.K....Tejasvi T. 2018 2 Hydroa Vacciniforme-Like Cutaneous T-Cell Lymphoma in an Adult Presenting With Facial Edema and Recurrent Oral Ulceration[malacards.org]
Thrombosis
  • The vesicle was situated intraepidermally with thrombosis and hemorrhage underneath. This confirmed the diagnosis of HV. In addition, dense cell infiltrate was seen in deep dermis and subcutaneous tissue.[ncbi.nlm.nih.gov]
Anterior Uveitis
  • A 7-year-old Thai boy had several episodes of hydroa vacciniforme which were accompanied by an anterior uveitis with corneal clouding and stellate keratic precipitates.[ncbi.nlm.nih.gov]
  • Twenty three months after his initial presentation, the patient returned with an acute, unilateral, granulomatous, anterior uveitis in the left eye and vesicular crusting of his lips and upper ears.[bjo.bmj.com]
  • Eye involvement is also rare, but photophobia, conjunctivitis, corneal infiltration with vascularization and anterior uveitis have been described.[clinicaladvisor.com]
Corneal Opacity
  • We herein report two patients with hydroa vacciniforme and concomitant ophthalmic involvement, primarily in the form of keratoconjunctivitis leading to corneal opacity.[ncbi.nlm.nih.gov]
  • In severe cases, hand and face deformities and corneal opacity may develop. hy·dro·a vac·ci·ni·for·me ( hī-drō'ă vak-sin-i-fōr'mē ) A recurrent eruption of erythema evolving to umbilicated bullae, occurring on exposure to the sun and affecting chiefly[medical-dictionary.thefreedictionary.com]
Conjunctival Hyperemia
  • hyperemia and corneal erosions, and 2 patients (3.2%) presented with severe HV complicated gastrointestinal involvement.[ncbi.nlm.nih.gov]
Flexion Contracture
  • 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.[ncbi.nlm.nih.gov]
  • 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.[thedoctorsdoctor.com]
Hand Deformity
  • Five patients suffered from hand deformation manifesting rigidity, flexor tetanus, or malposition of the first, second and third interphalangeal joints of hands. Two of the above mentioned patients manifested partial bone absorption of fingers.[ncbi.nlm.nih.gov]
Photosensitivity
  • Thirty-five patients with other photosensitive disorders were included as controls.[ncbi.nlm.nih.gov]
  • Photosensitization is a skin reaction due to the interaction between a photosensitizing substance in the skin and a determinate effective wavelength light radiation.[omicsonline.org]
Vesicular Rash
  • Our patient is a 10-year-old child -without pathological antecedents- who consulted for atrophic depressed scars after a vesicular rash, which begin with a sensation of skin burning a day after intense sun exposure.[panafrican-med-journal.com]
  • rash that heals with crusting and scarring of sun-exposed regions of the skin.[medical-dictionary.thefreedictionary.com]
Facial Swelling
  • A 35-year-old Chinese woman presented with a 2.5-year history of facial swelling in the left lower quadrant and a 10-month history of relapsing red papules and vesicles in the perioral area resembling hydroa vacciniforme.[ncbi.nlm.nih.gov]
  • In most cases signs and symptoms usually start to occur about 30 minutes to 2 hours after sun exposure and present as: Mild burning, itching or stinging Development of tense, swollen bumps ( papules ) and blisters ( vesicles ); there may be facial swelling[dermnetnz.org]
  • During acute episodes, systemic findings are present, including high fever, facial swelling, liver damage, and hematologic abnormalities. An association with hypersensitivity to mosquito bites has been described.[visualdx.com]
  • Swelling and Hydroa Vacciniforme-like Skin Lesions in Epstein-Barr Virus-associated T-cell Lymphoproliferation with Subsequent Development of Lymphoma. ( 27826612 ) Toksoy A....Wobser M. 2016 10 Epstein-Barr virus reactivation is induced, but abortive[malacards.org]
Facial Edema
  • Atypical HV-like eruptions are present on sun-exposed and nonexposed areas with facial edema, fever, and hepatosplenomegaly, unlike classic HV.[ncbi.nlm.nih.gov]
  • edema, arthralgia, lymphadenopathy, hepatosplenomegaly and/or increased liver enzymes.[orpha.net]
  • 1 Adult-onset hydroa vacciniforme-like lymphoma in a long-term resident of the United States. ( 29693057 ) Yu K.K....Tejasvi T. 2018 2 Hydroa Vacciniforme-Like Cutaneous T-Cell Lymphoma in an Adult Presenting With Facial Edema and Recurrent Oral Ulceration[malacards.org]
  • Medical history and reports in this study had been approved by the patient's parents. 3 Discussion HV-like lymphoma has some common features including facial edema, recurrent papulovesicles that generally proceed to necrosis and vacciniforme scars, before[journals.lww.com]
  • Clinically, patients present with facial edema and recurrent vesiculopapular rash followed by ulceration and crusting.[bloodjournal.org]
Headache
  • Aggravation of cutaneous manifestations was associated with general malaise, headache, fever, lymphadenopathy, hepatosplenomegaly, and an increase of several serum enzymes.[ncbi.nlm.nih.gov]
  • Depending on the case, it may be accompanied by fever, headache and gastrointestinal symptoms.[scielo.br]
  • […] in some individuals The following general signs and symptoms of Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma may be observed, if various body systems and organs are affected: Unintentional weight loss; changes in appetite Fatigue and weakness, headache[dovemed.com]
Seizure
  • We report a Korean boy with an atypical HV-like eruption and various systemic manifestations, including fever, sore throat, abdominal pain, headaches, seizures, and hematologic abnormalities for 2 years.[ncbi.nlm.nih.gov]

