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1.
J Am Acad Dermatol ; 90(6): 1182-1189, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38341148

ABSTRACT

BACKGROUND: There is no established standard of care for treating central centrifugal cicatricial alopecia (CCCA), and treatment approaches vary widely. OBJECTIVE: To develop consensus statements regarding the use of various pharmacological therapies in treating adults with CCCA. METHODS: We invited 27 dermatologists with expertise in hair and scalp disorders to participate in a 3-round modified Delphi study between January and March 2023. Statements met strong consensus if 75% of respondents agreed or disagreed. Statements met moderate consensus if 55% or more but less than 75% agreed or disagreed. RESULTS: In round 1, 5 of 33 (15.2%) statements met strong consensus, followed by 9 of 28 (32.1%) in round 2. After the final round 3 meeting, strong consensus was reached for 20 of 70 (28.6%) overall statements. Two statements achieved moderate consensus. LIMITATIONS: This study included only English-speaking, US-based dermatologists and did not consider nonpharmacological therapies. CONCLUSION: Despite varying opinions among dermatologists, consensus was reached for several statements to help clinicians manage CCCA. We also highlight areas that lack expert consensus with the goal of advancing research and therapeutic options for CCCA.


Subject(s)
Alopecia , Consensus , Delphi Technique , Humans , Alopecia/therapy , Alopecia/diagnosis , Alopecia/drug therapy , Cicatrix/therapy , Cicatrix/etiology , Dermatologists
2.
JAMA Dermatol ; 160(3): 341-350, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38324292

ABSTRACT

Importance: Current measures of alopecia areata (AA) severity, such as the Severity of Alopecia Tool score, do not adequately capture overall disease impact. Objective: To explore factors associated with AA severity beyond scalp hair loss, and to support the development of the Alopecia Areata Severity and Morbidity Index (ASAMI). Evidence Review: A total of 74 hair and scalp disorder specialists from multiple continents were invited to participate in an eDelphi project consisting of 3 survey rounds. The first 2 sessions took place via a text-based web application following the Delphi study design. The final round took place virtually among participants via video conferencing software on April 30, 2022. Findings: Of all invited experts, 64 completed the first survey round (global representation: Africa [4.7%], Asia [9.4%], Australia [14.1%], Europe [43.8%], North America [23.4%], and South America [4.7%]; health care setting: public [20.3%], private [28.1%], and both [51.6%]). A total of 58 specialists completed the second round, and 42 participated in the final video conference meeting. Overall, consensus was achieved in 96 of 107 questions. Several factors, independent of the Severity of Alopecia Tool score, were identified as potentially worsening AA severity outcomes. These factors included a disease duration of 12 months or more, 3 or more relapses, inadequate response to topical or systemic treatments, rapid disease progression, difficulty in cosmetically concealing hair loss, facial hair involvement (eyebrows, eyelashes, and/or beard), nail involvement, impaired quality of life, and a history of anxiety, depression, or suicidal ideation due to or exacerbated by AA. Consensus was reached that the Alopecia Areata Investigator Global Assessment scale adequately classified the severity of scalp hair loss. Conclusions and Relevance: This eDelphi survey study, with consensus among global experts, identified various determinants of AA severity, encompassing not only scalp hair loss but also other outcomes. These findings are expected to facilitate the development of a multicomponent severity tool that endeavors to competently measure disease impact. The findings are also anticipated to aid in identifying candidates for current and emerging systemic treatments. Future research must incorporate the perspectives of patients and the public to assign weight to the domains recognized in this project as associated with AA severity.


Subject(s)
Alopecia Areata , Humans , Alopecia/diagnosis , Alopecia Areata/diagnosis , Consensus , Morbidity , Quality of Life
4.
JAMA Dermatol ; 158(9): 1031-1039, 2022 09 01.
Article in English | MEDLINE | ID: mdl-35857290

