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JAAD Case Reports Jun 2024
PubMed: 38813066
DOI: 10.1016/j.jdcr.2024.04.021 -
Dermatology Practical & Conceptual Apr 2024The C-reactive protein to albumin ratio (CAR) lately has demonstrated as a prognostic factor and an indicator of disease activity, severity and prognosis in solid organ...
INTRODUCTION
The C-reactive protein to albumin ratio (CAR) lately has demonstrated as a prognostic factor and an indicator of disease activity, severity and prognosis in solid organ malignancies and inflammatory diseases. However, the effects of CAR have not been investigated in mycosis fungoides (MF) patients yet.
OBJECTIVES
This study aimed to determine the potential role of CAR as a diagnostic and a prognostic indicator in MF.
METHODS
We retrospectively investigated the electronic medical records of 97 patients with MF admitted to the Dermatology Clinic of Health Sciences University, Diskapi Yildirim Beyazit Training and Research Hospital between January 2014 and December 2020. In total, 60 patients with MF were enrolled in the study. CAR was evaluated, patient and control group. Also, the other clinicopathological factors including age, lactate dehydrogenase, stage of disease, beta-2-microglobulin levels, and sedimentation levels were evaluated.
RESULTS
The median value of CAR was 0.85 (0.10-7.51) in the patient group, whereas it was 0.39 (0.0-1.11) in the control group (P < 0.001). Patients with disease progression (N = 16, 13M, 3 F) had a median value of CAR 0.84 (0.10-7.51) and the median value of CAR (N = 44) was 0.86 (0.12-4.57) in the group of patients with stable disease. The CAR value had no prognostic significance (P > 0.05).
CONCLUSIONS
There is no association between the CAR and progression in the stage in MF patients. But the CAR is significantly higher in patients with MF than in the control group. The CAR can be a guide for us in cases where we have difficulty in diagnosing.
PubMed: 38810087
DOI: 10.5826/dpc.1402a97 -
Dermatology Practical & Conceptual Apr 2024
PubMed: 38810064
DOI: 10.5826/dpc.1402a84 -
Clinical Case Reports May 2024Unlike most cases, the lesions were localized to the dorsum of the hand, lacked pruritus (itching), and did not exhibit "sperm-like blood vessels," which are typically...
KEY CLINICAL MESSAGE
Unlike most cases, the lesions were localized to the dorsum of the hand, lacked pruritus (itching), and did not exhibit "sperm-like blood vessels," which are typically pathognomonic to classical MF.
ABSTRACT
The study presents a rare case involving a 44-year-old woman who developed a skin condition on the base of her left thumb. Initially misdiagnosed as pigmented purpura, the need for further investigation arose to determine the nature of the condition accurately. The medical evaluation encompassed a comprehensive analysis of the patient's skin ailment. A series of diagnostic examinations were conducted to ascertain the underlying cause. Although routine blood tests yielded unremarkable results, the distinct characteristics of the rash prompted a more thorough investigation. Subsequent assessment revealed that the skin condition was not pigmented purpura, as initially presumed, but rather a manifestation of cutaneous T-cell lymphoma (CTCL) known as mycosis fungoides (MF). MF is an infrequent lymphoma predominantly affecting individuals aged 45-65, exhibiting a male-to-female sex ratio of 2:1. The annual incidence of MF ranges from 0.3 to 0.96 cases per 100,000 individuals. The woman's skin exhibited discrete patches adorned with colored dots, progressively thickening and pigmentation. Notably, the absence of pruritus did not dispel suspicion. This case underscores the significance of accurately diagnosing uncommon dermatological disorders to facilitate appropriate medical intervention. The unique appearance of the rash and its distinctive features, despite normal blood results, enabled the identification of MF. The patient's treatment encompassed a combination of steroids and narrowband UV therapy. Vigilance, continued research, and heightened awareness are paramount for early intervention and improved patient outcomes. Such efforts contribute to an enhanced understanding of the complexities of this condition.
PubMed: 38770416
DOI: 10.1002/ccr3.8847 -
Cureus Apr 2024Sezary syndrome (SS) is a rare but aggressive type of cutaneous T-cell lymphoma (CTCL). Patients with SS have characteristic skin lesions (erythroderma) and a leukemic...
Sezary syndrome (SS) is a rare but aggressive type of cutaneous T-cell lymphoma (CTCL). Patients with SS have characteristic skin lesions (erythroderma) and a leukemic phase. The rash associated with CTCLs can often mimic common benign skin conditions such as psoriasis, atopic dermatitis, etc. and therefore can go undiagnosed until later stages. We present a case of a patient with SS who managed eczema for over one year with topical steroids before receiving a skin biopsy. Workup confirmed leukemic involvement, and the patient was started on systemic therapy with bexarotene. The patient continues to have a good response to systemic therapy. When treating patients with persistent rash of uncertain etiology and/or unresponsive to treatment, primary care physicians and internists need to consider SS/Mycosis fungoides as a possible differential and should have a low threshold to initiate early referral to dermatology for definitive diagnosis.
