Woringer-Kolopp (Pagetoid Reticulosis) disease successful response to bexarotene gel.

Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of CTCL. This report describes three female patients (mean age 66 years) with WK disease who had an effective treatment response to bexarotene 1% gel. This treatment could provide a safe alternative to other current treatment modalities which have higher risks of potential adverse effects and lack of access to other conventional treatments such as light therapy.

Pagetoid reticulosis in a prepubescent boy successfully treated with photodynamic therapy.

Pagetoid reticulosis or Woringer-Kolopp disease (WKD) is a rare variant of mycosis fungoides, consisting of localized patches or plaques containing intraepidermal proliferations of neoplastic T cells in a pagetoid distribution (similar to that of the adenocarcinomatous cells found in Paget disease of the nipple), which typically affects middle-aged and elderly men. We report a trial of photodynamic therapy (PDT) with topical aminolaevulinic acid (ALA), carried out on a 10-year-old boy with a solitary lesion of WKD on his foot, to avoid the long-term problems associated with the typical treatments for WKD of surgery and/or local irradiation. The plaque progressively flattened during treatment, and after nine PDT sessions over 13 months, the patient was clinically free of disease. PDT may be a viable alternative to surgery and local irradiation for localized cutaneous T-cell lymphoma, including WKD, especially in younger patients.

Woringer-Kolopp disease mimicking foot dermatitis.

Woringer-Kolopp disease, also known as localized pagetoid reticulosis, is a rare cutaneous lymphoproliferative disorder classified as a solitary variant of mycosis fungoides (MF). Despite the indolent and benign nature of the disease, misdiagnosis and inappropriate treatment may result in years of debilitating symptoms and even loss of function. We present the case of a patient with long-standing Woringer-Kolopp disease that mimicked foot dermatitis. Histopathologic examination demonstrated epidermotropic infiltration of atypical lymphocytes that were CD3+ CD4- CD8-. The patient was successfully treated with topical keratolytics and bexarotene gel 1% with minimal residual lesions after 8 years of follow-up. We discuss the characteristics of this rare disease in contrast with localized MF as well as more aggressive forms of epidermotropic T-cell lymphoma.

A case of pagetoid reticulosis treated with topical photodynamic therapy.

A 70-year-old male presented with reddish brown, keratotic plaque on his left leg which had grown slowly for ten years. The histopathological examination revealed acanthosis and bands of lymphocytes with significant epidermotropism. The intraepidermal lymphocytes showed large, pleomorphic and hyperchromatic nuclei with distinct nucleoli and perinuclear halos, positive for CD3, CD4 and CD30, negative for CD8, CD20 and high in Ki67 index. Accordingly, the diagnosis of Pagetoid reticulosis was made. The patient was then treated with topical photodynamic therapy once a week sequential to partial in-situ resection and preliminary skin dermabrasion and finally achieved complete remission without obvious scars or recurrence after four sessions.