Primary cutaneous Epstein-Barr virus–related lymphoproliferative disorders in 4 immunosuppressed children

Wallet-Faber, N.; Bodemer, Christine; Blanche, Stéphane; Delabesse, Éric; Eschard, C.; Brousse, Nicole; Fraïtag, Sylvie

doi:10.1016/j.jaad.2007.08.013

Cited by 25 publications

(13 citation statements)

References 24 publications

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“…Moreover, some patients showed spontaneous partial involvement. 14,15 Of the handful of cases of SPTCL described in pediatric patients, most were treated with multidrug chemotherapy regimens with variable responses and durations of remission. In our patient, lupus panniculitis was easily excluded by the many features observed in the biopsy including medium as well as small lymphocytes, more abundant karyorrhectic debris, and by a cytotoxic immunophenotype.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous Panniculitis-like T-Cell Clonal Proliferation in an Infant. Lymphoma or Reactive Lymphoproliferation?

Fraïtag¹,

Bader‐Meunier²,

Brousse³

et al. 2014

Pathology Case Reviews

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Subcutaneous panniculitis-like T-cell clonal proliferation is commonly seen as a cytotoxic subcutaneous lymphoma arising mainly in adults. This type of lymphoid proliferation in children, particularly in infants, is extremely rare. In most such cases, the prognosis is excellent even if it is associated with a hemophagocytic syndrome (HPS). Some cases may even regress spontaneously. The case presented here concerns a 6-month-old infant who presented with panniculitis lesions associated with an HPS that started 1 week after a chickenpox infection. The histologic findings showed a marked lobular panniculitis with extensive hyaline necrosis of adipocytes and massive infiltration of clonal small-to-medium lymphocytes with a cytotoxic, CD56 − , β-F1 + phenotype. In addition, the same lymphocytic proliferation was also observed in the upper dermis and the epidermis. Complete and stable remission of both cutaneous lesions and systemic manifestations was obtained with nonaggressive therapy consisting of systemic steroids followed by cyclosporin A. This observation demonstrates that in a young child, a clonal T-cell lymphoproliferation in the skin, even when it has an aggressive histologic appearance and is combined with an HPS, need to not be managed aggressively with chemotherapy. Because the disorder began a few days after a viral infection, it should be seen as a reactive clonal T-cell lymphoproliferation rather than a true T-cell lymphoma.

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Section: Discussionmentioning

confidence: 99%

Subcutaneous Panniculitis-like T-Cell Clonal Proliferation in an Infant. Lymphoma or Reactive Lymphoproliferation?

Fraïtag¹,

Bader‐Meunier²,

Brousse³

et al. 2014

Pathology Case Reviews

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“…Besides iatrogenic-induced or infection-related immunosuppression, cutaneous lymphomas may arise in the setting of congenital immunodeficiency disorders such as Wiskott-Aldrich syndrome or ataxia-telangiectasia, among many others [1,12]. The most frequent lymphoma type observed in this group of patients is diffuse large B-cell lymphoma, and clinicopathologic features are similar to those observed in immunocompetent hosts.…”

Section: Cutaneous Lymphoproliferative Disorders In Other Iatrogenic mentioning

confidence: 99%

Cutaneous Lymphoproliferative Disorders in Other Iatrogenic and Non‐Iatrogenic Immune Deficiencies

2014

Skin Lymphoma

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“…Sirolimus has been shown to be a useful treatment for Kaposi's sarcoma; whether its use as an immunosuppressive agent will alter the rates of Kaposi's sarcoma in transplant recipients remains to be seen [ 30 ]. Primary cutaneous Epstein-Barr virus-related lymphoproliferative disorders are rare; a recent case series described four cases in immunocompromised children, in which two of the lymphoproliferative disorders were B cell derived and two were T cell derived (the T cell-derived disorders were Epstein-Barr virus positive, which is extremely rare); three patients died after developing extracutaneous disease [ 31 ]. Recent elegant work showed that a previously undescribed polyomavirus may be the cause of Merkel cell carcinoma, a rare skin cancer more common in immunosuppressed hosts [ 32• ].…”

Section: Viral Skin and Soft Tissue Infectionsmentioning

confidence: 99%

Skin and soft tissue infections in the transplant population

Kotton

2008

Curr Infect Dis Rep

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Solid organ and hematopoietic stem cell transplant recipients are more likely to develop skin and soft tissue infections, which may be caused by common or atypical pathogens. Skin and soft tissue anatomic abnormalities may act as portals of entry for infection, and may result from surgery, venous access, immunosuppressive medications, and other etiologies. Systemic infection may sometimes be recognized by investigation of skin manifestations. Many noninfectious processes of the skin and soft tissues can mimic infection, complicating the diagnosis. Prompt dermatologic consultation and skin biopsy are essential in this vulnerable population. Treatment of infection in transplant recipients may necessitate reduction of immunosuppression, in addition to other adjunctive therapies. Interesting and important new findings about skin and soft tissue infections in transplant recipients are the focus of this review.

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Primary cutaneous Epstein-Barr virus–related lymphoproliferative disorders in 4 immunosuppressed children

Cited by 25 publications

References 24 publications

Subcutaneous Panniculitis-like T-Cell Clonal Proliferation in an Infant. Lymphoma or Reactive Lymphoproliferation?

Subcutaneous Panniculitis-like T-Cell Clonal Proliferation in an Infant. Lymphoma or Reactive Lymphoproliferation?

Cutaneous Lymphoproliferative Disorders in Other Iatrogenic and Non‐Iatrogenic Immune Deficiencies

Skin and soft tissue infections in the transplant population

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