Pagetoid dyskeratosis in dermatopathology

Abstract Currently, pagetoid dyskeratosis is believed to involve an accelerated keratinization process, possibly induced by mechanical trauma. It represents, in almost its totality, incidental histological findings of specific cells, except when it occurs in the hands, where it usually occurs simultaneously with skin lesions and local dyschromia. These are large, rounded keratinocytes, with pale cytoplasm and a pyknotic nucleus surrounded by a clear halo, which can be easily mistaken by other skin diseases. Its etiology is not completely elucidated, and the correct identification of this entity can be of great importance in the differential diagnosis of skin disorders and the understanding of the keratinization process of the epidermis.

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.

Pagetoid Bowen Disease Initially Misdiagnosed as Ectopic Extramammary Paget's Disease

Pagetoid Bowen disease is a histological variant of Bowen disease which demonstrates large pale staining cells (pagetoid cells). It requires differential diagnosis from other cutaneous malignancies with similar patterns, such as extramammary Paget's disease (EMPD) and Pagetoid melanoma in situ. Herein, we report a case of Pagetoid Bowen disease which was initially misdiagnosed as ectopic EMPD.

An Unusual Case of Sebaceous Gland Carcinoma of the Upper Eyelid - A Case Report

Sebaceous gland carcinoma of the eyelid is an aggressive malignant tumor derived from adnexal epithelium of sebaceous glands. Sebaceous gland carcinoma took the second place of all malignant eyelid tumors and occurred in 19.1%. The upper lid involved by sebaceous gland carcinoma is 54.6% and has a predilection 3 times more than the lower lid. It presents as a poorly differentiated lesion[1], which suggests a possibility of misdiagnosis because of it similarities to basal or squamous cell carcinoma. A 55-year-old male presented with a left upper eyelid swelling with an ulcerated wound over the lateral 2/3rd of the upper eyelid. A differential diagnosis of sebaceous gland carcinoma, squamous cell carcinoma and basal cell carcinoma of the eyelid was made. The X-ray orbit showed that there is a homogenous soft tissue density lesion along the lateral aspect of orbit on left side and a bony irregularity seen along the lateral margin of orbit on left side. B-scan showed mixed echogenic lesions in the subcutaneous plane involving the upper eyelid. A wide excision with lower lid switch and cheek rotation flap was performed. The diagnosis of sebaceous gland carcinoma was confirmed on histopathological findings. An early diagnosis and appropriate treatment may decrease the long-term morbidity. It may also extend the survival rates of such patients.

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.

Primary Sebaceous Carcinoma of the Corneaoscleral Limbus With Pagetoid Recurrence

We report a sebaceous carcinoma confined to the corneoscleral limbus without involvement of the eyelid. A 60-year-old man, who showed multiple masses on the corneaoscleral limbus and limbal ulceration but with normal eyelids, underwent surgical en-bloc excision of the masses. Histopathologic examination revealed a sebaceous carcinoma. Three weeks after excision, multiple pagetoid recurrences were found along the bulbar conjunctiva 2 mm away from the limbus. After the application of topical mitomycin C, the pagetoid spread regressed completely. After a 2 year follow-up, no other local or systemic recurrences were observed. This report shows that the ulcerative mass which is confined to only the corneoscleral limbus may be a sebaceous carcinoma even without eyelid involvement. Topical mitomycin C may be effective for treating pagetoid spread of sebaceous carcinoma of limbal origin.

A Case of pigmented pagetoid Bowen's disease / 대한피부과학회지

Bowen's disease is an intraepidermal squamous cell carcinoma also referred to as a squamous carcinoma in situ. Pigmented Bowen's disease is a rare variant and represents less than 2 % of all lesions. Clear cell Bowen's disease, also known as pagetoid Bowen's disease is also rare and characterized by an atypical proliferation of pale tumor cells within the epidermis. We report an unusual case of Bowen's disease in a 50-year-old man which presented as a brown to black colored scaly plaque with crusts on the back. The histopathologic feature of the skin lesion showed Bowen's disease with increased basal pigmentation, dermal melanophages and epidermal clear cells. So we termed this case 'pigmented pagetoid Bowen's disease'

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.

Multiple pagetoid Bowen's disease

A 61-year-old male was presented with oozing crusted erythematous skin lesions on the ex-tensor site of the right forearm, the extensor site of the left forearm, left calf, right shin, and the right portion of the buttock. He was diagnosed as having multiple pagetoid Bowen's disease by H&E staining, special staining and by an electron microscopic examination. He was treated with a topical application of 5-flurouracil cream and cryosurgery. The skin lesions were mostly healed after 7 months. We herein report a case of multiple pagetoid Bowen's disease on the sun-protected area that may be caused by arsenic ingestion.

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.