Plasmacytoid (CD68+CD123+) monocytes may play a crucial role in the pathogenesis of hydroa vacciniforme: a case report.
Hydroa vacciniforme (HV) is a rare, blistering, childhood photodermatosis that heals with smallpox-like scars, is associated with latent Epstein-Barr virus (EBV) infection, and spontaneously resolves by early adulthood. Cytotoxic T cells are suspected to mediate the histologic hallmark of HV-dense, perivascular, lymphocytic infiltrates and reticular degeneration and necrosis of the epidermis. We report a case of 13-year-old white girl with EBV-associated HV, whose lesional skin harbored a predominate CD68, CD123 infiltrate of plasmacytoid monocytes (PMs). Other significant pathologic attributes included abundant non-neutrophilic nuclear debris, necrosis of adnexal structures, lymphocytic vasculitis, and clusters of CD30 cells. PMs produce large amounts of type I interferon during viral infection, which induces apoptosis of some cell types and promotes the survival of others. This antiviral response can explain these aforementioned pathologic findings representing wide spread cell death and lymphoid proliferation, as well as eventual resolution of HV with time, via elimination of latent EBV-infected cells. The destruction of adnexal structures can account for the depressed circinate scars characteristic of HV. The validity of this proposed mechanism must be evaluated by first examining the prevalence and number of PMs in future case series of HV.
Varughese
Neal
N
Albany Medical College, Albany, NY, USA.
Petrella
Tony
T
Singer
Mitchel
M
Carlson
J Andrew
JA
D000293:Adolescent; D015703:Antigens, CD; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D006837:Hydroa Vacciniforme / Q000276:immunology* / Q000473:pathology* / Q000821:virology; D016130:Immunophenotyping; D009000:Monocytes / Q000276:immunology*
D015703:Antigens, CD