Our study presents a rare clinical variant of HVL with predominant periorbital edema. [ncbi.nlm.nih.gov]

Wherever possible, different examples are shown of the same disease, in order to illustrate as wide a variety of presentations as possible. [books.google.com]

Fang Published: 09 August 2016 A 35-year-old female presented with history of relapsing facial rash and fever since 3 years. The facial lesions progressed through small papules or vesicles before healing with atrophic scars. [academic.oup.com]

Eight months prior to hospital admission she presented dermatosis in areas exposed to the sun, characterized by vesicles, papules which evolved to ulcers and necrosis in arms and legs, she also presented with hepatosplenomegaly and facial edema. [scielo.unam.mx]

• Malaise

  All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. [ncbi.nlm.nih.gov]

  Patients with Sézary syndrome may also exhibit general, nonspecific symptoms including fevers, unintended weight loss, chills, fatigue, and malaise. [rarediseases.org]

  Systemic symptoms are present in the vast majority of the patients and include malaise, fever, weight loss, and often lymphadenopathy and hepatosplenomegaly. [plasticsurgerykey.com]

  In some cases malaise, fever or headaches accompany the acute phase. Lesions appear typically on the face and dorsal aspects of the hands. Mucosal involvement in HV is rare. It affects lips and tip of the tongue, both of which can be photoexposed. [clinicaladvisor.com]

  In adolescence, it is associated with a self-limiting infectious mononucleosis syndrome, manifested by fever, pharyngitis, malaise, and atypical lymphocytosis. [jpatholtm.org]

• Disability

  Please enable JS and disable any ad blocker [cancernetwork.com]

  […] overall risk: Age over 60 years Elevated level of serum lactate dehydrogenase - LDH (a type of enzyme) Performance status, i.e. the overall health condition of the individual, which could range from being fully active (low risk) to being completely disabled [dovemed.com]

• Amyloidosis

  B-cell lymphoma/leukaemia, unclassifiable（脾B細胞リンパ腫/白血病-分 類不能群） Waldenstrom's marcroglobulinemia（Waldenstromマクログロブリン血症） ＊Plasama cell neoplasms（形質細胞腫瘍） ＃　Plasma cell myeloma ＃ Solitary plasmacytoma of bone（骨孤立性形質細胞腫） ＃　Extraosseous plasmacytoma ① Primary amyloidosis [med.osaka-cu.ac.jp]

  […] myeloma plasma cell myeloma variants smoldering (asymptomatic) plasma cell myeloma non-secretory myeloma plasma cell leukemia plasmacytoma solitary plasmacytoma of bone extraosseous plasmacytoma monoclonal immunoglobulin deposition diseases primary amyloidosis [radiopaedia.org]

• Infertility

  Some chemotherapy agents can cause infertility in both men and women. There can be permanent damage to the testicles and ovaries, harming their ability to produce sperms or ova. In men, sperm banking can be considered before initiating therapy. [dovemed.com]

• Sore Throat

  (A) This 70-year-old female presented with a sore throat, painful swelling saliva, and tonsillar enlargement with a discrete ulcer. (B) The scanning power view shows a dense infiltrate beneath the ulcer. [jpatholtm.org]

• Photosensitivity

  Primary cutaneous hydroa vacciniforme (HV)-like lymphoma is a rare, Epstein-Barr virus-associated cutaneous neoplasm characterized by photosensitive papulovesicular eruption and usually associated with poor prognosis. [ncbi.nlm.nih.gov]

  “Photosensitivity disorders in children: Part I.”. J Am Acad Dermatol. vol. 67. 2012. Chantorn, R,, Lim, HW,, Shwayder, TA. “Photosensitivity disorders in children: Part II.”. J Am Acad Dermatol. vol. 67. 2012. Choi, D,, Kannan, S,, Lim, HW. [cancertherapyadvisor.com]

