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Journal of the European Academy of... Oct 2017Unilesional mycosis fungoides (UMF) and pagetoid reticulosis (PR) are variants of mycosis fungoides. Conventional therapy comprises surgical excision or radiotherapy,... (Review)
Review
BACKGROUND
Unilesional mycosis fungoides (UMF) and pagetoid reticulosis (PR) are variants of mycosis fungoides. Conventional therapy comprises surgical excision or radiotherapy, which may be associated with long-term side effects, especially when the lesion is located at a special site like the palms and soles. Therefore, alternative treatment options are needed to treat solitary lesions in the case of UMF or PR. Recently, topical photodynamic therapy (PDT) has been described to be an efficient and non-invasive therapeutical option with excellent clinical outcome.
OBJECTIVE
The objective of this study was to report a 43-year-old woman with plantar UMF treated successfully with PDT, and to compare our findings with the data on PDT in UMF and PR reported in the literature.
METHODS
The literature was analysed for articles on UMF and PR, respectively, treated with topical PDT. Various parameters including form of PDT and response to treatment were analysed and compared with our case.
RESULTS
A total of 24 patients were documented with 24 lesions treated with PDT, either using aminolevulinic acid (ALA) or methylaminolevulinate (MAL). In average, a single lesion was treated 4 times with PDT. In 21 cases (88%), complete response could be achieved, whereas three cases (13%) showed partial remission. None of the cases showed stable or progressive disease.
CONCLUSIONS
PDT is a safe, efficient and non-invasive therapeutical approach for the treatment of UMF and PR. It has no long-standing adverse events and therefore is of high therapeutic value especially in cases of UMF and PR located at special sites like the palms and soles. We propose to include topical PDT as therapeutic option for the treatment of UMF and PR in future guidelines on the management MF.
Topics: Administration, Topical; Adult; Aged; Child; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Photochemotherapy; Photosensitizing Agents; Treatment Outcome; Young Adult
PubMed: 28168737
DOI: 10.1111/jdv.14160 -
Dermatopathology (Basel, Switzerland) 2016Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an...
Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an intraepidermal proliferation of neoplastic T lymphocytes. Histopathologically, this disease has distinctive attributes. In this report, we present two cases of pagetoid reticulosis, compare its microscopic features to those of 'classical' mycosis fungoides, and provide a brief review of the pertinent literature.
PubMed: 27195265
DOI: 10.1159/000444660 -
Pagetoid reticulosis (epitheliotropic cutaneous T-cell lymphoma) in an adult alpaca (Vicugna pacos).Journal of Veterinary Diagnostic... Jul 2016A 9-year-old, intact female alpaca (Vicugna pacos) was presented for a second opinion with a 1-year history of nonpruritic, multifocal scaling and crusted cutaneous...
A 9-year-old, intact female alpaca (Vicugna pacos) was presented for a second opinion with a 1-year history of nonpruritic, multifocal scaling and crusted cutaneous lesions, mainly involving skin on the face, axillae, and ventral abdomen. Clinical abnormalities were limited to the skin, and the alpaca was otherwise healthy. The initial veterinarian had examined the alpaca, found no evidence of ectoparasites with laboratory testing, and had tried several trial therapies including oral antibiotics, ivermectin, and topical use of betadine solution. At the time of presentation, the lesions had neither improved nor worsened with any attempted therapy, and multiple skin biopsies were collected. Histopathology and immunohistochemical staining findings were consistent with the pagetoid reticulosis type of cutaneous epitheliotropic T-cell lymphoma. Our report describes the clinical, histopathologic, and immunophenotypic features of pagetoid reticulosis epitheliotropic cutaneous T-cell lymphoma in an alpaca.
Topics: Animals; Camelids, New World; Female; Immunophenotyping; Pagetoid Reticulosis; Skin Neoplasms
PubMed: 27154316
DOI: 10.1177/1040638716645833 -
Diagnostic Histopathology (Oxford,... Apr 2016Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant...
Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant presentations of this cutaneous T-cell lymphoma add a further layer of complexity, requiring a sophisticated and informed perspective when evaluating lymphoid infiltrates in the skin. Herein we discuss well-defined (WHO-EORTC) variants pagetoid reticulosis, granulomatous slack skin and folliculotropic mycosis fungoides as well as less well-defined morphologic/architectural variants, and divergent immunohistochemical presentations of this typically indolent T-cell lymphoproliferative disease.
PubMed: 29225700
DOI: 10.1016/j.mpdhp.2016.04.004 -
Dermatologic Therapy 2016Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of...
Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of CTCL. This report describes three female patients (mean age 66 years) with WK disease who had an effective treatment response to bexarotene 1% gel. This treatment could provide a safe alternative to other current treatment modalities which have higher risks of potential adverse effects and lack of access to other conventional treatments such as light therapy.
