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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 -
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 -
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 -
Anais Brasileiros de Dermatologia 2013Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded...
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.
Topics: Biopsy; Humans; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Pagetoid Reticulosis; Skin Neoplasms; Time Factors; Treatment Outcome
PubMed: 24173195
DOI: 10.1590/abd1806-4841.20132258 -
The Journal of Clinical and Aesthetic... Oct 2010Pagetoid reticulosis is a rare variant of mycosis fungoides. This rare condition typically presents as a solitary plaque located on the extremities with an indolent...
BACKGROUND
Pagetoid reticulosis is a rare variant of mycosis fungoides. This rare condition typically presents as a solitary plaque located on the extremities with an indolent clinical course (Woringer-Kolopp disease) or as a more generalized presentation with diffuse cutaneous involvement and a more aggressive clinical course (Ketron-Goodman disease).
PURPOSE
To review the cutaneous manifestations, pathology, and treatment of localized pagetoid reticulosis.
METHODS
The authors describe a 24-year-old woman with a slowly enlarging, localized plaque of seven months duration, representing the localized form of pagetoid reticulosis with CD8+ immunophenotype.
RESULTS
The histological, immunohistochemical, and clinical features of the patient's skin lesion were characteristic for a diagnosis of Woringer-Kolopp disease. Systemic work-up for lymphoma was negative.
CONCLUSION
Woringer-Kolopp disease is most commonly seen in middle-aged men as a solitary lesion of the extremities, and it should always be considered in the differential diagnosis when a patient presents with such a lesion. A histological analysis demonstrated atypical lymphocytes preferentially localized to the epidermis with a CD4+, CD8+, or CD4-/CD8- phenotype. The treatment of choice for a solitary lesion may be localized radiation therapy, but newer therapies, such as bexarotene, may warrant further investigation.
PubMed: 20967196
DOI: No ID Found -
Dermatology Research and Practice 2010Solitary pagetoid reticulosis, also known as Woringer-Kolopp disease, is a rare subtype of cutaneous T-cell lymphoma. The typical clinical presentation is a solitary,...
Solitary pagetoid reticulosis, also known as Woringer-Kolopp disease, is a rare subtype of cutaneous T-cell lymphoma. The typical clinical presentation is a solitary, localized psoriasiform or hyperkeratotic plaque or tumor located on the extremities. It primarily affects middle-aged males. Because the clinical features of pagetoid reticulosis are indistinctive, pagetoid reticulosis may progress for years before accurate diagnosis. We reported a 57-year-old Japanese woman who presented with a 1-year history of a solitary erythematous plaque on the left leg. Dermoscopic features simulated Bowen's disease showing dotted and glomerular vessels, whitish scaly areas, and a broad negative network. Dermoscopic features of pagetoid reticulosis have never been reported. We have discussed the diagnostic significance of the observed dermoscopic findings.
PubMed: 20706637
DOI: 10.1155/2010/850416 -
The American Journal of Surgical... Dec 2009The major differential diagnosis for a primary cutaneous T-cell lymphoproliferative disorder with CD30 (Ki-1) positivity includes primary cutaneous anaplastic large cell...
The major differential diagnosis for a primary cutaneous T-cell lymphoproliferative disorder with CD30 (Ki-1) positivity includes primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis, pagetoid reticulosis and transformed mycosis fungoides (MF). Little is known, however, about CD30 expression in nontransformed MF, whether it simply reflects the proliferative fraction and if either CD30 staining or the proliferative fraction are of prognostic significance. Therefore, 47 nontransformed MF biopsies were stained for CD30 and Ki-67. The proportions of positive cells were determined and correlated with each other as well as with age, stage at diagnosis, maximum stage and survival. All cases had at least rare dermal CD30-positive cells. Higher percentages of dermal CD30 and Ki-67-positive cells were associated with a higher stage at diagnosis, and together with epidermal CD30, associated with a higher maximum stage. The proportion of CD30 and Ki-67-positive cells did not correlate with each other. Survivals were shorter if the dermal CD30 or epidermal or dermal Ki-67% were greater than the median (4.7%, 14%, 13%) and in patients of greater than or equal to 60 years of age or with a high stage. Dermal Ki-67 as a continuous variable was an independent prognostic indicator (P<0.001), as were dermal Ki-67 (P=0.004) and dermal CD30 (P=0.027) when analyzed as dichotomous variables but not stage. Therefore, CD30 expression is not restricted to transformed MF but higher levels of dermal CD30 expression and, even more so, dermal Ki-67 levels are independent adverse prognostic indicators.