Workup

Despite the benign and self-limiting course of the disease, hydroa vacciniforme can severely impair the quality of life, especially in the absence of an early diagnosis. Since numerous photodermatoses exist, a thorough clinical and laboratory workup should be performed in all patients with sun exposure-induced skin lesions. A detailed patient history is useful in determining whether certain preexisting conditions (eg. systemic lupus erythematosus, allergies, dermatomyositis, pellagra, etc.) may be the underlying cause [3], whereas the course and progression of symptoms (and confirmation of their recurrence after sun exposure) are vital information for raising clinical suspicion. A meticulous physical examination of the skin is equally important, as it can further identify the distribution of the lesions and their characteristics. Laboratory studies usually yield no pathological findings in the case of hydroa vacciniforme [2], and because porphyrias (both hepatic and erythropoietic) have a strikingly similar clinical presentation to hydroa vacciniforme, urine and blood levels of porphyrins must be evaluated [3]. Some reports have assessed the presence of EBV through detection of its genetic material by polymerase chain reaction (PCR) testing [4] [5]. To make a definite diagnosis, a sample of the lesion through a biopsy should be obtained, showing necrosis of the epidermis and intraepidermal vesiculation [1].

Human Parvovirus B19
  • Notably, human parvovirus B19 DNA was detected in both skin and gingival lesions. Impairment of systemic immunity was not detected.[ncbi.nlm.nih.gov]

Treatment

  • Most participants had no to limited improvement of their symptoms with treatments.[ncbi.nlm.nih.gov]

Prognosis

  • Its diagnosis is based on monoclonal T-cell proliferation; however, its degree of malignancy is controversial owing to its variable prognosis. Elderly-onset cases of these diseases are extremely rare, and the clinical features remain unknown.[ncbi.nlm.nih.gov]
  • Prognosis - Hydroa vacciniforme Not supplied. Treatment - Hydroa vacciniforme Not supplied. Resources - Hydroa vacciniforme Not supplied.[checkorphan.org]
  • The prognosis of the condition is poor; only 1 in 3 survive after 2 years of diagnosis The prognosis depends upon a set of several factors, which include: Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis[dovemed.com]

Etiology

  • From Wikidata Jump to navigation Jump to search A rare skin disorder of unknown etiology affecting children.[wikidata.org]
  • Two patients with hydroa vacciniforme, a rare photodermatosis of unknown etiology, demonstrated distinctive scarring and vesiculobullous skin lesions on light-exposed body areas.[ncbi.nlm.nih.gov]
  • The etiology is unknown.[skincareguide.ca]
  • Until recently, the etiology was considered unknown (and thus, HV was classified into the group of idiopathic photodermatoses), but the strong association between Epstein-Barr virus (EBV) described across numerous reports indicates the clear role of this[symptoma.com]
  • The etiology of the condition is still unknown; there is theory of an abnormal sensitivity to the UVA (Ultraviolet rays type A) radiation in a range of 320 to 390 nm of wavelength.[oxyhives.org]

