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Dermatology Practical & Conceptual Feb 2022
PubMed: 35223183
DOI: 10.5826/dpc.1201a39 -
Cureus Jan 2022Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is...
Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is characterized by different subtypes based on clinical presentation and immunophenotyping studies. We aimed to study the clinical patterns and treatment response in cases of MF among the patients attending a tertiary referral hospital in Saudi Arabia. Methodology A retrospective study, case record-based study was done to review all the patients diagnosed with MF from January 2011 to May 2016. All cases with histopathological confirmation and immunophenotyping were included in the study. Treatment follow-up was reviewed for 9 months in all cases. Treatment response was graded based on a global physician assessment-complete response, good response, moderate response, and minimal or no response. Results Out of 34 cases of MF included in the study, 11 were hyperpigmented MF, 21 were hypopigmented MF, and there was one case each of poikilodermatous MF and pagetoid reticulosis. Of the total, fourteen (66.7%) of hypopigmented MF patients showed a complete response to phototherapy Narrowband UVB (NB-UVB) in combination with topical corticosteroids. Nine (81.8%) of hyperpigmented MF patients showed partial to the phototherapy NB-UVB in combination with the topical corticosteroid. Among the other types; one case of poikilodermatous MF (2.9%) showed a moderate response to phototherapy NB-UVB with topical corticosteroid and systemic acitretin. Conclusions The most common type of MF seen in our study was the hypopigmented type, affecting a younger age group, and the same showed a good response to phototherapy NB-UVB combined with topical corticosteroids.
PubMed: 35174031
DOI: 10.7759/cureus.21231 -
Photodiagnosis and Photodynamic Therapy Mar 2022A 70-year-old male presented with reddish brown, keratotic plaque on his left leg which had grown slowly for ten years. The histopathological examination revealed...
A 70-year-old male presented with reddish brown, keratotic plaque on his left leg which had grown slowly for ten years. The histopathological examination revealed acanthosis and bands of lymphocytes with significant epidermotropism. The intraepidermal lymphocytes showed large, pleomorphic and hyperchromatic nuclei with distinct nucleoli and perinuclear halos, positive for CD3, CD4 and CD30, negative for CD8, CD20 and high in Ki67 index. Accordingly, the diagnosis of Pagetoid reticulosis was made. The patient was then treated with topical photodynamic therapy once a week sequential to partial in-situ resection and preliminary skin dermabrasion and finally achieved complete remission without obvious scars or recurrence after four sessions.
Topics: Aged; Humans; Male; Pagetoid Reticulosis; Photochemotherapy; Skin; Skin Neoplasms
PubMed: 34838698
DOI: 10.1016/j.pdpdt.2021.102649 -
Cureus Oct 2021Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic...
Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic manifestations. Pagetoid reticulosis commonly presents as a localized slow-growing or indolently manifesting hyperkeratotic patch/plaque on the extremities that is confined to the epidermis. Diagnosis is confirmed via a skin biopsy of the affected area followed by a cytologic examination. Treatment typically entails topical corticosteroids, alkylating agents, and retinoids. With disseminated disease, a multidisciplinary approach involving chemotherapy and radiation is necessary. To raise awareness about pagetoid reticulosis, its diagnosis, and management, we report a case localized to the left forehead and earlobe of a 57-year-old female.
PubMed: 34754679
DOI: 10.7759/cureus.18524 -
Journal of Pediatric Hematology/oncology Oct 2022Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional...
BACKGROUND
Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional data to better understand the clinicopathologic features of MF in children.
MATERIALS AND METHODS
This study was a retrospective analysis of pediatric MF patients (diagnosed at age 0 to 18 y).
RESULTS
Thirteen pediatric patients with MF were identified. Female predominance was observed with a ratio of 1.6:1. Median values for age of onset of skin lesions and age at the time of histologic diagnosis were 5 and 12 years, respectively. All patients had early stage (stage IA to IIA) of MF at the time of diagnosis. Hypopigmented MF comprised 77% of all study patients, followed by classic MF (15%) and pagetoid reticulosis (8%). The lower extremity (especially proximal leg) followed by trunk and upper extremity were most commonly affected sites. Seven of 9 patients who had available immunohistochemistry data showed CD8 + predominance. Five of 8 patients whose follow-up data was available, achieved complete response with narrowband ultraviolet B treatment, while 2 and 1 had near complete response and partial response, respectively.