PubMed: 38765439
DOI: 10.7759/cureus.58570 -
The Journal of Investigative Dermatology May 2024Cutaneous T-cell lymphoma (CTCL) is characterized by malignant T-cells proliferating in a unique tumor microenvironment (TME) dominated by keratinocytes. Skin...
Cutaneous T-cell lymphoma (CTCL) is characterized by malignant T-cells proliferating in a unique tumor microenvironment (TME) dominated by keratinocytes. Skin colonization and infection by Staphylococcus aureus (S. aureus) is a common cause of morbidity and suspected of fueling disease activity. Here we show that expression of HLA-DR, high-affinity receptors for Staphylococcal enterotoxins (SE), by keratinocytes correlates with IFN-γ expression in the TME. Importantly, IFN-γ induces HLA-DR, SE-binding, and SE-presentation by keratinocytes to malignant T-cells from Sézary syndrome (SS) patients, and malignant and non-malignant T-cell lines derived from SS and Mycosis fungoides patients. Likewise, preincubation of keratinocytes with supernatant from patient-derived SE-producing S. aureus triggers proliferation in malignant T-cells and cytokine release (including IL-2), when cultured with non-malignant T-cells. This is inhibited by pre-treatment with engineered bacteriophage S. aureus-specific endolysins. Furthermore, mutations in the HLA-DR binding sites of SE type-A, and siRNA-mediated knockdown of Janus Kinase-3 (JAK3) and IL-2Rγ block induction of malignant T-cell proliferation. In conclusion, we show that, upon exposure to patient-derived S. aureus and SE, keratinocytes stimulate IL-2Rγ/JAK3-dependent proliferation of malignant and non-malignant T-cells in an environment with non-malignant T-cells. These findings suggest that keratinocytes in the TME play a key role in S. aureus mediated disease activity in CTCL.
PubMed: 38762064
DOI: 10.1016/j.jid.2024.04.018 -
The Journal of Dermatological Treatment Dec 2024Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. This study was conducted to evaluate efficacy and safety of interferon (IFN) α-2a...
Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. This study was conducted to evaluate efficacy and safety of interferon (IFN) α-2a combined with phototherapy for early-stage MF. Thirteen patients with early-stage MF received subcutaneous injections of IFN α-2a at 3 million IU combined with phototherapy three times per week for 6 months. Treatment efficacy was measured by changes in body surface area (BSA) score and modified severity-weighted assessment tool (mSWAT) score at 1, 3, and 6 months after treatment. Histopathologic examinations of skin lesions were performed before and after treatment. After 3 months of treatment, all 13 patients achieved a partial response, and BSA and mSWAT scores were significantly lower than those at baseline ( < 0.001). After 6 months, BSA and mSWAT scores were significantly lower than those at baseline ( < 0.001) and after 3 months ( < 0.05). Eleven patients achieved complete remission and two patients achieved a partial response (overall response rate, 100%). Histopathologic examination showed a significant decrease in the number of atypical lymphocytes in both epidermis and dermis. No severe adverse effects occurred. IFN α-2a in combination with phototherapy may be an effective and safe alternative modality for early-stage MF.
Topics: Humans; Mycosis Fungoides; Male; Middle Aged; Female; Prospective Studies; Skin Neoplasms; Adult; Interferon alpha-2; Treatment Outcome; Aged; Injections, Subcutaneous; Interferon-alpha; Combined Modality Therapy; Phototherapy; Neoplasm Staging; Recombinant Proteins
PubMed: 38754985
DOI: 10.1080/09546634.2024.2350231 -
JAAD Case Reports Jun 2024
PubMed: 38741658
DOI: 10.1016/j.jdcr.2024.03.016 -
Case Reports in Oncological Medicine 2024Though mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), it has no curative treatment. The aim of current topical and systemic...
Though mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), it has no curative treatment. The aim of current topical and systemic treatment is centered around relieving symptoms and optimizing disease-free time. The use of surgical management to achieve the same goals of symptomatic reduction is not well described in the current literature. We present a case of refractory MF that failed chemotherapy, radiotherapy, and UV light therapy. Despite medical management, the tumor burden progressed to significant compression neuropathy of the ulnar and median nerves. To reduce tumor burden and attempt to provide symptomatic relief, a surgical plan was developed to include radical resection of the tumor of the left upper extremity (LUE) with release of the cubital tunnel, carpal tunnel, Guyon canal, and coverage with split-thickness skin graft. The patient reported decreased symptomatology interfering with her daily activities and, overall, a better quality of life postoperatively. Surgical intervention, in addition to established medical standards of care, for symptomatic relief of compression neuropathy from tumor mass effect for refractory CTCL should be considered to achieve quality of life goals for patients.
PubMed: 38706790
DOI: 10.1155/2024/6645278