ABSTRACT

Importance: Given that mycosis fungoides-cutaneous T-cell lymphoma (MF/CTCL) is chronic, there is a need for additional therapies with minimal short- and long-term adverse effects. Topical synthetic hypericin ointment, 0.25%, activated with visible light is a novel, nonmutagenic photodynamic therapy (PDT). Objectives: To determine the efficacy and safety of topical synthetic hypericin ointment, 0.25%, activated with visible light as a nonmutagenic PDT in early-stage MF/CTCL. Design, Settings, and Participants: This was a multicenter, placebo-controlled, double-blinded, phase 3 randomized clinical trial (FLASH study) conducted from December 2015 to November 2020 at 39 academic and community-based US medical centers. Participants were adults (≥18 years) with early-stage (IA-IIA) MF/CTCL. Interventions: In cycle 1, patients were randomized 2:1 to receive hypericin or placebo to 3 index lesions twice weekly for 6 weeks. In cycle 2, all patients received the active drug for 6 weeks to index lesions. In cycle 3 (optional), both index and additional lesions received active drug for 6 weeks. Main Outcomes and Measures: The primary end point was index lesion response rate (ILRR), defined as 50% or greater improvement in modified Composite Assessment of Index Lesion Severity (mCAILS) score from baseline after 6 weeks of therapy for cycle 1. For cycles 2 and 3, open label response rates were secondary end points. Adverse events (AEs) were assessed at each treatment visit, after each cycle, and then monthly for 6 months. Data analyses were performed on December 21, 2020. Results: The study population comprised 169 patients (mean [SD] age, 58.4 [16.0] years; 96 [57.8%] men; 120 [72.3%] White individuals) with early-stage MF/CTCL. After 6 weeks of treatment, hypericin PDT was more effective than placebo (cycle 1 ILRR, 16% vs 4%; P = .04). The ILRR increased to 40% in patients who received 2 cycles of hypericin PDT (P < .001 vs cycle 1 hypericin) and to 49% after 3 cycles (P < .001 vs cycle 1 hypericin). Significant clinical responses were observed in both patch and plaque type lesions and were similar regardless of age, sex, race, stage IA vs IB, time since diagnosis, and number of prior therapies. The most common treatment-related AEs were mild local skin (13.5%-17.3% across cycles 1-3 vs 10.5% for placebo in cycle 1) and application-site reactions (3.2%-6.9% across cycles 1-3 vs 4% for placebo in cycle 1). No drug-related serious AEs occurred. Conclusion and Relevance: The findings of this randomized clinical trial indicate that synthetic hypericin PDT is effective in early-stage patch and plaque MF/CTCL and has a favorable safety profile. Trial Registration: ClinicalTrials.gov Identifier: NCT02448381.


Subject(s)
Lymphoma, T-Cell, Cutaneous , Mycosis Fungoides , Photochemotherapy , Skin Neoplasms , Adult , Anthracenes , Female , Humans , Lymphoma, T-Cell, Cutaneous/drug therapy , Lymphoma, T-Cell, Cutaneous/pathology , Male , Middle Aged , Mycosis Fungoides/pathology , Ointments/therapeutic use , Perylene/analogs & derivatives , Photochemotherapy/adverse effects , Photosensitizing Agents/adverse effects , Skin Neoplasms/pathology , Treatment Outcome
6.
J Natl Compr Canc Netw ; 20(3): 285-308, 2022 03.
Article in English | MEDLINE | ID: mdl-35276674

ABSTRACT

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorders arising from mature T cells, accounting for about 10% of non-Hodgkin lymphomas. PTCL-not otherwise specified is the most common subtype, followed by angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, anaplastic lymphoma kinase-positive, anaplastic large cell lymphoma, anaplastic lymphoma kinase-negative, and enteropathy-associated T-cell lymphoma. This discussion section focuses on the diagnosis and treatment of PTCLs as outlined in the NCCN Guidelines for T-Cell Lymphomas.


Subject(s)
Immunoblastic Lymphadenopathy , Lymphoma, T-Cell, Peripheral , Lymphoma, T-Cell , Humans , Immunoblastic Lymphadenopathy/diagnosis , Immunoblastic Lymphadenopathy/pathology , Immunoblastic Lymphadenopathy/therapy , Lymphoma, T-Cell/diagnosis , Lymphoma, T-Cell/therapy , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/therapy
7.
Blood ; 140(5): 419-437, 2022 08 04.
Article in English | MEDLINE | ID: mdl-34758074