CONCLUSIONS
Our study demonstrated female sex and CD8 + profile predominance. Hypopigmented MF constituted the majority of cases. We observed good responses with narrowband ultraviolet B treatment.
Topics: Adolescent; CD8-Positive T-Lymphocytes; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Mycosis Fungoides; Retrospective Studies; Skin Diseases; Skin Neoplasms
PubMed: 34699461
DOI: 10.1097/MPH.0000000000002354 -
International Journal of Dermatology Apr 2022Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent cutaneous lymphomas. They were not described in a large Brazilian cohort yet. We aimed, with this...
BACKGROUND
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent cutaneous lymphomas. They were not described in a large Brazilian cohort yet. We aimed, with this single-center, retrospective cohort analysis, to describe the characteristics and outcomes of MF/SS in a tertiary public health service in Brazil.
METHODS
MF/SS patients evaluated at the University of São Paulo Medical School between 1989 and 2018 were included. Data were collected at diagnosis. Demographic, clinical, histopathological, immunopathological, molecular, laboratory, and follow-up data were analyzed.
RESULTS
Among 727 patients, 92.6% (673) were diagnosed with MF, 7.4% (54) with SS. There were 51.2% (372) of males, 48.8% (355) of females. The median age was 51.8 years; it was higher in erythrodermic MF (60.2) and SS (60.9). Among MF, 41.8% (281) had classic MF, 4.9% (33) folliculotropic MF, 1.8% (12) granulomatous slack skin, and 0.3% (2) pagetoid reticulosis. Common subtypes included erythrodermic (14.1%, 95), hypopigmented (10.8%, 73), and poikilodermatous MF (10.8%, 73). Extracutaneous involvement was rare. Five, 10, 20, and 30-year overall survival rates were 97.3%, 92.4%, 82.6%, and 82.6% for early-stage, and 58.6%, 42.7%, 20.8%, and 15.4% for advanced-stage disease, respectively. After multivariate analysis, SS diagnosis, folliculotropic MF, erythrodermic MF, clinical stage, age (≥60 years), increased lactate dehydrogenase, and large cell transformation conferred poorer prognosis.
CONCLUSIONS
We observed a higher percentage of hypopigmented MF compared to the literature, and demographic (older age) and prognostic (poorer prognosis) similarities between erythrodermic MF and SS, suggesting a possible relationship between these erythrodermic lymphomas. Factors associated with a poorer prognosis were compatible with the literature.
Topics: Brazil; Cohort Studies; Female; Humans; Male; Middle Aged; Mycosis Fungoides; Neoplasm Staging; Prognosis; Retrospective Studies; Sezary Syndrome; Skin Neoplasms
PubMed: 34435672
DOI: 10.1111/ijd.15865 -
Veterinary and Comparative Oncology Mar 2022Canine cutaneous lymphoma is an uncommon lymphoma in dogs. Most canine cutaneous lymphoma cases have a T-cell origin. Canine cutaneous T-cell lymphoma (CTCL) is...
Canine cutaneous lymphoma is an uncommon lymphoma in dogs. Most canine cutaneous lymphoma cases have a T-cell origin. Canine cutaneous T-cell lymphoma (CTCL) is classified into epitheliotropic and nonepitheliotropic cutaneous lymphomas, and each type of lymphoma is subclassified into several histological subtypes. Limited information is available regarding the prognostic significance of clinical variables and histopathological subtypes in dogs with CTCL. This retrospective study aimed to investigate the influence of clinical variables and histopathological subtypes on the prognosis of dogs with CTCL. Forty-six dogs diagnosed with CTCL by histopathological examination were included. Histopathological specimens were reexamined and classified into CTCL subtypes. The influence of the type of skin lesion, histopathological subtype, haematological examination results and treatment response on the overall survival time (OS) was examined. Thirty-one dogs were diagnosed with epitheliotropic CTCL (mycosis fungoides in 28 dogs; pagetoid reticulosis in 3 dogs) and 15 dogs were diagnosed with nonepitheliotropic CTCL (anaplastic large T-cell lymphoma in 6 dogs; peripheral T-cell lymphoma, not otherwise specified, in 9 dogs). The OS of dogs diagnosed with epitheliotropic CTCL (141 days) was significantly shorter than that of dogs diagnosed with nonepitheliotropic CTCL (374 days). As clinical variables, the presence of neoplastic lymphocytes in peripheral blood, thrombocytopenia and initial chemotherapeutic response was related to prognosis. Our results demonstrated that histopathological subtype and several clinical variables were found to influence the prognosis of dogs with CTCL.
