Pagetoid reticulosis C84.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Epidermotropic reticulosis; Localized mycosis fungoides with epidermotropism; Mycosis fungoides type pagetoid reticulosis; pagetoid reticulosis; Reticulosis epidermotropic; Retikless pagetoid; Unilesional mycosis fungoides; Woringer-Kolopp disease; Woringer-Kolopp M.

History
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Kolopp and Woringer, 1939

Definition
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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.

Etiopathogenesis
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Indolent (survival time > 10 years), epidermotropic T-cell lymphoma.

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

Localization
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Distal lower extremity, distal upper extremity.

Clinical features
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  • Localized type (Woringer-Kolopp): Initially described, mostly asymptomatic, sharply limited, moderately intense erythema with varying scaling and very slow growth tendency. Over the years, increasing infiltration of the lesion with formation of a disc-shaped, red or reddish-brown, slowly appositionally growing, scaly, possibly also hyperkeratotic plaque. Formation of arcuate and ring-shaped lesions. In the case of long duration, extensive erosions and ulcer formation.
  • Disseminated type (Ketron and Goodman): Disseminated, reddened, rapidly growing, scaly plaques, especially in older men. Tendency to generalization.

    Notice! These are probably T-cell lymphomas which must be classified as CD8+ cutaneous T-cell lymphomas (see below lymphoma, cutaneous T-cell lymphoma, cytotoxic (CD8-positive)

    ).

    Notice! In contrast to Mycosis fungoides a systemic involvement is missing. The life expectancy is not limited.

Histology
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  • 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.

Differential diagnosis
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Therapy
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  • 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.

Progression/forecast
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Very favourable prognosis, especially with localised type. Transition to a "classical" Mycosis fungoides is possible.

Literature
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  1. Kerscher M et al. Pagetoid reticulosis therapy with PUVA bath photochemotherapy. In: Plewig G et al (Ed.) Progress in practical dermatology and venereology 1995
  2. Luther H et al (1989) Pagetoid reticulosis of the type Ketron-Goodman. 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 infiltrate intra-épidermique d'apparance tumorale. Ann Dermatol Syphilogr (Paris) 10: 945-958

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020