ABSTRACT

The number of patients with primary cutaneous lymphoma (PCL) relative to other non-Hodgkin lymphomas (NHLs) is small and the number of subtypes large. Although clinical trial guidelines have been published for mycosis fungoides/Sézary syndrome, the most common type of PCL, none exist for the other PCLs. In addition, staging of the PCLs has been evolving based on new data on potential prognostic factors, diagnosis, and assessment methods of both skin and extracutaneous disease and a desire to align the latter with the Lugano guidelines for all NHLs. The International Society for Cutaneous Lymphomas (ISCL), the United States Cutaneous LymphomaConsortium (USCLC), and the Cutaneous Lymphoma Task Force of the European Organization for the Research and Treatment of Cancer (EORTC) now propose updated staging and guidelines for the study design, assessment, endpoints, and response criteria in clinical trials for all the PCLs in alignment with that of the Lugano guidelines. These recommendations provide standardized methodology that should facilitate planning and regulatory approval of new treatments for these lymphomas worldwide, encourage cooperative investigator-initiated trials, and help to assess the comparative efficacy of therapeutic agents tested across sites and studies.


Subject(s)
Lymphoma, T-Cell, Cutaneous , Mycosis Fungoides , Sezary Syndrome , Skin Neoplasms , Clinical Trials as Topic , Humans , Lymphoma, T-Cell, Cutaneous/diagnosis , Lymphoma, T-Cell, Cutaneous/pathology , Lymphoma, T-Cell, Cutaneous/therapy , Mycosis Fungoides/diagnosis , Mycosis Fungoides/pathology , Mycosis Fungoides/therapy , Neoplasm Staging , Sezary Syndrome/diagnosis , Sezary Syndrome/pathology , Sezary Syndrome/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Skin Neoplasms/therapy , United States
8.
JAMA Dermatol ; 157(4): 1-11, 2021 04 01.
Article in English | MEDLINE | ID: mdl-33656556

ABSTRACT

Importance: A recent expert consensus exercise emphasized the importance of developing a global network of patient registries for alopecia areata to redress the paucity of comparable, real-world data regarding the effectiveness and safety of existing and emerging therapies for alopecia areata. Objective: To generate core domains and domain items for a global network of alopecia areata patient registries. Evidence Review: Sixty-six participants, representing physicians, patient organizations, scientists, the pharmaceutical industry, and pharmacoeconomic experts, participated in a 3-round eDelphi process, culminating in a face-to-face meeting at the World Congress of Dermatology, Milan, Italy, June 14, 2019. Findings: Ninety-two core data items, across 25 domains, achieved consensus agreement. Twenty further noncore items were retained to facilitate data harmonization in centers that wish to record them. Broad representation across multiple stakeholder groups was sought; however, the opinion of physicians was overrepresented. Conclusions and Relevance: This study identifies the domains and domain items required to develop a global network of alopecia areata registries. These domains will facilitate a standardized approach that will enable the recording of a comprehensive, comparable data set required to oversee the introduction of new therapies and harness real-world evidence from existing therapies at a time when the alopecia areata treatment paradigm is being radically and positively disrupted. Reuse of similar, existing frameworks in atopic dermatitis, produced by the Treatment of Atopic Eczema (TREAT) Registry Taskforce, increases the potential to reuse existing resources, creates opportunities for comparison of data across dermatology subspecialty disease areas, and supports the concept of data harmonization.


Subject(s)
Alopecia Areata/epidemiology , Alopecia Areata/therapy , Registries , Alopecia Areata/diagnosis , Consensus , Delphi Technique , Humans , Internationality , Severity of Illness Index , Surveys and Questionnaires
9.
J Am Acad Dermatol ; 84(6): 1594-1601, 2021 Jun.
Article in English | MEDLINE | ID: mdl-32926985

ABSTRACT

BACKGROUND: We previously reported the Alopecia Areata Consensus of Experts study, which presented results of an international expert opinion on treatments for alopecia areata. OBJECTIVE: To report the results of the Alopecia Areata Consensus of Experts international expert opinion on diagnosis and laboratory evaluation for alopecia areata. METHODS: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Consensus threshold was set at greater than or equal to 66%. RESULTS: Of 148 questions, expert consensus was achieved in 82 (55%). Round 1 consensus was achieved in 10 of 148 questions (7%). Round 2 achieved consensus in 47 of 77 questions (61%). The final face-to-face achieved consensus in 25 of 32 questions (78%). Consensus was greatest for laboratory evaluation (12 of 14 questions [86%]), followed by diagnosis (11 of 14 questions [79%]) of alopecia areata. Overall, etiopathogenesis achieved the least category consensus (31 of 68 questions [46%]). LIMITATIONS: The study had low representation from Africa, South America, and Asia. CONCLUSION: There is expert consensus on aspects of epidemiology, etiopathogenesis, clinical features, diagnosis, laboratory evaluation, and prognostic indicators of alopecia areata. The study also highlights areas where future clinical research could be directed to address unresolved hypotheses in alopecia areata patient care.