Topics: Animals; Dog Diseases; Dogs; Lymphoma, Non-Hodgkin; Lymphoma, T-Cell, Cutaneous; Prognosis; Retrospective Studies; Skin Neoplasms
PubMed: 34250731
DOI: 10.1111/vco.12752 -
Journal of Cutaneous Pathology Dec 2021Primary cutaneous CD30+ lymphoproliferative diseases are the second most common group of cutaneous T-cell lymphomas, including lymphomatoid papulosis (LyP), primary... (Review)
Review
BACKGROUND
Primary cutaneous CD30+ lymphoproliferative diseases are the second most common group of cutaneous T-cell lymphomas, including lymphomatoid papulosis (LyP), primary cutaneous anaplastic large-cell lymphoma (pcALCL), and borderline cases. These diseases form a spectrum and may show overlapping histopathological, phenotypic, and genetic features. In the 2016 WHO classification, LyP with 6p25.3 rearrangement was introduced as a rare new subtype of LyP and showed distinctive clinicopathological features. The striking biphasic histopathologic pattern presented with larger transformed lymphocytes diffusely infiltrating the dermis and smaller atypical cells infiltrating the epidermis as in pagetoid reticulosis.
METHODS
Herein we report two cases of pcALCL with rearrangement involving the DUSP22-IRF4 locus on 6p25.3 that show the same particular biphasic histopathologic pattern. We review the literature regarding five similar reported cases and discuss the clinical, pathologic immunotype and follow-up features.
RESULTS
Our findings suggest that the biphasic histopathologic pattern is not a unique characteristic of LyP with 6p25.3 rearrangement.
CONCLUSION
Cutaneous CD30+ lymphoproliferative diseases with 6p25.3 rearrangement may have the same biphasic histopathological pattern and favorable prognosis, although a variety of clinical manifestations ranging from LyP to pcALCL and even anaplastic lymphoma kinase negative systemic ALCL with secondary cutaneous involvement may be observed.
Topics: Aged; Chromosomes, Human, Pair 6; Dual-Specificity Phosphatases; Gene Rearrangement; Humans; Interferon Regulatory Factors; Lymphoma, Primary Cutaneous Anaplastic Large Cell; Male; Middle Aged; Mitogen-Activated Protein Kinase Phosphatases; Skin Neoplasms
PubMed: 34164830
DOI: 10.1111/cup.14089 -
International Journal of Dermatology Sep 2021
Topics: Humans; Leukemia-Lymphoma, Adult T-Cell; Pagetoid Reticulosis; Skin; Skin Diseases; Skin Neoplasms
PubMed: 33559154
DOI: 10.1111/ijd.15446 -
Journal of the American Podiatric... Nov 2020Woringer-Kolopp disease is a rare variant of mycosis fungoides, a type of cutaneous T-cell lymphoma. Described is a case of a small annular plaque on the foot diagnosed...
Woringer-Kolopp disease is a rare variant of mycosis fungoides, a type of cutaneous T-cell lymphoma. Described is a case of a small annular plaque on the foot diagnosed histologically as Woringer-Kolopp disease and treated successfully with topical and intralesional steroids. In addition, a brief review of the literature and treatment options is provided.
Topics: Humans; Mycosis Fungoides; Pagetoid Reticulosis; Skin Neoplasms
PubMed: 33301589
DOI: 10.7547/18-029