Pagetoid reticulosis positive for cytotoxic CD20 and CD30: A case report

Pagetoid reticulosis is an indolent cutaneous T-cell lymphoma and presents as erythema or plaque with a well-defined border on the distal areas of the extremities. Immunophenotypic studies show that in most cases, neoplastic lymphocytes are positive for CD4, whereas CD20 and CD30 double positivity was rarely reported. In this paper, we report an 80-year-old woman who presented with erythema on the extremities for 3 years. Skin biopsy on the right forearm was performed. Histopathologically, the erythematous lesions were characterized by atypical lymphocytes with significant epidermotropism. Immunohistochemical staining showed high proliferation as evidenced by high Ki-67 index and that the tumor cells were positive for CD20 and CD30 but negative for CD7 and CD56. The patient was treated with one cycle of radiotherapy and is currently doing well.

A Case of Woringer-Kolopp Disease of the Hand / 대한피부과학회지

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.

Case for diagnosis / Caso para diagnostico

Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.

A doença de Ketron-Goodman foi inicialmente considerada uma forma disseminada de reticulose pagetóide. Mas, de acordo com o atual sistema de classificação e dependendo do quadro clínico-patológico deve ser antes vista como um linfoma T CD8 agressivo epidermotrópico, linfoma T gama/delta ou micose fungóide, estadio tumoral. Pretendemos realçar esta doença rara que pode suscitar dúvidas no diagnóstico. Neste caso, a apresentação e evolução foram indolentes com boa resposta a um tratamento pouco agressivo, não se enquadrando bem nas novas propostas de classificação da doença.

A Case of Woringer-Kolopp Disease on the Leg / 대한피부과학회지

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.

A Case of Disseminated Pagetoid Reticulosis / 대한피부과학회지

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.