Topics: Adult; Age Factors; Aged; Aged, 80 and over; Biopsy; Cell Proliferation; Disease Progression; Female; Humans; Immunohistochemistry; Kaplan-Meier Estimate; Ki-1 Antigen; Ki-67 Antigen; Male; Middle Aged; Mycosis Fungoides; Neoplasm Staging; Proportional Hazards Models; Risk Assessment; Risk Factors; Skin Neoplasms; Time Factors; Treatment Outcome
PubMed: 19898220
DOI: 10.1097/PAS.0b013e3181bf677d -
Case Reports in Dermatology Sep 2009Pagetoid reticulosis (PR) is a rare form of cutaneous T-cell lymphoma [Mod Pathol 2000;13:502-510]. Two variants of the disease are described: the localized type...
Pagetoid reticulosis (PR) is a rare form of cutaneous T-cell lymphoma [Mod Pathol 2000;13:502-510]. Two variants of the disease are described: the localized type Woringer-Kolopp disease (WKD) and the disseminated type Ketron-Goodman disease (KGD). KGD may have disseminated lesions, high rate of recurrence and a guarded prognosis [Mod Pathol 2000;13:502-510]. In patients with KGD, therefore, long-term observation is necessary. Disappearance of cutaneous lesions does not mean resolution of the disease [J Am Acad Dermatol 2002;47:183-186]. Herein we report the case of an 84-year-old man with erythematous patches of the trunk and the upper and lower extremities in whom the diagnosis of KGD was made. We describe this case for the rarity of this pathology and for the good response to therapy (IFN).
PubMed: 20652112
DOI: 10.1159/000236057 -
Journal of the American Academy of... Apr 2008We present a rare case of pagetoid reticulosis arising in a 5-year-old white boy. He had a history of a large chronic erythematous, scaly patch on his left buttock that...
We present a rare case of pagetoid reticulosis arising in a 5-year-old white boy. He had a history of a large chronic erythematous, scaly patch on his left buttock that had shown intermittent partial response to a topical antifungal medication. A punch biopsy specimen revealed dramatic epidermal hyperplasia, with parakeratosis and prominent exocytosis of single and clustered mononuclear cells (Pautrier's microabscesses) into the epidermis. Some of these exhibited hyperchromatic nuclei with irregular contours. They stained prominently for CD3, CD4, and CD8, with a predominance of CD8(+) cells. T-cell receptor gene rearrangement by polymerase chain reaction was negative for a clonal process on a second biopsy specimen that was nondiagnostic on routine sections. Pagetoid reticulosis is an indolent, unilesional variant of mycosis fungoides, in which the atypical T cells may express a CD4(-)/CD8(+) phenotype. This is in contrast to primary cutaneous epidermotropic CD8(+) cytotoxic T-cell lymphoma, which is often very aggressive with a poor outcome. Pagetoid reticulosis is exceedingly rare in children and adolescents. Two features predict a benign course in this 5-year-old child: the unilesional clinical presentation and the CD8 predominance of the epidermal lymphocytes.
Topics: CD8 Antigens; Child, Preschool; Electrons; Humans; Male; Mycosis Fungoides; Skin Neoplasms
PubMed: 18342715
DOI: 10.1016/j.jaad.2007.07.030 -
Dermatology Online Journal Jan 2008Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one...
Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one or several scaly patches and plaques with an acral distribution. We present a case of a 58-year-old woman, otherwise healthy, with a 5-month history of asymptomatic, hyperkeratotic plaques on the hands and feet. Histological and immunohistochemical analysis confirmed the diagnosis. Nearly complete spontaneous regression was noted 7 months after the initial examination. After 5 years no evidence of the disease remained.
Topics: Female; Foot Diseases; Hand; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous; Middle Aged; Skin Neoplasms
PubMed: 18319035
DOI: No ID Found