Pagetoid reticulosis

Woringer-Kolopp disease · localised pagetoid reticulosis · Ketron-Goodman (disseminated)

Pagetoid reticulosis is a rare WHO-recognised localised variant of mycosis fungoides characterised by a solitary, slowly progressive, well-demarcated psoriasiform or verrucous plaque, usually on an acral / extensor distal extremity. Histology shows striking epidermotropism of atypical lymphocytes ("pagetoid" pattern, resembling Paget cells). The localised Woringer-Kolopp form has an indolent course and excellent prognosis; the disseminated Ketron-Goodman variant is now considered classical MF / aggressive CTCL.

CurrentLast reviewed 16 May 2026

Pathogenesis and classification

Clonal T-cell proliferation with marked epidermotropism.
Woringer-Kolopp (localised): solitary, indolent; CD4+ or CD8+; some CD30+.
Ketron-Goodman (disseminated): now considered aggressive CTCL, classical MF, or CD8+ aggressive epidermotropic CTCL — not a true PR variant.
WHO-HAEM5 classifies localised pagetoid reticulosis as a MF variant.

Clinical features

Solitary, slowly progressive (years) well-demarcated, red-brown or pink-grey, psoriasiform or verrucous plaque.
Site: acral / extensor extremities — dorsum hand, foot, knee, elbow, leg.
1-10 cm diameter; occasionally larger.
Asymptomatic; rarely pruritic.
Prognosis excellent for localised disease; no systemic dissemination reported.

Investigations

Skin biopsy: hyperplastic epidermis infiltrated by single and clustered atypical lymphocytes with halo (pagetoid pattern); minimal dermal infiltrate.
IHC: CD3+, CD4+ or CD8+ (CD8+ commoner in localised PR); CD30 positive in some; loss of CD7; high Ki-67.
TCR gene rearrangement: clonal.
Full skin examination and lymph-node palpation (mandatory exclusion of more extensive MF).
Bloods: FBC, blood-film flow cytometry (rule out Sézary).

Differentials

Classical mycosis fungoides patch / plaque — multiple lesions; truncal predominant.
Bowen disease (cSCC in situ) — keratotic; biopsy + IHC distinguishes.
Psoriasis (single plaque), verruca vulgaris, nummular eczema.
Squamous cell carcinoma.
Lichen simplex chronicus.
Pagetoid melanoma / amelanotic melanoma — biopsy + melanocytic IHC excludes.

Management

Localised radiotherapy — first-line for Woringer-Kolopp; typically 20-30 Gy.
Surgical excision for small accessible lesions.
Topical: super-potent corticosteroids; topical mechlorethamine; imiquimod (limited evidence).
Long-term dermatology follow-up given small risk of evolution.
Counsel about excellent prognosis with localised disease.

References

Woringer F, Kolopp P. Lésion erythemato-squameuse polycyclique de l'avant-bras évoluant depuis 6 ans chez un garçonnet de 13 ans. Ann Dermatol Syphiligr. 1939;10:945-958.

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

Willemze R et al. The 2018 WHO-EORTC classification of cutaneous lymphomas. Blood. 2019;133:1703-1714.

Gilson D et al. British Association of Dermatologists and U.K. Cutaneous Lymphoma Group guidelines for the management of primary cutaneous lymphomas 2018. Br J Dermatol; 2019;180:496-526.