  […] photobiology; Evaluation of patients with photodermatologic conditions; Photoimmunology; Photoaging; Photocarcinogenesis; Polymorphous Light Eruption (PMLE); Actinic Prurigo; Hydroa vacciniforme and solar urticaria; Chronic actinic dermatitis; drug-induced photosensitivity [books.google.com]

  There are case reports in the literature which have associated prior cutaneous injuries, with cutaneous lymphoma as determined by photosensitization. [scielo.unam.mx]

  Photodynamic DNA-breaking activity of serum from patients with various photosensitivity dermatoses. Arch Dermatol Res. 1995. 287(6):586-90. [Medline]. Ziering CL, Rabinowitz LG, Esterly NB. [emedicine.medscape.com]

• Cutaneous Manifestation

  Figure 21–1 Figure 21–2 Like other lymphoproliferative disorders, Hodgkin's disease may have both specific and nonspecific cutaneous manifestations. [accesspediatrics.mhmedical.com]

  Other entities with cutaneous manifestations in the B-cell group include lymphomatoid granulomatosis, which can show direct skin involvement or manifest as a ‘paraneoplastic’ lymphohistiocytic panniculitis. [kundoc.com]

  Iwatsuki K, Ohtsuka M, Harada H, Han G, Kaneko F: Clinicopathologic manifestations of Epstein-Barr virus-associated cutaneous lymphoproliferative disorders. Arch Dermatol 1997;133:1081-1086. [karger.com]

  The cutaneous manifestations, including the hydroa vacciniforme-like lesions, have resolved completely. [medicaljournals.se]

  Endogenous: The porphyrias with cutaneous manifestations are the most common cause. These eruptions have characteristic features and are chronic. Table II. [cancertherapyadvisor.com]

• Photophobia

  In patients affected by this condition is very frequent the great ocular compromise given by a walleye, conjunctival congestion, corneal keratopatía, photophobia and cicatricial ectropion which is affecting in a severe way to one of the eyes of the patient [oxyhives.org]

  Eye involvement is also rare, but photophobia, conjunctivitis, corneal infiltration with vascularization and anterior uveitis have been described. [clinicaladvisor.com]

  Case report A 6 year old Indian boy was referred to the Proctor Foundation with a 3 month history of recurrent, self resolving episodes of redness, pain, and photophobia in his right eye associated with a “bump” on his temporal conjunctiva. [bjo.bmj.com]

• Suggestibility

  A long-term follow-up is suggested to be performed to inspect the progression for this tumor. [ncbi.nlm.nih.gov]

  The findings of a retrospective study by Gibson et al. 3 suggest that biopsies that contain CLL-type cells, which lack proliferation centers, in the presence of non-enlarged or slightly enlarged lymph nodes on imaging (< 1.5 cm) represent an indolent [am.asco.org]

  This suggests that EBV1 HV often is monoclonal, regardless of the presence or absence of systemic symptoms. Furthermore, no difference in the number of EBER1 infiltrators among these disorders has been found. [intechopen.com]

  Together, these reports suggest that hydroa vacciniforme may not only progress from a smouldering stage to a lymphoid malignancy, but that it could itself be a lymphoid neoplasm. [bjo.bmj.com]

  Two reports of HV in siblings have been documented, suggesting a genetic component to HV. [7] Some cases have occurred in the setting of hematopoietic malignancy. [emedicine.medscape.com]

• Confusion

  While these advances have increased understanding of skin lymphomas, they have also produced an extensive and sometimes confusing litany of articles, studies, and classification schemes. [books.google.com]

  Cutaneous T-cell lymphoproliferative disorders: approach for the surgical pathologist: recent advances and clarification of confused issues. [e-immunohistochemistry.info]

  At OncoCare Cancer Centre, we sometimes see patients who have received a report and are confused by the cancer names or terminology. [oncocare.sg]

  HVLL can be confused with leishmaniasis, syphilis, tuberculosis, paracoccidioidomycosis, and other deep mycoses such as rhinosporidiosis or mucormycosis. [intechopen.com]

  […] 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 the following may be observed: Confusion [dovemed.com]

