Pedjetoid reticulosis: causes, symptoms, diagnosis, treatment

Pagetoid reticulosis (syn. Woringer-Kolopp disease). Described by FR Woringer and P. Kolopp in 1939. The term "Pagetoid reticulosis" was introduced by O. Biaun-Falco et al. in 1973, based on the observed invasion of the lower layers of the epidermis by atypical cells with light cytoplasm, resembling Paget cells in appearance. Clinically, mostly on the skin of the extremities, separate, clearly delimited, sometimes ring-shaped or arcuate erythematosquamous plaques of a red-violet, red-brown color are found. Disseminated foci are also observed.

Pathomorphology. Acanthosis, parakeratosis, and spongiform changes with formation of vesicles of various sizes are observed in the epidermis. Infiltration by mononuclear cells with polymorphic, hyperchromic nuclei and lysis of the cytoplasm around the nucleus is visible in areas of edema. The lower parts of the epidermis and upper parts of the dermis are infiltrated by large lymphocytes with light cytoplasm of the pagetoid type. Histiocytes and single eosinophils are also found among the infiltrate cells. Individual infiltrate cells are often observed in sebaceous hair follicles and sweat glands. Sometimes the infiltrate is perivascular. Electron microscopic data indicate a heterogeneous nature of the infiltrate. The latter contains lymphocytes with varying degrees of differentiation, from small forms to large, stimulated forms, cells with cerebriform nuclei and atypical mononuclear cells with histiocyte features. The immunophenotype of cells in limited forms of pagetoid reticulosis is similar to that in mycosis fungoides. In disseminated forms, CD8+ may be encountered. PCR, as a rule, reveals genome rearrangement of the T-cell receptor of clonal cells.

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