Microcystic adnexal carcinoma associated with primary immunodeficiency, recurrent diffuse herpes simplex virus infection, and cutaneous T-cell lymphoma.
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.
Lei
J Y
JY
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Wang
Y
Y
Jaffe
E S
ES
Turner
M L
ML
Raffeld
M
M
Sorbara
L
L
Morris
J
J
Holland
S M
SM
Duray
P H
PH
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
D014408:Biomarkers, Tumor