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Pagetoid reticulosis of Woringer-Kolopp

  • Author(s): Dusan, Skiljevic
  • Zoran, Bogdanovic
  • Sonja, Vesic
  • Jelica, Vukicevic-Sretenovic
  • Mirjana, Gajic-Veljic
  • Ljiljana, Medenica
  • et al.
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Pagetoid reticulosis of Woringer-Kolopp
Skiljevic Dusan, Bogdanovic Zoran, Vesic Sonja, Vukicevic-Sretenovic Jelica, Gajic-Veljic Mirjana, Medenica Ljiljana
Dermatology Online Journal 14 (1): 18

Institute of Dermatovenereology, Department of Dermatovenereology, School of Medicine University of Belgrade, Belgrade, Serbia. limed@EUnet.yu

Abstract

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" [1]. The term pagetoid reticulosis was proposed by Braun-Falco in 1973 [2]. 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].


Clinical synopsis

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).


Figure 1Figure 2
Figure 1. Asymptomatic hyperkeratotic scaly plaques localized on both palms
Figure 2. Plaques have prominent edges

Figure 3Figure 4
Figure 3. Small round hyperkeratotic, yellowish plaques on the right sole
Figure 4. Hyperkeratosis and acanthosis in epidermis and dense, diffuse lymphocytic infiltrate, mainly in papillary dermis, with prominent epidermotropism

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.


Discussion

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) [8]. 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 [9]. 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 [10].

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.

References

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2. Braun-Falco O, Marghescu S, Wolff HH. Pagetoide reticulose. Morbus Woringer-Kolopp. Hautarzt. 1973;24:11-21. PubMed

3. Steffen C. Ketron-Goodman Disease, Woringer-Kolopp Disease, and Pagetoid Reticulosis. Am J Dermatopathol 2005; 27(1): 68-85. PubMed

4. Cribier B. History: Frederic Woringer (1903-1964) and Woringer-Kolopp Disease. Am J Dermatopathol 2005; 27(6): 534-45. PubMed

5. Ralfkiaer E, Jaffe ES. Mycosis fungoides and Sézary syndrome. In: Jaffe ES, Harris NL, eds. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Geneva, Switzerland: World Health Organization; 2001. ISBN 92 832 2411 6

6. Willemze R, Kerl H, Sterry W, et al. EORTC classification for primary cutaneous lymphomas: a proposal from the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer (EORTC). Blood. 1997;90: 354-371. PubMed

7. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105 (10): 3768-85. PubMed

8. Sterry W, Hauschild A. Loss of leukocyte common antigen (CD45) on atypical lymphocytes in the localized but not disseminated type of Pagetoid reticulosis. Br J Dermatol. 1991; 125:238-242. PubMed

9. Haghighi B, Smoller BR, LeBoit PE, et al. Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular and clinicopathologic study. Mod Pathol. 2000;13: 502-510. PubMed

10. Drillenburg P, Bronkhorst CM, Van der Wal AC, et al. Expression of adhesion molecules in pagetoid reticulosis (Woringer-Kolopp disease). Br J Dermatol. 1997;136:613-616. PubMed

11. Yagi H, Hagiwara T, Shirahama S, et al. Disseminated pagetoid reticulosis: Need for long-tem follow-up. JAAD 1994; 30(2): 345-9. PubMed

12. Ioannides G, Engel MF, Rywlin AM. Woringer-Kolopp disease (pagetoid reticulosis). Am J Dermatopathol. 1983;5(2):153-8. PubMed

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