title: Microcystic adnexal carcinoma associated with primary immunodeficiency, recurrent diffuse herpes simplex virus infection, and cutaneous T-cell lymphoma.
abstract: Cutaneous microcystic adnexal carcinoma (MAC) is a rare and poorly understood tumor that predominantly occurs in the head and neck. MAC usually affects people in their fourth and fifth decades. Some patients have had a history of radiation. We present a case of MAC occurring in the left antecubital fossa of an 18-year-old white woman with an unusual immunodeficiency syndrome. The patient also developed a squamous cell carcinoma, a cutaneous T-cell malignancy, and a perigastric leiomyoma. A congenital infection of herpes simplex virus (HSV) persisted throughout her life. The association of HSV infection with MAC and squamous cell carcinoma and that of peripheral T-cell lymphoma with Epstein-Barr virus is discussed in relation to her immunodeficiency.
authors.affiliation: Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
authors.forename: J Y
authors.initials: JY
authors.lastname: Lei
authors.forename: Y
authors.initials: Y
authors.lastname: Wang
authors.forename: E S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: M L
authors.initials: ML
authors.lastname: Turner
authors.forename: M
authors.initials: M
authors.lastname: Raffeld
authors.forename: L
authors.initials: L
authors.lastname: Sorbara
authors.forename: J
authors.initials: J
authors.lastname: Morris
authors.forename: S M
authors.initials: SM
authors.lastname: Holland
authors.forename: P H
authors.initials: PH
authors.lastname: Duray
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D018280:Carcinoma, Skin Appendage / Q000737:chemistry / Q000150:complications* / Q000473:pathology; D002294:Carcinoma, Squamous Cell / Q000737:chemistry / Q000150:complications / Q000473:pathology; D005260:Female; D006561:Herpes Simplex / Q000150:complications*; D006801:Humans; D007124:Immunoenzyme Techniques; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry / Q000150:complications* / Q000473:pathology; D012008:Recurrence; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology; D012878:Skin Neoplasms / Q000737:chemistry / Q000150:complications* / Q000473:pathology
chemical_list: D014408:Biomarkers, Tumor