Topics: Administration, Cutaneous; Aged; Aged, 80 and over; Antineoplastic Agents; Bexarotene; Biomarkers, Tumor; Biopsy; Female; Gels; Humans; Immunohistochemistry; Middle Aged; Pagetoid Reticulosis; Remission Induction; Skin Neoplasms; Tetrahydronaphthalenes; Treatment Outcome
PubMed: 26332008
DOI: 10.1111/dth.12281 -
CMAJ : Canadian Medical Association... Nov 2015
Topics: Aged; Clobetasol; Diagnosis, Differential; Foot Diseases; Glucocorticoids; Humans; Male; Pagetoid Reticulosis; Psoriasis; Skin Neoplasms
PubMed: 26303240
DOI: 10.1503/cmaj.141318 -
Journal of Cutaneous Pathology Jun 2015Pagetoid reticulosis (PR) is a low-grade primary cutaneous T-cell lymphoma showing localized patches or plaques with an intrapeidermal proliferation of neoplastic...
Pagetoid reticulosis (PR) is a low-grade primary cutaneous T-cell lymphoma showing localized patches or plaques with an intrapeidermal proliferation of neoplastic T-cells with heterogeneous immunophenotype. We describe a 73-year-old woman with a 8-year history of gluteal lesions of PR, whom large blast cells were CD4/CD8 double negative T-cells with an activated cytotoxic profile. The case was investigated using a broad panel of monoclonal antibodies including TCRγM1, a new available antibody that recognizes the γ chain subunit of the T-cell receptor (TCR) in formalin-fixed paraffin-embedded tissue. Large blast cells were simultaneously positive for TCRαβ and TCRγδ with an activated cytotoxic phenotype. It is worldwide accepted the mutual exclusive expression of TCRαβ and TCRγδ but six different studies, dealing with TCRγδ expression in various types of extra-nodal lymphomas, reported cases whom tumor cells expressed simultaneously TCRαβ and TCRγδ. Our data and those of similar reports, suggest the possibility of existence of a subset of extra-nodal T-cell lymphomas showing simultaneous expression by tumor cells of TCRγδ and TCRαβ with an immunoprofile consistent with an origin from TCRγδ+ T lymphocytes. This unusual subset has preferential, but not exclusive, skin localization and variable epidermotropism.
Topics: Aged; Biopsy; Female; Humans; Immunohistochemistry; Immunophenotyping; Pagetoid Reticulosis; Polymerase Chain Reaction; Receptors, Antigen, T-Cell, alpha-beta; Receptors, Antigen, T-Cell, gamma-delta; Skin Neoplasms; T-Lymphocytes
PubMed: 25754647
DOI: 10.1111/cup.12486 -
Clinical and Experimental Dermatology Jun 2015
Topics: Adult; Female; Hand Dermatoses; Humans; Laser Therapy; Pagetoid Reticulosis; Photochemotherapy; Skin Neoplasms; Treatment Outcome
PubMed: 25645304
DOI: 10.1111/ced.12528 -
Pagetoid reticulosis Woringer-Kolopp type, a particular variant of mycosis fungoides: a case report.Romanian Journal of Morphology and... 2014According to the definition of WHO, pagetoid reticulosis (PR) is characterized by the presence of unique skin lesion in the shape of patches or plaques caused by the...
According to the definition of WHO, pagetoid reticulosis (PR) is characterized by the presence of unique skin lesion in the shape of patches or plaques caused by the proliferation of atypical T-cells in epidermis. We report the case of a 50-year-old female, presenting a 50×65 mm erythematous scaly patch, well-defined, slightly infiltrated, discrete polycyclic, on the right buttock, which appeared and developed in about 6-8 months. The clinical and dermatoscopic aspects suggested a Bowen's disease. A skin biopsy was performed. The histological examination of the pieces confirmed the presence of monoclonal lymphoid infiltrate with obvious phenomena of epidermotropism. Immuno-phenotypically the tumoral cells express intensively CD3 and CD8 and focally CD4. Surgical excision was performed without radiotherapy and chemotherapy. The patient was followed-up for 42 months without local relapses, distant lymph nodes or visceral metastases. Microscopic aspect of the lesion corresponds to stage I-II of mycosis fungoides (MF) (patches or plaques); the diagnosis was PR Woringer-Kolopp type. The most recent classification of skin lymphomas (WHO, 2008) confirms that the immunohistochemical profile of T-cells can be CD4+ and CD8- or CD4- and CD8+, and frequently CD30+, in contrast to the classical versions of MF. Pagetoid reticulosis type Woringer-Kolopp, a rare particular variant of mycosis fungoides, is suspected in cases with unique, distinctive lesion and confirmed only by histological immunohistochemistry. Treatment consists of surgical excision of the lesion with long follow-up.
Topics: Biopsy; Dermoscopy; Female; Humans; Immunohistochemistry; Middle Aged; Pagetoid Reticulosis; Skin; T-Lymphocytes
PubMed: 25611283
DOI: No ID Found -
Dermatology Online Journal Oct 2014Pagetoid reticulosis, also known as Woringer-Kolopp disease, is a rare variant of cutaneous T-cell lymphoma usually presenting as an isolated patch or plaque on the...
Pagetoid reticulosis, also known as Woringer-Kolopp disease, 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. The following case demonstrates clearance of this condition with intensity modulated radiation therapy, whereas most cases are managed with electron beam therapy if radiation therapy is implemented.
Topics: Aged, 80 and over; Humans; Male; Pagetoid Reticulosis; Radiotherapy, Intensity-Modulated; Skin Neoplasms
PubMed: 25525999
DOI: No ID Found