WORINGER-KOLOPP DISEASE

By Gary M. White, MD

Woringer-Kolopp Disease This solitary lesion on the thigh was the only evidence of CTCL in this patient.


Woringer-Kolopp disease (WKD), also known as localized pagetoid reticulosis or unilesional CTCL, represents a solitary lesion of cutaneous T-cell lymphoma (CTCL).

Clinical           

A red, violaceous, or brown indurated, scaly, or hyperkeratotic patch or plaque most often on the hands or feet but occurring anywhere which has been slowly enlarging for many years is characteristic of Woringer-Kolopp disease. It may eventuate into a nodular plaque. Progression of the localized type to disseminated lesions (AKA pagetoid reticulosis) occurs rarely.

Differential Diagnosis and Workup

The term Woringer-Kolopp disease is best restricted to a solitary lesion that histologically shows an epidermotropic infiltrate of atypical convoluted lymphocytes with the T helper phenotype similar to CTCL lesions. The documentation of clonal restriction is helpful. Similar lesions occurring as a response to arthropod assault have been reported [Arch Dermatol. 1999 Dec;135(12):1543-4, 1546-7]. A thorough HP, CXR, CBC and bone marrow biopsy are appropriate and should be negative for any evidence of systemic lymphoma.

Treatment

Surgical excision and/or local radiation may be all the treatment that is necessary. Photodaynamic therapy and PUVA have also been used. Close clinical followup is important although WKD appears to be a benign unilesional T-cell lymphoproliferative process with few if any reported patients going on to overt lymphoma. Imiquimod 5% 3/week x 2 months cleared a 62 year-old-woman's WKD [JEADV 2016;30;324].

If excision or radiation therapy clears the lesion, but the patient subsequently develops one or more lesions in other areas, then more traditional treatment for CTCL should be considered.

References

An 89 year-old man with an ulcerated, crusting plaque of the left foot [Dermatology Online Journal ]

A 24-year-old woman presented with a slowly enlarging, 10x10mm, erythematous plaque with central scaling and a raised border on the medial side of the distal right foot x 7 months. The Journal of Clinical and Aesthetic Dermatology

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