Cutaneous Involvement in Patients With Angioimmunoblastic Lymphadenopathy With Dysproteinemia<subtitle>A Clinical, Immunohistological, and Molecular Analysis</subtitle>

Angioimmunoblastic T Cell Lymphomas: Frequent Cutaneous Skin Lesions and Absence of Human Herpes Viruses

“…However, a wide range of skin lesions has been described including papules, plaques, nodules, erosions, urticarial or purpuric eruptions or diffuse erythema [5][6][7][8] .…”

“…In previous reports, skin lesions occurred in 40% to 50% of AITL cases, but the skin pathology was reported to be merely nonspecific [5][6][7][8] . Because of the rarity of AITL and the near absence of skin biopsies, the skin lesions have not been clarified in the dermatologic literature 7,8 .…”

Follicular center helper T-cell (TFH) marker positive mycosis fungoides/Sezary syndrome

“…15,[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] Clinically, primary cutaneous CD4( þ ) small/ medium-sized pleomorphic T-cell lymphoma and angioimmunoblastic T-cell lymphoma present or may present with primary cutaneous involvement. 20,[36][37][38][39][40] Other CD4( þ ) T-cell lymphomas with primary cutaneous manifestations include mycosis fungoides and its variants including Sezary syndrome, adult T-cell leukemia/lymphoma and primary cutaneous CD30 þ lymphoproliferative disorders. 41 CD10 has not been reported to be a feature of mycosis fungoides or Sezary syndrome.…”

“…20,34 Angioimmunoblastic T-cell lymphoma is an aggressive nodal-based disease that involves skin in roughly 50% of individuals. [38][39][40] The neoplastic T cells in angioimmunoblastic T-cell lymphoma express the follicular center helper T-cell markers CD10, PD-1, and CXCL-13 in the majority of cases. 27,[41][42][43] Angioimmunoblastic T-cell lymphoma is also notable for an association with EBV as 70% or more angioimmunoblastic T-cell lymphoma lesions contain EBV( þ ) B cells, a finding helpful in establishing the diagnosis.…”

Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

“…54,55 However, angioimmunoblastic T-cell lymphoma often presents as a diffuse, pruritic, maculopapular eruption and shows a perivascular infiltrate, vascular proliferation, and/or vasculitis on biopsy, 53,55 features that are not typical of PC-SMTCL. In addition, angioimmunoblastic Tcell lymphoma may include Epstein-Barr virus-positive cells 53,55 and commonly expresses CD10, including in extranodal sites. 53 Finally, patients with angioimmunoblastic T-cell lymphoma have evidence of systemic disease and typically follow an aggressive course with a 5-year survival less than 30%.…”