Subject(s)
Alopecia Areata/diagnosis , Consensus , Dermatology/standards , Global Burden of Disease , Alopecia Areata/epidemiology , Alopecia Areata/etiology , Alopecia Areata/therapy , Comorbidity , Delphi Technique , Dermatology/methods , Dermoscopy , Hair Follicle/diagnostic imaging , Hair Follicle/growth & development , Hair Follicle/pathology , Humans , International Cooperation , Practice Guidelines as Topic , Prognosis , Risk Factors , Severity of Illness Index
10.
J Natl Compr Canc Netw ; 18(11): 1460-1467, 2020 11 02.
Article in English | MEDLINE | ID: mdl-33152703

ABSTRACT

Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of T-cell lymphoma associated with an aggressive clinical course and a worse prognosis. HSTCL develops in the setting of chronic immune suppression or immune dysregulation in up to 20% of cases and is most often characterized by spleen, liver, and bone marrow involvement. Diagnosis and management of HSTCL pose significant challenges given the rarity of the disease along with the absence of lymphadenopathy and poor outcome with conventional chemotherapy regimens. These Guidelines Insights focus on the diagnosis and treatment of HSTCL as outlined in the NCCN Guidelines for T-Cell Lymphomas.


Subject(s)
Lymphoma, T-Cell , Humans , Lymphoma, T-Cell/diagnosis , Lymphoma, T-Cell/epidemiology , Lymphoma, T-Cell/therapy , Practice Guidelines as Topic , Prognosis
11.
J Natl Compr Canc Netw ; 18(5): 522-536, 2020 05.
Article in English | MEDLINE | ID: mdl-32380458

ABSTRACT

Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma (CTCL), and Sézary syndrome (SS) is a rare erythrodermic and leukemic subtype of CTCL characterized by significant blood involvement. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary for the treatment of advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as romidepsin, brentuximab vedotin, and mogamulizumab. These NCCN Guidelines Insights discuss the diagnosis and management of MF and SS (with a focus on systemic therapy).


Subject(s)
Lymphoma, T-Cell, Cutaneous/pathology , Mycosis Fungoides/diagnosis , Skin Neoplasms/pathology , Guidelines as Topic , Humans , Mycosis Fungoides/pathology
12.
J Am Acad Dermatol ; 83(2): 703-704, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32305443
13.
J Am Acad Dermatol ; 83(1): 123-130, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32165196

ABSTRACT

BACKGROUND: A systematic review failed to identify any systemic therapy used in alopecia areata (AA) where use is supported by robust evidence from high-quality randomized controlled trials. OBJECTIVE: To produce an international consensus statement on the use and utility of various treatments for AA. METHODS: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Agreement of 66% or greater was considered consensus. RESULTS: In the first round, consensus was achieved in 22 of 423 (5%) questions. After a face-to-face meeting in round 3, overall, consensus was achieved for only 130 (33%) treatment-specific questions. There was greater consensus for intralesional treatment of AA (19 [68%]) followed by topical treatment (25 [43%]). Consensus was achieved in 45 (36%) questions pertaining to systemic therapies in AA. The categories with the least consensus were phototherapy and nonprescription therapies. LIMITATIONS: The study included a comprehensive list of systemic treatments for AA but not all treatments used. CONCLUSION: Despite divergent opinions among experts, consensus was achieved on a number of pertinent questions. The concluding statement also highlights areas where expert consensus is lacking and where an international patient registry could enable further research.


