Woringer-Kolopp disease is a rare variant of mycosis fungoides and it is also known as localized pagetoid reticulosis. It presents as a solitary, localized hyperkeratotic patch or plaque on the extremities with a slowly progressive course. A 51-year-old female presented with a 6-year history of a well-defined erythematous plaque with an indurate border on the left leg. The lesion was asymptomatic and it had gradually enlarged. A skin biopsy showed hyperkeratosis and acanthotic epidermis with an infiltration of atypical pagetoid cells, and these cells were revealed to be the cytotoxic T cell phenotype on immunohistochemical staining. We report here on a case of Woringer-Kolopp disease along with a review of the relevant literature.
Biopsy ; Epidermis ; Extremities ; Female ; Humans ; Leg ; Middle Aged ; Mycosis Fungoides ; Pagetoid Reticulosis ; Phenotype ; Skin

A 26-year-old Korean man had an erythematous scaly plaque on his right thigh for fifteen years. He was diagnosed with chronic eczema and treated with topical steroid, but no clinical improvement was obtained for 15 years. Laboratory studies including complete blood cell count, urinalysis, liver and renal function test, and peripheral blood smear were normal. A biopsy specimen showed hyperkeratosis, acanthosis, and mononuclear cellular infiltration in the epidermis. These cells had a perinuclear halo and showed a Pautrier's microabscess like configuration. In the dermis, there was a band like infiltration of inflammatory cells. Infiltrates of the epidermis were negative for leukocyte common antigen(CD45) and positive for Pan T cell(CD45RO). He was treated with topical PUVA twice a week for twelve weeks. Clinical and pathological improvements were obtained. We propose PUVA may be a useful therapeutic modality to treat Woringer Kolopp disease.
Adult ; Biopsy ; Blood Cell Count ; Dermis ; Eczema ; Epidermis ; Humans ; Leukocytes ; Liver ; Pagetoid Reticulosis* ; Thigh ; Urinalysis

Pagetoid reticulosis(PR) is a rare lymphoproliferative disorder that has been divided into the localized and disseminated type. There has been ongoing debate about the nature of PR. Some authors have regarded PR as a variant of mycosis fungoides(MF). However recent reported cases suggest that PR may represent a spectrum of lymphoproliferative disorders that may not be related to MF. We report a case of a 65-year-old woman who had an erythematous plaque on the anterior chest, buttock and lower extremities for 2 years. Histologically there was dense infiltrate of predominantly CD8-/CD30+ atypical mononuclear cells in the epidermis that produce a pagetoid appearance. We believe this is the first reported case of disseminated pagetoid reticulosis in the Korean literature.
Aged ; Buttocks ; Epidermis ; Female ; Humans ; Lower Extremity ; Lymphoproliferative Disorders ; Pagetoid Reticulosis* ; Thorax

Woringer-Kolopp disease, so called pagetoid reticulosis, is most likely represent a chronic localized from of reticulosis. A 24-year old male patient visited with asymptomatic slowly enlarging, scaly single patch on his left thigh for 10 years duration. Histopathologic studies were taken with H-E stain, D-PAS, transmission electronmicroscopy and monoclonal antibody(OK6) tissue stain. Infiltration of atypical cells showed in the lower portion of epidermis like pagetoid patterned, none in dermis. It seems to be that the cell in this case probably originated from the stimuIated T-lymphocyte.
Dermis ; Epidermis ; Humans ; Male ; Pagetoid Reticulosis* ; T-Lymphocytes ; Thigh ; Young Adult

Woringer-Kolopp(W-K) disease is a rare, localized, histologically malignant, but clinically indolent lymphoproliferative disorder. It usually shows only a single slowly enlarging skin lesion mainly on the extremities. Some authors have regarded W-K disease as a variant of mycosis fungoides. However, recent studies suggest that W-K disease may represent a spectrum of T cell lymphoproliferative disoreers that may not be related to mycosis fungoides. We report a case of Woringer-Kolopp disease in a 60-year-old male who presented with a solitary slowly growing tumor on his left palm for 3 years. Histopathologic examination showed marked acanthosis and pagetoid infiltration confined to the epidermis. He was treated with 4,000 red electron beam irradiation to the area with complete resolution of the lesion. Three years later, a similar lesion appeared on his left foot dorsum. He was treated as previously with a good response and there has been no new lesion during the last 1 year follow up period.
Epidermis ; Extremities ; Follow-Up Studies ; Foot ; Humans ; Lymphoproliferative Disorders ; Male ; Middle Aged ; Mycosis Fungoides ; Pagetoid Reticulosis* ; Skin

Woringer-Kolopp disease, also known as localized pagetoid reticulosis, is a rare variant of mycosis fungoides that presents as a solitary localized hyperkeratotic patch or plaque on the extremities and follows a benign course. Effective treatments for Woringer-Kolopp disease include skin-directed therapies such as topical nitrogen mustard, high-potency topical steroids, and phototherapy. Surgical excision has been pursued in cases of small, localized lesions. A 39-year-old man presented with a 3-month history of an asymptomatic plaque on his hand. Physical examination showed a 10-mm-diameter solitary round erythematous hyperkeratotic plaque with a slightly raised edge on the dorsum of his left hand. A skin biopsy revealed that numerous atypical lymphocytes had infiltrated the upper dermis and expanded into the epidermis with a pagetoid pattern. These atypical pagetoid cells were strongly positive for CD3, CD8, and T-cell intracellular antigen-1; focally positive for CD4; and negative for CD20, CD30, and CD56. A subsequent general examination revealed no evidence of systemic involvement and the lesion was treated with surgical excision. Here we report a rare case of Woringer-Kolopp disease.
Adult ; Biopsy ; Dermis ; Epidermis ; Extremities ; Hand* ; Humans ; Lymphocytes ; Mechlorethamine ; Mycosis Fungoides ; Pagetoid Reticulosis* ; Phototherapy ; Physical Examination ; Skin ; Steroids ; T-Lymphocytes