Long-standing CD4+/CD8+ Woringer-Kolopp Disease: A Report of A Rare Case.

Abstract

Introduction:

Woringer-Kolopp disease is a localized pagetoid reticulosis (PR). It is a very rare variant of mycosis fungoides (MF).

Clinical Case:

A 44-year-old Malay lady presented with intermittent pruritic erythematous papular lesions over the right for about 20 years but did not seek treatment. The lesion gradually increased in size and turned into a verrucoid plaque. Ultimately, it became ulcerated and is associated with foul-smelling pus discharge. On examination, a large, verrucous plaque (16cm) with ulceration and erosion on the right heel, extending to the lower is calf is seen. No other cutaneous lesions are present. Systemic and lymph nodes examination are negative. Skin biopsy cultures and TB PCR are negative. HPE reveals neoplastic T-cells with cerebriform nuclei, perinuclear halo and nuclear pleomorphism confined to the epidermis. These cells are positive for LCA, CD3, CD4, CD5 and CD8. Other relevant immunostains were negative. The proliferative index is high (>80%). MRI of the right leg shows suspicious regional tendon involvement. Staging CT scan shows no disseminated disease. No atypical lymphocytes seen in peripheral blood smear. Infective screenings are negative.

Discussion:

PR is an indolent disease with excellent prognosis as opposed to primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma (PCACTL), its disseminated form. Histopathologically, PR has to be distinguished from conditions which are associated with pagetoid epidermotropic growth and other acral presentations of T-cell lymphomas such as MF palmaris et plantaris (MFPEP), PCACTL and Type-D lymphomatoid papulosis (LP-D). Co-expression of CD4 and CD8 in PR is rare but has been described in several literatures. Till date, localized radiation therapy produces the best treatment response.

< Keywords > :  Woringer-Kolopp disease, Pagetoid reticulosis, Mycosis