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В статье представлено собственное клиническое наблюдение редко встречающегося диссеминированного педжетоидного ретикулеза у 72-летней пациентки. Приведены клинико-морфологические критерии и гистологические признаки, на основании которых была верифицирована данная патология. Показана важная роль в постановке диагноза патогистологического исследования кожных элементов с обязательным применением иммуногистохимического типирования. Для снижения вероятности ошибочной диагностики педжетоидного ретикулеза врачам важно знать особенности клинического течения и специфические патоморфологические признаки заболевания., The article presents our own clinical case of rare disseminated pagetoid reticulosis in a 72-year-old patient. The authors provide clinical and morphological criteria and histological signs of the disease. Histopathological examination of skin elements with mandatory use of immunohistochemical typing plays an important role in the diagnosis. To reduce the likelihood of error diagnosis of pagetoid reticulosis, doctors should know the features of clinical course and specific pathomorphological signs of the disease

Pagetoid reticulosis (PR) is a rare form of cutaneous T-cell lymphoma [Mod Pathol 2000;13:502–510]. Two variants of the disease are described: the localized type Woringer-Kolopp disease (WKD) and the disseminated type Ketron-Goodman disease (KGD). KGD may have disseminated lesions, high rate of recurrence and a guarded prognosis [Mod Pathol 2000;13:502–510]. In patients with KGD, therefore, long-term observation is necessary. Disappearance of cutaneous lesions does not mean resolution of the disease [J Am Acad Dermatol 2002;47:183–186]. Herein we report the case of an 84-year-old man with erythematous patches of the trunk and the upper and lower extremities in whom the diagnosis of KGD was made. We describe this case for the rarity of this pathology and for the good response to therapy (IFN).

In this historical review I will synopsize the original articles by Lloyd W. Ketron and M.H. Goodman who described Ketron-Goodman disease, by Frederic Woringer and Pierre Kolopp who described Woringer-Kolopp disease, and by Otto Braun-Falco and colleagues who described pagetoid reticulosis. In their publications, each of these authors reported on one patient. I will review the clinical picture of the three patients, their histopathology, and the pathogenesis of each disease as suggested by the above authors. Then the views of others that have written on the subject recently will be reviewed particularly as to their conception of the diseases. Publications that describe the histopathology of the patch (early) stage of mycosis fungoides will be redacted to compare it to the histopathology of Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis. I will discuss whether any or all of them are diseases sui generis, whether they are one, two, or three entities, or whether any or all are but forms of mycosis fungoides.

Disseminated pagetoid reticulosis is considered to have a poor prognosis. Some authors regard this disease as an epidermotropic type of mycosis fungoides rather than a distinct entity. We report the case of an 83-year-old woman with disseminated pagetoid reticulosis who subsequently developed tumoral lesions and died soon after the appearance of these tumours. Our case is in agreement with findings that disseminated pagetoid reticulosis has an aggressive clinical behaviour.