-
Journal of Cutaneous Pathology Dec 2021Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive peripheral T-cell lymphoma. There is evidence that patients with epidermotropic... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Primary cutaneous gamma/delta (γδ) T-cell lymphoma (PCGDTCL) is a rare, aggressive peripheral T-cell lymphoma. There is evidence that patients with epidermotropic PCGDTCL may have an improved prognosis compared with those with only dermal and/or subcutaneous involvement.
METHODS
Systematic review of the literature and application of inclusion criteria yielded 48 manuscripts detailing the cases of 104 patients.
RESULTS
Of the 104 patients, 57 were male (51.4%) and 47 were female (48.5%) Based on provided histopathologic descriptions, 57 cases (54.8%) had no epidermotropism, 47 cases (45.2%) patients demonstrated any degree of epidermotropism, and 25 cases were predominantly epidermotropic (25/104, 24%). Five-year overall survivals for patients with no epidermotropism, any epidermotropism, and predominantly epidermotropic presentation were 32.8%, 28.9%, and 40.0%, respectively (p = 0.40). The most commonly performed immunohistochemical markers were CD3, CD4, CD8, CD5, CD7, CD30, CD56, TCR beta, TCR γ, and TCR δ. There was no statistically significant difference in immunophenotype between groups. Lesion morphology was described in the majority of cases (85/104, 80.9%); most cases presented as a combination of nodules, plaques, and tumors (77.4%). Several cases had more atypical presentations, including "mycosis-fungoides-like" and ulcerated.
CONCLUSION
In PCGDTCL, neither epidermotropism nor predominantly epidermotropic phenotype predict a better prognosis. In addition, the case report literature in dermatology and dermatopathology is rich and highly valuable.
Topics: Female; Humans; Intraepithelial Lymphocytes; Lymphoma, T-Cell, Cutaneous; Male; Skin Neoplasms
PubMed: 34151455
DOI: 10.1111/cup.14082 -
Photodermatology, Photoimmunology &... Nov 2021
Topics: Humans; Lymphoma, T-Cell, Cutaneous; Mycosis Fungoides; Photochemotherapy; Skin Neoplasms
PubMed: 34013563
DOI: 10.1111/phpp.12698 -
American Journal of Clinical Pathology Aug 2021Several studies suggested that CD30 expression is a favorable prognostic marker in transformed mycosis fungoides (tMF). However, evidence in this field is still unclear.... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Several studies suggested that CD30 expression is a favorable prognostic marker in transformed mycosis fungoides (tMF). However, evidence in this field is still unclear. This systematic review and meta-analysis aimed to evaluate the prognostic significance of CD30 in tMF.
METHODS
Electronic databases were searched from their inception to June 2020 for all studies assessing the prognostic value of CD30 in tMF. Pooled hazard ratio (HR) for death was calculated; a P value less than .05 was considered significant. Inconsistency index (I2) was used to assess statistical heterogeneity among studies.
RESULTS
Seven studies with 323 patients were included. CD30 expression in tMF was significantly associated with a decreased hazard of death both on univariate (HR, 0.459; 95% confidence interval [CI], 0.319-0.660; P < .001) and multivariate analysis (HR, 0.503; 95% CI, 0.345-0.734; P < .001), and the statistical heterogeneity among studies was null in all analyses (I2 = 0%).
CONCLUSIONS
tMF cases with CD30 expression in large cells have a hazard of death two times lower than CD30-negative cases.
Topics: Biomarkers, Tumor; Humans; Ki-1 Antigen; Mycosis Fungoides; Prognosis; Proportional Hazards Models; Skin Neoplasms
PubMed: 33769436
DOI: 10.1093/ajcp/aqaa261 -
Journal of the European Academy of... Jul 2021Dermoscopy and trichoscopy are non-invasive methods used as auxiliary tools in diagnostics of different dermatoses. To date, no systematic review concerning the utility... (Review)
Review
Dermoscopy and trichoscopy are non-invasive methods used as auxiliary tools in diagnostics of different dermatoses. To date, no systematic review concerning the utility of dermoscopy and trichoscopy in the diagnostics of primary cutaneous lymphomas has been published. The aim of this study was to summarize the current state of knowledge on this topic based on systematic search of PubMed database and related references published before 8th of August 2020. Besides dermoscopic features, type of dermoscope, polarization mode, magnification, number of cases and histopathological correlation were analysed. A total of 34 records were included into the final analysis, evaluating 141 patients diagnosed with primary cutaneous T-cell lymphomas and 70 patients with primary cutaneous B-cell lymphomas. Most of the analysed records evaluated dermoscopic features (n = 206); trichoscopy was analysed in only 5 cases. Structures most commonly observed in classical mycosis fungoides (n = 108) were fine short linear vessels/linear vessels, spermatozoa-like vessels and orange-yellow patchy areas. In folliculotropic mycosis fungoides (n = 12), most frequently observed were comedonal lesions/comedo openings/central keratotic plugs and white halo around hair follicles/perifollicular accentuation. Primary cutaneous marginal zone B-cell lymphoma (n = 42) and primary cutaneous follicle centre lymphoma (n = 20) most commonly presented with salmon-coloured background and fine short/linear irregular/serpentine vessels. For other PCL, with less than 10 cases reported in the analysed records, details have been provided in the article. Most observations analysed in this systematic review rely on findings from case reports/case series (with the level of evidence V) and lack a control group. A few studies provided information concerning technical aspects of dermoscopic/trichoscopic examination. The role of dermoscopy/trichoscopy in diagnostics of cutaneous lymphomas requires further studies, especially in entities where dermoscopic features have been described in only single or a few cases. However, it seems that this practical, accessory tool in future may provide additional clues during clinical assessment.
