Microcystic adnexal carcinoma associated with primary immunodeficiency, recurrent diffuse herpes simplex virus infection, and cutaneous T-cell lymphoma

Am J Dermatopathol. 2000 Dec;22(6):524-9. doi: 10.1097/00000372-200012000-00008.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Biomarkers, Tumor / analysis
  • Carcinoma, Skin Appendage / chemistry
  • Carcinoma, Skin Appendage / complications*
  • Carcinoma, Skin Appendage / pathology
  • Carcinoma, Squamous Cell / chemistry
  • Carcinoma, Squamous Cell / complications
  • Carcinoma, Squamous Cell / pathology
  • Female
  • Herpes Simplex / complications*
  • Humans
  • Immunoenzyme Techniques
  • Immunologic Deficiency Syndromes / complications*
  • Immunologic Deficiency Syndromes / pathology
  • Lymphoma, T-Cell, Cutaneous / chemistry
  • Lymphoma, T-Cell, Cutaneous / complications*
  • Lymphoma, T-Cell, Cutaneous / pathology
  • Recurrence
  • Skin Diseases / complications*
  • Skin Diseases / pathology
  • Skin Neoplasms / chemistry
  • Skin Neoplasms / complications*
  • Skin Neoplasms / pathology


  • Biomarkers, Tumor