Woringer-Kolopp disease


Woringer-Kolopp disease

Description, Causes and Risk Factors:

Woringer-Kolopp disease, is a form of cutaneous T-cell lymphoma that demonstrates striking epidermotropism (movement towards the epidermis, as in the migration of T lymphocytes into the epidermis in mycosis fungoides) on histologic examination. Two variants of the disease are described: the localized type (Woringer-Kolopp disease [WKD]) and the disseminated type (Ketron-Goodman disease [KGD]). WKD typically presents as a solitary, slowly growing cutaneous plaque on the extremities. The lesional epidermis is markedly acanthotic and is infiltrated by cytologically atypical mononuclear cells demonstrating striking epidermotropism. The histologic features of KGD are identical to those of WKD. The distinction between the two entities rests on clinical grounds alone. The Ketron-Goodmann variant is characterized by dissemination of lesions, possible association with other lesions characteristic of mycosis fungoides (MF), and, at times, progression to fatal outcome. Some authors have suggested that because of its more aggressive biologic behavior, KGD should be regarded as a variant of MF showing prominent epidermotropism and that the term PR (pagetoid reticulosis) should be reserved for the indolent form of the disease (WKD). The nature of the atypical cells in PR was the subject of much controversy in literature over the past years. At present, however, immunophenotypic and genophenotypic studies have clearly established a T-cell origin and monoclonality of the atypical epidermotropic cells. Cases of PR with T-helper phenotype (CD4), T-cytotoxic/suppressor (CD8) phenotype, and CD4/CD8 double-negative phenotype are described.

Woringer-Kolopp disease

WKD is frequently considered as a slowly growing indolent disease which may be cured after total excision, but recent reports have shown the development of a more aggressive disseminated form long after removal of a localized lesion. It is unclear which patients are at risk of progression from WKD to KGD or systemic lymphoma with a less favorable prognosis. Crowson and Magro reported the unpredictability of the biological behavior of atypical epidermotropic lymphoid infiltrates on the basis of any single criterion. Thus, careful correlation of histopathological, immunophenotypic, genotypic, and - perhaps most importantly - clinical features is required to discriminate PR from MF and T-cell lymphomas. In some patients, although conversion from WKD to the disseminated form was not diagnosed, the doubling of the lesion's size in just 3 months was interesting. This finding may be considered as an indicator of a behavioral change from an indolent to a more aggressive form without evidence of disease dissemination, which is uncommon.

Many aspects of the disease are still the subject of controversy. These include basic issues, such as whether localized Woringer-Kolopp disease is an inflammatory or neoplastic disorder; if neoplastic, whether it is benign or malignant; and the relationship of PR to MF. The early literature concentrates mainly on the nature of the pagetoid intraepidermal cells that are the histologic hallmark of the disease. Some authors speculate that the atypical cells are related to Merkel cells. Others favor a histiocytic origin based on ultrastructural and enzyme histochemical observations. However, more recent studies unequivocally establish a T-cell lineage for the atypical epidermotropic cells. Further subtyping reveals three different phenotypes for the large epidermotropic cells, namely a CD4-positive T-helper phenotype, CD8-positive T-cytotoxic/suppressor phenotype, or a CD4/CD8 double-negative phenotype.

Symptoms:

The most important clinical feature of this disease is manifestation as a single very slowly enlarging verrucoid, plaque-type skin lesion without internal organ involvement. Histologically, the epidermis is extremely acanthotic and densely infiltrated with numerous atypical appearing cells that are entirely absent from the dermis, which simply contains a banal dense chronic inflammatory infiltrate.

Diagnosis:

The diagnosis is based on clinical presentation and is confirmed by a skin biopsy. Histological findings reveal a hyperplastic epidermis with marked infiltration by atypical 'haloed' lymphocytes that have medium-sized, pleomorphic nuclei. Immunohistology shows either a memory helper T-cell (CD3+, DC4+, CD8-) or a cytotoxic T-cell (CD3+, CD4-, CD8+) phenotype. CD30 may be positive. The differential diagnosis includes other epidermotropic lymphomas, such as CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma.

Treatment:

Although there is no consensus on the treatment of Woringer-Kolopp disease, ultraviolet, photodynamic and radiation therapy have been suggested to be effective in achieving local control in WKD, whereas radiotherapy, chemotherapy or combined modality therapy have been reported to have widely variable efficacy in KGD.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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