Topics: Dermoscopy; Humans; Lymphoma, B-Cell; Lymphoma, T-Cell, Cutaneous; Male; Mycosis Fungoides; Skin Neoplasms
PubMed: 33710688
DOI: 10.1111/jdv.17219 -
JAMA Dermatology Apr 2021Comprehensive data on childhood mycosis fungoides (MF) is scarce.
IMPORTANCE
Comprehensive data on childhood mycosis fungoides (MF) is scarce.
OBJECTIVE
To describe clinical features, immunophenotypes, various treatment options, and prognosis of MF in children and adolescents.
EVIDENCE REVIEW
This systematic review searched MEDLINE via PubMed, Embase, Cochrane, and Scopus databases in October 2019. The search terms included mycosis fungoides, infant, children, and adolescent. No filter for the publication period was used, but studies written in a language other than English were excluded. Reference lists of the relevant articles were also searched manually. Case series and case reports were included if data on childhood MF were extractable. The Asan Medical Center database for cases of childhood MF was also searched. Patients were treated from January 1, 1990, to July 31, 2019, and were younger than 20 years at the time of diagnosis. The methodologic quality of the included studies was assessed with items from the Newcastle-Ottawa scale. Data were analyzed from December 9, 2019, to September 4, 2020.
FINDINGS
A total of 571 unique patients were included. The mean (SD) age at diagnosis was 12.2 (4.2) years; at onset, 8.6 (4.2) years. The female-to-male ratio was 1:1.6 (350 male patients [61.3%]). Among 522 patients with data available at diagnosis, stage 1 disease constituted 478 cases (91.6%), followed by stage 2 (39 [7.5%]) and stage 4 (5 [1.0%]). Among the 567 patients with data available, the most common variant of MF was the hypopigmented form (309 [54.5%]), followed by classic MF (187 [33.0%]). The MF lesions were predominantly the CD4+ and CD8+ immunophenotype in 99 (49.5%) and 79 (39.5%) of 200 patients, respectively. Among the treatments, narrowband UV-B was the most frequently used (150 of 426 [35.2%]). Most patients were alive with the disease (185 of 279 [66.3%]); 83 of 279 (29.8%) were in complete remission; and 11 of 279 (3.9%) had died by the last follow-up. A longer time from onset to diagnosis (hazard ratio [HR], 1.24; 95% CI, 1.06-1.45), granulomatous slack skin (HR, 12.25; 95% CI, 1.99-75.26), granulomatous MF (HR, 14.59; 95% CI, 1.31-162.00), a history of organ transplant (HR, 10.15; 95% CI, 0.98-105.37), and stage 2 disease at the time of diagnosis (HR, 10.22; 95% CI, 2.94-35.50) were associated with worse outcomes.
CONCLUSIONS AND RELEVANCE
The findings of this review suggest that there is often a significant delay until the establishment of a correct diagnosis of childhood MF, which may be detrimental to the prognosis.
Topics: Adolescent; Age Factors; Child; Female; Humans; Male; Mycosis Fungoides; Prognosis
PubMed: 33656521
DOI: 10.1001/jamadermatol.2021.0083 -
Dermatology (Basel, Switzerland) 2021Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (SMPLPD) is a provisional entity within the 2016 World Health Organization...
The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series.
BACKGROUND
Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (SMPLPD) is a provisional entity within the 2016 World Health Organization classification of primary cutaneous lymphomas. The condition is currently classified as a lymphoproliferative disorder to emphasize its benign course and discourage aggressive, systemic treatment modalities.
