Pagetoid reticulosis C84.4

Misnomer for a localized, low-malignant cutaneous T-cell lymphoma (special form of Mycosis fungoides) with unusually pronounced epidermotropy. Clinically, a distinction is made between a localized and a disseminated type, the entity of which is doubted by some authors.

Any age group, m>f; occurrence also described in childhood.

Localized type (Woringer-Kolopp): Initially circumscribed, usually asymptomatic, sharply circumscribed red-brown erythema with few symptoms, usually mildly scaling. There is a continuous but slow growth tendency. Over months/years, there is increasing infiltration of the lesions with formation of disc-shaped, red or red-brown, slowly appositionally growing, scaling, possibly hyperkeratotic plaques. With further growth, typically, arcuate or annular lesions form. With long duration also areal erosions and ulcerations.

Disseminated type (Ketron and Goodman): Occurring primarily in older men, disseminated, reddened, rapidly growing, scaly plaques. Tendency to generalize.

Notice. These were probably T-cell lymphomas that must be classified as CD8+ cutaneous T-cell lymphoma(see also below Lymphoma, cutaneous T-cell lymphoma, cytotoxic (CD8-positive)).

Notice. In contrast to mycosis fungoides, systemic involvement is absent. Life expectancy is not limited.

• Acanthoid widened epidermis, papillomatosis, pagetoid penetration of the epidermis and epidermal adnexa with medium to large, atypical lymphoid cells These show prominent, chromatin dense, partially indented nuclei. A pronounced "halo reaction" of the exophytic lymphoid cells is typical (in HE section optically empty cytoplasm around the nucleus, see below Mycosis fungoides). As a result, the epidermis is spongy in places. Conspicuously few mitoses are detectable. The dermis shows low perivascular round cell infiltrates (small lymphocytes).
• Electron microscopy: Intraepidermal Lutzner cell-like and blister-like cells.
• Immunohistology: Tumor cells CD3, CD4, CD5 pos.; rarely CD30 pos.; some cases with CD4 neg. and CD8 pos. tumor cells have been reported (not corresponding to the classical cases of pagetoid reticulosis; assignment to cytotoxic cutaneous T-cell lymphomas).
• Molecular biology: Detection of T-cell receptors of the alpha-beta or gamma-delta type.

• For isolated herds, generous excision;
• selective PUVA bath therapy (4 times/week), alternatively photodynamic therapy.
• X-ray or electron irradiation.
• In individual cases good therapy results were achieved with Imiquimod.
• In disseminated foci, see below cutaneous T-cell lymphoma.

1. Kerscher M et al. Pagetoid reticulosis therapy with PUVA bath photochemotherapy. In: Plewig G et al (eds) Progress in Practical Dermatology and Venereology 1995.
2. Luther H et al (1989) Pagetoid reticulosis of the ketron-Goodman type. Dermatologist 40: 530-535
3. Matsuzaki Y et al (2009) Localized pagetoid reticulosis (Woringer-Kolopp disease) in early childhood. J Am Acad Dermatol 61:120-123.
4. Mendese GW et al (2012) Pagetoid reticulosis in a prepubescent boy successfully treated with photodynamic therapy. Clin Exp Dermatol 37:759-761
5. Miedler JD et al (2008) Pagetoid reticulosis in a 5-year-old boy. J Am Acad Dermatol 58:679-681.
6. Morariu SH et al (2014) Pagetoid reticulosis Woringer-Kolopp type, a particular variant of mycosis fungoides: a case report. Rome J Morphol Embryol 55:1469-1472.
7. Nakada T et al (2002) Disseminated pagetoid reticulosis (Ketron-Goodman disease): six-year follow-up. J Am Acad Dermatol 47(2 Suppl): S183-186
8. Su O et al (2014) Treatment of localized pagetoid reticulosis with imiquimod: a case report and literature review. J Eur Acad Dermatol Venereol doi: 10.1111/jdv.12718.
9. Wang T et al (2014) Localized pagetoid reticulosis (Woringer-Kolopp disease) completely response to NB-UVB combined with IFN. Dermatol Ther 27:227-229
10. Woringer F, Kolopp P (1939) Lésion érythemato-squameuse polycyclique de l'avant-bras évoluant depuis 6 ans chez un garçonnet de 13 ans. Histologiquement infiltrat intra-épidermique d'apparance tumorale. Ann Dermatol Syphilogr (Paris) 10: 945-958