Subject(s)
Alopecia Areata/therapy , Administration, Oral , Administration, Topical , Adrenal Cortex Hormones/therapeutic use , Age Factors , Alopecia Areata/drug therapy , Combined Modality Therapy , Complementary Therapies , Delphi Technique , Dermatologic Agents/therapeutic use , Expert Testimony , Humans , Injections, Intralesional , Phototherapy , Severity of Illness Index , Treatment Outcome
14.
J Am Acad Dermatol ; 82(2): 412-419, 2020 Feb.
Article in English | MEDLINE | ID: mdl-31622643

ABSTRACT

BACKGROUND: There are currently no treatments for alopecia areata (AA) that are universally effective or approved by the US Food and Drug Administration. Oral ruxolitinib has shown efficacy in extensive AA. Ruxolitinib cream would potentially avoid systemic adverse effects. OBJECTIVE: To assess the efficacy and safety of 1.5% ruxolitinib cream in patients with AA who had at least 25% hair loss by Severity of Alopecia Tool score. METHODS: This was a 2-part study. Part A was an open-label, 24-week study of 1.5% ruxolitinib cream in patients with 25% to 99% hair loss followed by a 24-week extension period. Part B was a double-blind, vehicle-controlled, 24-week study of 1.5% ruxolitinib cream in patients with 25% to 100% hair loss, followed by a crossover to ruxolitinib cream in the vehicle group for 24 weeks and additional treatment time for the ruxolitinib cream group. RESULTS: Although Part A results suggested potential efficacy of 1.5% ruxolitinib cream, there was no significant difference in hair regrowth based on 50% improvement in Severity of Alopecia Tool scores between patients receiving 1.5% ruxolitinib cream and vehicle in part B. There were no significant safety issues with 1.5% ruxolitinib cream. LIMITATIONS: Single strength of ruxolitinib cream. CONCLUSIONS: The 1.5% ruxolitinib cream did not have a significant effect in patients with AA.


Subject(s)
Alopecia Areata/drug therapy , Pyrazoles/administration & dosage , Adolescent , Adult , Aged , Double-Blind Method , Female , Humans , Male , Middle Aged , Nitriles , Pharmaceutical Vehicles , Pyrazoles/adverse effects , Pyrimidines , Severity of Illness Index , Skin Cream , Treatment Outcome , Young Adult
15.
J Clin Endocrinol Metab ; 104(7): 2875-2891, 2019 07 01.
Article in English | MEDLINE | ID: mdl-30785992

ABSTRACT

OBJECTIVE: To determine the current state of knowledge and provide evidence-based recommendations that could be valid for all specialists taking care of female pattern hair loss (FPHL), a common form of hair loss in women that is characterized by the reduction of hair density in the central area of the scalp, whereas the frontal hairline is generally well conserved. PARTICIPANTS: An expert task force appointed by the Androgen Excess and PCOS Society, which included specialists from dermatology, endocrinology, and reproductive endocrinology. DESIGN: Levels of evidence were assessed and graded from A to D. Peer-reviewed studies evaluating FPHL published through December 2017 were reviewed. Criteria for inclusion/exclusion of the published papers were agreed on by at least two reviewers in each area and arbitrated by a third when necessary. CONCLUSIONS: (i) The term "female pattern hair loss" should be used, avoiding the previous terms of alopecia or androgenetic alopecia. (ii) The two typical patterns of hair loss in FPHL are centrifugal expansion in the mid scalp, and a frontal accentuation or Christmas tree pattern. (iii) Isolated FPHL should not be considered a sign of hyperandrogenism when androgen levels are normal. (iv) The assessment of patients with FPHL is primarily clinical. (v) In all patients with FPHL, assessment of a possible androgen excess is mandatory. Measurement of vitamin D, iron, zinc, thyroid hormones, and prolactin are optional but recommended. (vi) Treatment of FPHL should start with minoxidil (5%), adding 5α-reductase inhibitors or antiandrogens when there is severe hair loss or hyperandrogenism.