OBJECTIVE
To provide a relevant synthesis for the dermatological practitioner on the prevalence, presentation, and treatment of SMPLPD.
METHODS
We conducted an updated systematic literature review and a retrospective chart review of diagnosed cases of SMPLPD from 2 Canadian academic cutaneous lymphoma centers.
RESULTS
A total of 23 studies with 136 cases were extracted from the systematic review and 24 patients from our retrospective chart review. SMPLPD proved relatively common accounting for 12.5% of all cutaneous T-cell lymphomas encountered in our cutaneous lymphoma clinics, second in frequency only to mycosis fungoides. The typical clinical presentation was that of an older individual (median age 59 years) with an asymptomatic solitary lesion on their upper extremity. The most common clinical differentials were cutaneous lymphoid hyperplasia, basal cell carcinoma, and lymphoma unspecified. T follicular helper markers were reliably detected. The main treatment modalities were surgical excision, local radiation therapy, and topical or intralesional steroids. Cure was achieved in the vast majority of cases.
CONCLUSIONS
SMPLPD is an underdiagnosed T-cell lymphoma with an overtly benign clinical course. The condition has an excellent prognosis and responds well to skin-directed therapies. Practitioners should be aware of this condition to avoid aggressive systemic treatments.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; CD4-Positive T-Lymphocytes; Diagnosis, Differential; Female; Humans; Lymphoma, T-Cell, Cutaneous; Male; Middle Aged; Retrospective Studies; Skin Neoplasms; Young Adult
PubMed: 33326960
DOI: 10.1159/000511473 -
Archives of Dermatological Research Aug 2021While isotretinoin has been the gold-standard of therapy for severe acne since its approval in 1982, its anti-inflammatory properties makes it a potentially applicable...
While isotretinoin has been the gold-standard of therapy for severe acne since its approval in 1982, its anti-inflammatory properties makes it a potentially applicable and versatile therapy for a wide variety of dermatologic conditions yet to be explored. This systematic review comprehensively recounts the success of oral isotretinoin in non-acne cutaneous diseases and provide insight into future directions of isotretinoin utility. A systematic literature review was performed using PubMed. Search terms included "isotretinoin" OR "accutane" AND "skin" OR "dermatology" OR "hair" OR "nails" OR "rosacea" OR "psoriasis" OR "pityriasis rubra pilaris" OR "condyloma acuminata" OR "granuloma annulare" OR "darier's disease" OR "non-melanoma skin cancer" OR "frontal fibrosing alopecia" OR "cutaneous lupus erythematosus" OR "hidradenitis suppurativa" OR "photodamaged skin" OR "skin aging" OR "wart" OR "flat warts" OR "plane warts" OR "lichen planus" OR "dissecting cellulitis" OR "folliculitis decalvans" OR "sebaceous hyperplasia" OR "cutaneous t-cell lymphoma" OR "mycosis fungoides." A total of 169 studies discuss the use of oral isotretinoin for 16 non-acne dermatologic conditions, the most common being non-melanoma skin cancers (0.2-8.2 mg/kg/day), cutaneous T-cell lymphomas (0.5-2 mg/kg/day), and rosacea (0.22-1 mg/kg/day). Inflammatory conditions such as rosacea, granuloma annulare, and hidradenitis suppurativa benefit from lower oral isotretinoin dosage of 0.3-1 mg/kg/day, whereas, hyperkeratotic diseases such as psoriasis and pityriasis rubra pilaris, consistently respond better to higher dosages of up to 2-4 mg/kg/day for lesion clearance. Recurrence of disease following discontinuation of isotretinoin have been reported for rosacea, psoriasis, granuloma annulare, Darier's disease, dissecting cellulitis, and non-melanoma skin cancers. Disease exacerbation was reported in some patients with hidradenitis suppurativa. Off-label isotretinoin is an effective treatment choice for dermatological conditions beyond acne. Further prospective, randomized human trials are needed to clarify when and how to prescribe off-label isotretinoin for maximum efficacy and safety.
Topics: Administration, Oral; Dermatologic Agents; Dose-Response Relationship, Drug; Humans; Isotretinoin; Off-Label Use; Skin Diseases; Treatment Outcome
PubMed: 33151346
DOI: 10.1007/s00403-020-02152-4 -
Cancers Oct 2020Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack...
Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack information from a global perspective. A systematic review and meta-analysis was conducted in Medline and the Cochrane Library based on a previously registered protocol and according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA). We selected publications that enrolled at least 100 patients with primary cutaneous lymphomas according to the current classifications. The relative frequencies (proportions) of subtypes were compared between studies and geographic regions in a meta-analysis. In total, 26 studies met our inclusion criteria, reporting on altogether 16,953 patients. Within primary cutaneous lymphomas, CTCL appeared to be 15% more frequent in Asian populations. Mycosis fungoides (MF) accounted for 62% of CTCL, with an important heterogeneity in frequencies between studies and continents. The proportion of Sézary syndrome (SS) was 3%, stable worldwide. Rare CTCL, such as NK/T-cell lymphoma or subcutaneous panniculitis-like lymphoma, were more frequent in Asian studies. This global meta-analysis of CTCL confirmed the predominance of CTCL among primary cutaneous lymphomas (83% on average) in the three analyzed continents, most of which were MF cases. It revealed the same proportions of SS across continents, and the heterogeneity of MF frequencies, suggesting the possible role of environmental factors in the pathophysiology of the latter. Registration number: CRD42020148295 (PROSPERO).
PubMed: 33050643
DOI: 10.3390/cancers12102921 -
Journal of Cutaneous Pathology Apr 2021The surface protein CD30 is a therapeutic target of monoclonal antibody therapy. Knowledge of the frequency of CD30 expression and its prognostic relevance is therefore...
BACKGROUND
The surface protein CD30 is a therapeutic target of monoclonal antibody therapy. Knowledge of the frequency of CD30 expression and its prognostic relevance is therefore interesting, not only in lymphoproliferative disorders (LPD) but also in solid tumors of the skin.
METHODS
A review was completed in PubMed for all published reports of CD30 expression in cutaneous lymphomas, mastocytosis, epithelial tumors and sarcomas from 1982 to April 2019. Only accessible articles in English and German were considered. Entities with an expected CD30 expression, such as CD30-positive LPD, were not evaluated.
RESULTS
The electronic research identified 1091 articles and a further 34 articles were obtained from manual bibliographic reference. Overall 91 articles were included that examined CD30 expression in various entities of cutaneous neoplasms and matched the inclusion criteria.
CONCLUSION
Apart from cutaneous CD30-positive LPD, the best-studied group for CD30 expression was mycosis fungoides (MF). CD30 positivity was found in 32% of classical (patch and plaque stage) and in 59.4% cases of transformed MF. CD30 was also frequently expressed in cutaneous mastocytosis (96.5%). In solid tumors, some single reports describe CD30 expression by tumor cells, but CD30-reactive lymphocytes were frequently observed in the tumor microenvironment (TME), especially in keratoacanthoma (KA).
Topics: Carcinoma; Humans; Immunotherapy; Keratoacanthoma; Ki-1 Antigen; Lymphoma, T-Cell, Cutaneous; Lymphoproliferative Disorders; Mastocytosis; Mycosis Fungoides; Prognosis; Sarcoma; Skin Neoplasms; Tumor Microenvironment
PubMed: 33047376
DOI: 10.1111/cup.13894 -
Journal Der Deutschen Dermatologischen... Dec 2020The association between Borrelia burgdorferi and primary cutaneous lymphoma is still unclear. This systematic review and meta-analysis aims to define the association of... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The association between Borrelia burgdorferi and primary cutaneous lymphoma is still unclear. This systematic review and meta-analysis aims to define the association of Borrelia burgdorferi with primary cutaneous lymphoma and its different entities.
METHODS
Electronic databases were searched for all studies that assessed the presence of Borrelia burgdorferi DNA in specimens of primary cutaneous lymphoma. The association between Borrelia and primary cutaneous lymphomas was assessed with an odds ratio (significant p < 0.05); cutaneous specimens with no lymphoproliferative disorders were used as controls. A secondary analysis was performed to assess the prevalence of Borrelia infection in different lymphoma entities.
RESULTS
Ten studies with 506 primary cutaneous lymphomas and 201 controls were included. The prevalence of Borrelia DNA positivity was highly heterogeneous among studies from different regions. Borrelia DNA positivity was significantly associated with primary cutaneous lymphomas (odds ratio = 10.88; p < 0.00001). The prevalence of Borrelia DNA positivity was similar among different entities (marginal zone: 7.3 %; follicular: 8.1 %; diffuse large B-cell: 7.5 %; mycosis fungoides: 8 %).
CONCLUSIONS
Borrelia burgdorferi is significantly associated with primary cutaneous lymphomas, with no differences among the several lymphoma entities (both B-cell and T-cell), but with strong geographical differences. Molecular testing for Borrelia would be justified in patients with primary cutaneous lymphoma from endemic areas.
Topics: Borrelia Infections; Borrelia burgdorferi; DNA, Bacterial; Humans; Lymphoma, B-Cell; Lymphoma, T-Cell, Cutaneous; Skin Neoplasms
PubMed: 33029842
DOI: 10.1111/ddg.14289