Woringer-Kolopp Disease, a Rare Dermatologic Malignancy Presenting in a Young Male

Abstract

Abstract Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease (WKD), is a rare variant of cutaneous T-cell lymphoma usually presenting as an isolated patch or plaque on the extremities. Immunohistochemical staining of T-cell markers can be variable, but as the name implies the epidermotropic lymphocytes consistently display a “pagetoid” appearance. Two variants of the disease are described: the localized type WKD and the disseminated type (Ketron-Goodman disease or KGD). We present the case of a 31 years old patient with no other known personal history, who is a dentist working with irritating chemicals such as orthophosphoric acid 36% and hydrofluoric acid 9.5%, who presented to our clinic for a rash consisting of papulo-vesicles with a diameter of 0,5-1.3 cm, intensely pruritic, firm to palpation, arranged on a diffuse erythematous background, located on the left hand on metacarpophalangeal (MCP) joints I-II, that had been evolving for about 1 year, with healing under local cortisone treatment but rapid relapse with local extension of lesions. Diagnosis is confirmed via a skin biopsy of the affected area followed by a cytologic examination. Histopathological appearance and immunophenotypic profile of the lymphoid infiltrate (CD3+, CD4-, CD8+) supported the diagnosis of PR, the localized form WKD. Treatment typically entails topical corticosteroids, alkylating agents, and retinoids but as an additional therapy in cases with localized form, narrowband ultraviolet B (NB-UVB) with remission of lesions and their recurrence, has also been chosen for our pacient.