Pagetoid reticulosis: causes, symptoms, diagnosis, treatment

Pagetoidic reticulosis (blue sickness of Vorenje-Koloppa). Described by FR Woringer and P. Kolopp in 1939. The term "pagetoid reticulosis" was introduced by O. Biaun-Falco et al. In 1973, on the basis of the observed invasion of the lower layers of the epidermis by atypical cells with a light cytoplasm, reminiscent of the appearance of Paget cells. Clinically, mostly on the skin of the limbs, separate erythema-squamous plaques of a red-violet, red-brown fasting are clearly separated, sometimes annular or arcuate. Dyssemia-irradiated foci are also observed.

Pathomorphology. Acanthosis, parakeratosis, spongioform changes with formation of vesicles of various sizes are noted in the epidermis. In places of edema, infiltration with mononuclear cells with polymorphic, hyperchromic nuclei and cytoplasmic lysis around the nucleus is visible. The lower parts of the epidermis and the upper parts of the dermis are infiltrated by large lymphocytes with a light cytoplasm of the pagetoid type. Among the cells of the infiltrate are also histiocytes, individual eosinophils. Individual cells of the infiltrate are often observed in the sebaceous-hair follicles and sweat glands. Sometimes infiltration of a perivascular nature. Electron microscopic data indicate a heterogeneous character of the infiltrate. The latter contains lymphocytes with different degrees of differentiation, ranging from small forms to large, stimulated forms, cells with cerebral nuclei and atypical mononuclear cells with signs of histiocytes. The immunophenotype of cells with limited forms of pagetoidic reticulosis is similar to that of mushroom mycosis. With disseminated forms, CD8 + can occur. PCR, as a rule, reveals a reorganization of the genome of the T-cell receptor of clonal cells.

[1], [2], [3], [4], [5], [6]