Localized pagetoid reticulosis (Woringer-Kolopp disease) in early childhood. 2009

Yasushi Matsuzaki, and Kazuyuki Kimura, and Hajime Nakano, and Katsumi Hanada, and Daisuke Sawamura
Department of Dermatology, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori, Japan. ymatsu@cc.hirosaki u.ac.jp

Localized pagetoid reticulosis, also known as Woringer-Kolopp disease, is an uncommon cutaneous lymphoproliferative disorder classified as a solitary variant of mycosis fungoides. We describe a case of localized pagetoid reticulosis that occurred in early childhood. A 6-year-old boy presented with an erythematous plaque on the left thigh. His parents had first noted brownish erythema of his thigh a few months after birth. The size and elevation of the plaque gradually increased. Physical examination showed well-demarcated reddish plaque, 6.2 x 2.3 cm, with central erosion. Histopathological examination showed an epidermotropic infiltrate of medium atypical lymphocytes with hyperchromatic and pleomorphic nuclei. The atypical cells infiltrated as individual cells or clusters in the epidermis. Immunohistologically, the phenotype of the atypical cells was CD3(+), CD4(-), CD8(+), CD45RO(+), CD20(-), CD30(-), and CD79a(-). We discuss the characteristics of this rare disease, including the differential diagnoses.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D009182 Mycosis Fungoides A chronic, malignant T-cell lymphoma of the skin. In the late stages, the LYMPH NODES and viscera are affected.
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D015704 CD4 Antigens 55-kDa antigens found on HELPER-INDUCER T-LYMPHOCYTES and on a variety of other immune cell types. They are members of the immunoglobulin supergene family and are implicated as associative recognition elements in MAJOR HISTOCOMPATIBILITY COMPLEX class II-restricted immune responses. On T-lymphocytes they define the helper/inducer subset. T4 antigens also serve as INTERLEUKIN-15 receptors and bind to the HIV receptors, binding directly to the HIV ENVELOPE PROTEIN GP120. Antigens, CD4,CD4 Molecule,CD4 Receptor,CD4 Receptors,Receptors, CD4,T4 Antigens, T-Cell,CD4 Antigen,Receptors, Surface CD4,Surface CD4 Receptor,Antigen, CD4,Antigens, T-Cell T4,CD4 Receptor, Surface,CD4 Receptors, Surface,Receptor, CD4,Surface CD4 Receptors,T-Cell T4 Antigens,T4 Antigens, T Cell
D017252 CD3 Complex Complex of at least five membrane-bound polypeptides in mature T-lymphocytes that are non-covalently associated with one another and with the T-cell receptor (RECEPTORS, ANTIGEN, T-CELL). The CD3 complex includes the gamma, delta, epsilon, zeta, and eta chains (subunits). When antigen binds to the T-cell receptor, the CD3 complex transduces the activating signals to the cytoplasm of the T-cell. The CD3 gamma and delta chains (subunits) are separate from and not related to the gamma/delta chains of the T-cell receptor (RECEPTORS, ANTIGEN, T-CELL, GAMMA-DELTA). Antigens, CD3,CD3 Antigens,T3 Antigens,CD3 Antigen,T3 Antigen,T3 Complex,Antigen, CD3,Antigen, T3,Antigens, T3
D017730 Ki-1 Antigen A member of the tumor necrosis factor receptor superfamily that may play a role in the regulation of NF-KAPPA B and APOPTOSIS. It is found on activated T-LYMPHOCYTES; B-LYMPHOCYTES; NEUTROPHILS; EOSINOPHILS; MAST CELLS and NK CELLS. Overexpression of the Ki-1 antigen in hematopoietic malignancies make it clinically useful as a biological tumor marker. Signaling of the receptor occurs through its association with TNF RECEPTOR-ASSOCIATED FACTORS. Antigens, CD30,Antigens, Ki-1,Ber-H2 Antigens,CD30 Antigens,Ki-1 Antigens,Tumor Necrosis Factor Receptor Superfamily, Member 8,Ber-H2 Antigen,CD30 Antigen,TNFRSF8 Receptor,Antigen, Ber-H2,Antigen, CD30,Antigen, Ki-1,Antigens, Ber-H2,Antigens, Ki 1,Ber H2 Antigen,Ber H2 Antigens,Ki 1 Antigen,Ki 1 Antigens,Receptor, TNFRSF8

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