Epidemiology

  • Occurs in children and associated with sun sensitivity ( WHO 2008 ) Increased frequency in Asians and Native Americans from Central and South America, and Mexico Epidemiology Mainly children and adolescents from Asia, or Native Americans from Central[pathologyoutlines.com]
  • Papulovesicular eruption resulting in ulceration and scarring Wasting Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Progression and Transformation Recurrent skin lesion s for 10-15 years before progression to system ic involvement Epidemiology[seer.cancer.gov]
  • ULTRAVIOLET AND VISIBLE RADIATION THERAPY 319 Chapter 23 UltravioletA1 and Visible Light Therapy 335 Chapter 24 Psoralen Photochemotherapy 347 Chapter 25 Extracorporeal Photochemotherapy Photopheresis 359 Chapter 26 Photodynamic Therapy 369 Chapter 9 The Epidemiology[books.google.com]
  • Pipelzadeh MH, Jalali A, Taraz M, Pourabbas R, Zaremirakabadi A (2007) An epidemiological and a clinical study on scorpionism by the Iranian scorpion Hemiscorpius lepturus. Toxicon 50: 984-992.[omicsonline.org]
  • […] type Mycosis fungoides Precursor T-cell lymphoblastic lymphoma Peripheral T-cell lymphoma, not otherwise characterized Primary cutaneous anaplastic large cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Lupus [10] Herpes simplex [11] [1] Epidemiology[wikidoc.org]
Sex distribution
Age distribution

Pathophysiology

  • Part 1: pathophysiology and technical aspects of photobiologic studies. Actas Dermosifiliogr 105: 112-121.[omicsonline.org]
  • Historical Perspective HVLL reports date back to 1986; [5] however, it is not until 2008 that the WHO recognized HVLL as a separate entity from Hydroa vacciniforme and classified it under "EBV positive T-cell lymphoproliferative disorders of childhood". [2] Pathophysiology[wikidoc.org]
  • Pathophysiology The etiology of hydroa vacciniforme (HV) is uncertain. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption.[emedicine.medscape.com]
  • Etiology Pathophysiology The pathogenesis of HV is not fully understood. Sunlight is required to provoke eruption, and in the temperate climate the onset is usually in spring or summer.[clinicaladvisor.com]

Prevention

  • OBJECTIVE: To describe a severe type of hydroa vacciniforme (HV) in order to provide basis for treatment and prevention of this disease.[ncbi.nlm.nih.gov]

References

Article

  1. Park H-Y, Park J-H, Lee K-T, et al. A Case of Hydroa Vacciniforme. Ann Dermatol. 2010;22(3):312-315.
  2. De Almeida HL, Sartori DS, Kopp J, Velloso CD, Jorge VM. Extensive hydroa vacciniforme. An Bras Dermatol. 2013;88(4):620-622.
  3. Lehmann P, Schwarz T. Photodermatoses: Diagnosis and Treatment. Dtsch Arztebl Int. 2011;108(9):135-141.
  4. Huh SY, Choi M, Cho KH. A Case of Epstein-Barr Virus-associated Hydroa Vacciniforme. Ann Dermatol. 2009;21(2):209-212.
  5. Jung S-E, Cho KH, Lee MW, Kim YC. Hydroa Vacciniforme-Like Eruption Associated with Epstein-Barr Virus Infection in an Older Adult. Ann Dermatol. 2015;27(6):789-791.
  6. Lysell J, Wiegleb Edström D, Linde A, et al. Antiviral therapy in children with hydroa vacciniforme. Acta Derm Venereol. 2009;89(4):393-397.
  7. Jeng BH, Margolis TP, Chandra NS, McCalmont TH. Ocular findings as a presenting sign of hydroa vacciniforme. Br J Ophthalmol. 2004;88(11):1478-1479.
  8. Gupta G, Man I, Kemmett D. Hydroa vacciniforme: a clinical and follow-up study of 17 cases. J Am Acad Dermatol. 2000;42:208–213.
  9. Balestri R, Neri I. Hydroa vacciniforme. CMAJ : Canadian Medical Association Journal. 2010;182(17):E796.

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Last updated: 2019-07-11 21:18