Reticulose pagetoide em um cão: relato de caso / Pagetoid reticulosis in a dog: case report

Reticulose pagetoide é a expressão utilizada para uma das mais raras formas de linfoma descritas em cães. Trata-se de um distúrbio linfoproliferativo cutâneo que emerge de linfócitos T intraepidérmicos. Descreve-se aqui um caso de reticulose pagetoide com apresentação localizada, muito semelhante à doença de Woringer-Kolopp dos humanos, que acometeu um cão, Boxer, fêmea de 10 anos de idade. O paciente foi atendido devido ao aparecimento de um nódulo na região nasolabial, com evolução clínica de cerca de 30 dias. Histologicamente o nódulo era composto por uma proliferação linfoide intraepidérmica e anexal. Os linfócitos neoplásicos, células T confirmadas por meio da imunomarcação positiva para CD3 e negativa para CD79, eram grandes células redondas e monomórficas. Apesar de rara, a reticulose pagetoide é um distúrbio linfoproliferativo de fácil suspeita com base apenas na morfologia celular. Esse neoplasma deverá ser lembrado sempre que um padrão linfoide intraepidérmico estiver presente.(AU)

Reticulosis pagetoide is an expression used for one of the rare forms of lymphoma described in dogs. It is a cutaneous lymphoproliferative disorder that emerges from intraepidermal T-cells. We describe a case of pagetoid reticulosis with localized presentation, very similar to Woringer-Kolopp's disease in humans, which affected a 10-year-old Boxer female. The patient was seen due to the appearance of a nodule in the nasolabial region with clinical evolution of about 30 days. Histologically, it was composed of an intraepidermal and annexal lymphoid proliferation. Neoplastic lymphocytes, T-cells confirmed by CD3-positive and CD79-negative immunolabeling, were large, round, and monomorphic cells. Although rare, pagetoid reticulosis is an easily suspected lymphoproliferative disorder based only on cell morphology. This neoplasm should be remembered whenever an intraepidermal lymphoid pattern is present.(AU)

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.

Case for diagnosis / Caso para diagnostico

Childhood Granulomatous Periorificial Dermatitis is an acneiform facial rash that affects the periorificial area in children. The clinical aspectare asymptomatic 1-3 mm papules of, monomorphic, erythematous or hypopigmented in periorificial areas - mouth, nose and eyes. It's a benign and self-limited disease that heals spontaneously without scarring and specific therapy. Differential diagnoses include perioral dermatitis, granulomatous-rosacea, sarcoidosis, and lupus miliaris disseminatus faciei. We present the case of a 4-year-old boy, presenting papules in periorificials areas. Due to its low incidence and low number of publications we report the present case.

A Dermatite Periorificial Granulomatosa da Infância é erupção facial acneiforme que afeta área periorificial do segmento cefálico de pré-puberes. Consiste em pápulas assintomáticas de 1 a 3 mm, monomorfas, eritematosas ou hipopigmentadas em áreas periorificiais - boca, nariz e olhos. A doença é benigna e auto-limitada, curando sem deixar cicatriz e por regra sem terapia específica. Diagnósticos diferenciais incluem a dermatite perioral, rosácea granulomatosa, sarcoidose e lúpus miliar da face. Relata-se paciente de 4 anos, masculino, com erupção papulosa há 2 anos em áreas periorificais. Devido à sua baixa incidência e o reduzido número de publicações relata-se o presente caso.

Case for diagnosis / Caso para diagnostico

We present a case of bullosis diabeticorum. It is a rare disorder, probably underdiagnosed, associated with long-term diabetes mellitus. Its etiology remains unclear. It is characterized by tense blisters, with serous content, recurrent and spontaneous on normal skin especially in the acral regions. Displays self-limiting course. No specific laboratory tests for diagnosis of this bullous disease exist. Clinical and conservative management to prevent secondary infection reduces morbidity in diabetic patients.

Apresenta-se um caso de bulose diabeticorum, que consiste em uma desordem rara de etiologia ainda incerta, provavelmente subdiagnosticada, associada ao diabetes mellitus de longa evolução. Caracteriza-se por bolhas tensas, recorrentes, de conteúdo seroso e aparecimento espontâneo sobre pele pouco inflamada, especialmente nas regiões acrais, que evolui com curso autolimitado. Não há testes laboratoriais específicos para o diagnóstico desta bulose. O reconhecimento clínico e o manejo conservador para evitar infecção secundária reduz a morbidade nos pacientes diabéticos.

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.

A Case of Woringer Kolopp Disease Treated with Topical PUVA / 대한피부과학회지

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.

A Case of Woringer - Kolopp Disease ( Pagetoid Reticulosis ) / 대한피부과학회지

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.

A Case of Woringer-Kolopp Disease / 대한피부과학회지

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.