• Irritability

  Although the eruption may be present on skin not covered by clothing the affected areas may have been exposed to airborne agents or the patient may have come into contact with an irritating agent or allergen. [cancertherapyadvisor.com]

• Human Herpesvirus 8

  Human herpesvirus 8 (HHV-8)associated peritoneal primary effusion lymphoma (PEL) in two HIV-negative elderly patients. Am J Hematol 2004;76:88–91. 164. Chadburn A, Hyjek E, Mathew S, et al. [kundoc.com]

• Liver Biopsy

  A liver biopsy showed a dense infiltrate of atypical CD56 – /CD3 – /CD8 + and EBV + highly proliferative cells, classified as extranodal EBV-associated NK/T-cell lymphoma ( Fig. S1 ). [medicaljournals.se]

  (A) A 21-yearold man presented with severe oral ulcer, recurrent pneumonia, thrombocytopenia, and elevated liver enzymes for 2 years. Liver biopsy reveals atypical T lymphocytes infiltrating the sinusoidal and hepatic lobules. [jpatholtm.org]

  According to the affected organs, CAEBV can mimic interstitial pneumonia in lung biopsies, myocarditis in heart biopsies and dermatitis in skin biopsies. [frontiersin.org]

  Some cases can have associated hemophagocytic lymphohistiocytosis (HLH), best demonstrated in bone marrow, lymph node and liver biopsies 91,94. Numerous EBV þ cells are seen. [kundoc.com]

treatment and/or systemic treatment, when the whole body is affected In some children, the condition progresses rapidly, while in others the progress is slower. [dovemed.com]

A complete reference to the latest understanding of lymphocytic infiltrates The Cutaneous Lymphoid Proliferations provides a complete, authoritative treatment of the clinical features, phenotypes, pathogenesis, histopathology, diagnosis, and treatment [books.google.com]

Treatment for patients with EBV-associated HV is not satisfactory. [e-ijd.org]

This report presents 2 cases of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 2 and 3 years, respectively. [ncbi.nlm.nih.gov]

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]

The prognosis is poor. [tititudorancea.com]

[…] and Mexico Epidemiology Mainly children and adolescents from Asia, or Native Americans from Central and South America, and Mexico Rare in adults Sites Sun exposed skin, particularly the face (cheeks, nose, lower lip) but also ears and dorsum of hands Etiology [pathologyoutlines.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]

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]

Each entity is described using brief text that summarises its epidemiology, pathogenesis and clinical features. The diagnostic process is then described and illustrated using both clinical and histopathologic images. [books.google.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]

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]

Epstein-Barr virus-associated Hodgkin's disease: epidemiologic characteristics in international data. Int J Cancer 1997 ; 70 : 375 –82. ↵ Zhou X, Hamilton-Dutoit S, Yan Q, et al. [jcp.bmj.com]

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]

These entities often pose diagnostic challenges, both clinically and pathologically, and it is important to understand their unique pathophysiology for correct diagnoses and optimal management. [jpatholtm.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]

Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis. Hematol Am Soc Hematol Educ Program. (2015) 2015:177–82. doi: 10.1182/asheducation-2015.1.177 PubMed Abstract | CrossRef Full Text | Google Scholar 9. [frontiersin.org]

We aim to reduce toxicity, control symptoms, prevent disfigurement, eliminate pain, and emotional distress associated with cancer-directed therapy. [oncocare.sg]

How can Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma be Prevented? Currently, the cause of Hydroa Vacciniforme-like Cutaneous T-Cell Lymphoma is unknown. It may not be hence possible to prevent the tumor from forming. [dovemed.com]

Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. 1995 Jun. 132(6):956-63. [Medline]. Hashizume H, Tokura Y, Oku T, Iwamoto Y, Takigawa M. [emedicine.medscape.com]

Photoprotection may not prevent the skin lesions or systemic symptoms. [4],[9] Immunosuppressive therapy, such as systemic corticosteroids or cyclosporine A has been used, but the disease usually flares after decreasing the dose, as occurred in this reported [e-ijd.org]