Subject(s)
Alopecia/diagnosis , Hyperandrogenism/diagnosis , 5-alpha Reductase Inhibitors/therapeutic use , Alopecia/epidemiology , Alopecia/pathology , Alopecia/therapy , Androgen Antagonists/therapeutic use , Female , Humans , Hyperandrogenism/drug therapy , Hyperandrogenism/epidemiology , Hyperandrogenism/metabolism , Low-Level Light Therapy , Mineralocorticoid Receptor Antagonists/therapeutic use , Minoxidil/therapeutic use , Platelet-Rich Plasma , Polycystic Ovary Syndrome/epidemiology , Polycystic Ovary Syndrome/metabolism , Scalp/pathology , Spironolactone/therapeutic use , Vasodilator Agents/therapeutic use
16.
J Am Acad Dermatol ; 80(6): 1538-1543.e1, 2019 Jun.
Article in English | MEDLINE | ID: mdl-29017840

ABSTRACT

BACKGROUND: Focal atrichia is a common clinical finding in female pattern hair loss, the specificity and histologic findings of which need further clarification. OBJECTIVE: To determine the frequency of focal atrichia in various types of hair loss and its histologic characteristics in female pattern hair loss. METHODS: Part 1 of the study was a review of 250 consecutive female patients seen with hair loss for the presence of focal atrichia, and part 2 examined paired biopsy specimens from haired areas versus those from areas with focal atrichia in 18 subjects with female pattern hair loss. RESULTS: Focal atrichia was seen in 46 of 104 of women with female pattern hair loss (44%), including 67% of those with the late-onset subtype versus 15% of those with the early-onset subtype, compared with in 3 of 146 of those with other hair disorders (2%). Biopsy findings of focal atrichia in female pattern hair loss showed primarily a more progressive miniaturization process than that of haired areas of the scalp. LIMITATIONS: Some women with female pattern hair loss may have had concomitant chronic telogen effluvium. CONCLUSIONS: When present, focal atrichia is a clinical clue to the diagnosis of female pattern hair loss, particularly the late-onset subtype.


Subject(s)
Alopecia/pathology , Hair Follicle/pathology , Adolescent , Adult , Aged , Alopecia/diagnosis , Biopsy , Female , Humans , Middle Aged , Young Adult
17.
J Natl Compr Canc Netw ; 16(2): 123-135, 2018 Feb.
Article in English | MEDLINE | ID: mdl-29439173

ABSTRACT

Natural killer (NK)/T-cell lymphomas are a rare and distinct subtype of non-Hodgkin's lymphomas. NK/T-cell lymphomas are predominantly extranodal and most of these are nasal type, often localized to the upper aerodigestive tract. Because extranodal NK/T-cell lymphomas (ENKL) are rare malignancies, randomized trials comparing different regimens have not been conducted to date and standard therapy has not yet been established for these patients. These NCCN Guidelines Insights discuss the recommendations for the diagnosis and management of patients with ENKL as outlined in the NCCN Guidelines for T-Cell Lymphomas.


Subject(s)
Lymphoma, T-Cell/diagnosis , Lymphoma, T-Cell/therapy , Disease Management , Humans , Lymphoma, T-Cell/etiology
18.
J Am Acad Dermatol ; 79(3): 470-478.e3, 2018 Sep.
Article in English | MEDLINE | ID: mdl-29128463

ABSTRACT

BACKGROUND: Although alopecia areata is a common disorder, it has no US Food and Drug Administration-approved treatment and evidence-based therapeutic data are lacking. OBJECTIVE: To develop guidelines for the diagnosis, evaluation, assessment, response criteria, and end points for alopecia areata. METHODS: Literature review and expert opinion of a group of dermatologists specializing in hair disorders. RESULTS: Standardized methods of assessing and tracking hair loss and growth, including new scoring techniques, response criteria, and end points in alopecia areata are presented. LIMITATIONS: The additional time to perform the assessments is the primary limitation to use of the methodology in clinical practice. CONCLUSION: Use of these measures will facilitate collection of standardized outcome data on therapeutic agents used in alopecia areata both in clinical practice and in clinical trials.


Subject(s)
Alopecia Areata/diagnosis , Hair/growth & development , Outcome Assessment, Health Care/methods , Practice Guidelines as Topic , Alopecia Areata/drug therapy , Data Collection , Diagnostic Self Evaluation , Endpoint Determination , Humans , Severity of Illness Index
19.
Mod Pathol ; 30(5): 761-772, 2017 05.
Article in English | MEDLINE | ID: mdl-28128277

ABSTRACT

Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αß T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αß/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.


Subject(s)
Lymphoma, T-Cell, Cutaneous , Skin Neoplasms , T-Lymphocytes, Cytotoxic/pathology , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , World Health Organization , Young Adult
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