Cutaneous myeloid dendritic cell dyscrasia: A cutaneous clonal monocytosis associated with chronic myeloproliferative disorders and peripheral blood monocytosis.
Monocytes are critical components of the innate immune system and they can differentiate into dendritic cells (DCs). Cutaneous neoplasms of dendritic cell origin are uncommon and mostly represented by histiocytic lesions derived primarily from Langerhans cells. The myeloid DC (mDC) while recognized in the immunology literature does not have a well-defined neoplastic cutaneous counterpart. Eleven patients with a diagnosis of cutaneous mDC dyscrasia were evaluated. Routine hematoxylin and eosin stain were performed followed by selective phenotypic studies. The patients were older without a gender predilection and exhibited an asymptomatic papular skin rash with a waxing and waning course. The biopsies demonstrated a dermal based monomorphic small mononuclear cell infiltrate. The cells expressed CD14, CD11c, HLA-DR, as well as granzyme and lysozyme that defines terminally differentiated monocyte/dendritic cells. Expression of BDCA-3 (CD141) by the tumor cells indicated that they were myeloid dendritic cells (mDC2). Each patient had a prior or subsequent diagnosis of an abnormal bone marrow biopsy that included myelodysplastic syndrome, myelofibrosis, chronic myelomonocytic leukemia, and acute myelogenous leukemia. We propose the term cutaneous mDC cell dyscrasia for distinctive infiltrates of differentiated mDCs reflective of underlying myeloproliferative disease. The clinical course is variable and can be indolent although it is strongly correlated with myelodysplastic syndrome that included leukemia.
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, 10065. Electronic address: cym2003@med.cornell.edu.
Cynthia M
CM
Magro
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, 10065.
Shabnam
S
Momtahen
Clarient, Aliso Viejo, CA.
Shalini
S
Verma
OSF ST. Francis Medical Center, Peoria, IL.
Ronnie M
RM
Abraham
Integrated Medical Professions, Melville, NY.
Constantin
C
Friedman
Ohio State University Comprehensive Cancer Center, Columbus, OH, 43210; Phylogeny Inc., Powell, OH.
Gerard J
GJ
Nuovo
Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, 10065.
Wayne
W
Tam
D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology*; D002908:Chronic Disease; D003713:Dendritic Cells / Q000166:cytology*; D005260:Female; D006801:Humans; D007801:Langerhans Cells / Q000473:pathology; D015470:Leukemia, Myeloid, Acute / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000473:pathology*; D009196:Myeloproliferative Disorders / Q000209:etiology / Q000473:pathology*; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology*
Chronic myeloproliferative disorders; Clonal monocytosis; Cutaneous myeloid dendritic cell dyscrasia; Myeloid dendritic cell