Pagetoid reticulosis of Woringer-Kolopp
Institute of Dermatovenereology, Department of Dermatovenereology, School of Medicine University of Belgrade, Belgrade, Serbia. limed@EUnet.yu
Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one or several scaly patches and plaques with an acral distribution. We present a case of a 58-year-old woman, otherwise healthy, with a 5-month history of asymptomatic, hyperkeratotic plaques on the hands and feet. Histological and immunohistochemical analysis confirmed the diagnosis. Nearly complete spontaneous regression was noted 7 months after the initial examination. After 5 years no evidence of the disease remained.
Localized pagetoid reticulosis is an uncommon disease described by Woringer and Kolopp in 1939 as "polycyclic erythematous scaly lesions with 6-year evolution on the forearm of 13-year-old boy" . The term pagetoid reticulosis was proposed by Braun-Falco in 1973 . The origin of the pagetoid cells remained undetermined for many years; melanocytes, histiocytes, Merkel cells, true Paget cells had all been proposed. Finally, lymphocytes were proven to be the cells responsible for the epidermotropic infiltrate [3, 4]. Now pagetoid reticulosis of Woringer-Kolopp is classified as one of the indolent forms of primary cutaneous T-cell lymphoma [5, 6, 7].
A 58-year-old woman was seen at our Institute in April 2002 for a 5-month history of asymptomatic scaly plaques localized on the palms and soles. Family and personal history were unremarkable. On examination, there were several round to oval, hyperkeratotic, erythematous, yellowish, scaly plaques with prominent margins, 1-2 cm in diameter, localized on both palms and the right sole (Figs. 1-3).
Histopathologic examination revealed hyperkeratosis and acanthosis of the epidermis and a dense, diffuse, lymphocytic infiltrate, mainly in the papillary dermis, with massive infiltration of the epidermis. Most lymphocytes in the infiltrate were large, with hyperchromatic and convoluted nuclei; some of them had a pericellular halo (Fig. 4). Immunohistochemical analysis showed a predominance of CD8+ cells over CD4+ cells in the infiltrate with variable loss of pan T-cell markers; CD45 was expressed in approximately 50 percent and CD45RO in approximately 60 percent of tumor cells.
Laboratory studies (CBC, blood chemistries) were within normal limits; physical examination, echosonography of the abdomen, chest X-ray, and bone marrow examination revealed no systemic involvement.
The patient was not interested in aggressive therapy and merely applied various topical corticosteroids and some herbal remedies from time to time. However, 7 months after the initial examination, most lesions had regressed, except for two small plaques localized on right sole. When examined after 5 years the patient had no cutaneous lesions.
Pagetoid reticulosis of Woringer and Kolopp is a rare disease. Based upon histopathologic, immunophenotypic, and genetic features, as well as clinical behavior, it is now classified as an indolent cutaneous T-cell lymphoma [5, 6, 7]. Although current classifications of lymphomas [5, 6, 7] place pagetoid reticulosis in or close to the mycosis fungoides group, there are several characteristics that make it unique among the types of CTCL. Pagetoid reticulosis features acral distribution of lesions, pronounced hyperkeratosis and acanthosis, prominent epidermotropism, and expression of a cutaneous lymphocyte antigen (which interacts with E-selectin on skin endothelium) . Epidermotropism is probably the result of strong expression of alpha E beta 7 (which interacts with E-cadherin on epithelial cells). Tumor cells may be CD4 positive or CD8 positive; CD30 is often expressed . Another interesting immunohistochemical finding is the absence of CD45 expression, which is crucial for the function of lymphocyte tyrosine kinase p56lck that plays an important role in lymphocyte growth and transformation. This fact may explain the indolent biological behavior of Pagetoid reticulosis .
Surgery and radiotherapy have been curative in most patients. Our patient was not interested in any kind of therapy other than topical steroids, but she entered remission in 7 months.
Although this form of lymphoma is notorious for its indolent clinical behavior, there are reports that record dissemination and development of systemic lymphoma in some cases [11, 12]. Therefore, long-term follow-up is recommended.
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