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2.1
Primary Cutaneous Lymphoma
Cutaneous Lymphoma

Presentation

Primary cutaneous lymphomas rarely present to the hand surgeon. Diagnosis of primary cutaneous lymphoma in the upper extremity can be difficult to make, as they are rare, and may be mistaken for other lesions. [ncbi.nlm.nih.gov]

Case Presentation A 78–year old woman presented to the dermatology department with a 5–week history of multiple erythematous nodules on left cheek (Figure 1). These nodules did not bleed and only caused mild itching occasionally. [austinpublishinggroup.com]

Most systemic lymphomas present with enlargement within the lymphatic system, such as enlarged lymph nodes or an enlarged spleen, and may also present with symptoms such as fever, night sweats, and weight loss. [bmc.org]

Entire Body System

  • Pallor

    Note the change in the patient who appears ill with pallor, cyanosis, and/or bradycardia was child asleep or awake. Physical examination may reveal rhinorrhea and visual disturbances. [miketnelson.com]

Respiratoric

  • Nasal Ulcer

    Figure 2 Clinical presentation of PCAECyTCL in two different patients. ( a ) Oral and nasal ulcerated macules (Case 25). ( b ) Extensive annular erosive plaques some with hemorrhagic features (Case 25). ( c ) Annular and arcuate ulcerated plaques and [nature.com]

Skin

  • Skin Lesion

    In this report, we describe a 60-year-old man with primary cutaneous lymphoma, who presented with multiple painless reddish nodular skin lesion on the different sites of his body and there was no evidence of systemic involvement. [ncbi.nlm.nih.gov]

    […] affects other sites in 10–15% Represents an aggressive subtype of PCBCL Multiple skin lesions on the leg have poor prognosis Primary cutaneous diffuse large B-cell lymphoma, other Large B-cell lymphoma presents with skin lesions on the head, the trunk [dermnetnz.org]

    The skin lesions may even go away on their own, without any treatment. [cancer.org]

    The majority of patients had multiple, generalised skin lesions (79%), 10.5% multiple but localized, 10.5% a single large lesion. Skin lesions were tumors in 47.4% of patients, erythroderma in 21%, patches in 15.8%, and plaques in 15.8%. [haematologica.org]

    […] of the tumor Untreated skin lesion s increase in size, rarely metastases to other site s Epidemiology and Mortality Age: 51 years median age Sex: slight male predominance Sources Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J [seer.cancer.gov]

  • Dermatitis

    These include: skin diseases, such as: eczema or atopic dermatitis psoriasis lichen planus lupus erythematosus a group of rashes of unknown cause, such as chronic superficial dermatitis, digitate dermatosis or parapsoriasis granulomatous skin diseases [lymphomas.org.uk]

    CTCL often mimics eczema, psoriasis, or other chronic dermatitis, and because of this it’s common that the diagnosis of CTCL is delayed, sometimes by years or decades. [clfoundation.org]

    Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific dermatitis. The cause of CTCL is unknown. [en.wikipedia.org]

    TIA1+, betaF1+; EBV- Estimated 5 year survival is 0% Must rule out mycosis fungoides (e) Cutaneous gamma / delta T cell lymphoma : Multiple plaques and tumors, similar to disseminated pagetoid reticulosis Epidermal involvement with necrosis, interface dermatitis [pathologyoutlines.com]

    更新日: 19/03/30 14:23 研究者氏名 菅谷 誠 eメール sugayamder iuhw.ac.jp 所属 国際医療福祉大学 部署 医学部皮膚科 職名 主任教授 学位 医学博士(東京大学) 論文 テキストで表示 1 2 3 > Keratinocyte Proline-Rich Protein Deficiency in Atopic Dermatitis Leads to Barrier Disruption. [researchmap.jp]

  • Skin Disease

    These include: skin diseases, such as: eczema or atopic dermatitis psoriasis lichen planus lupus erythematosus a group of rashes of unknown cause, such as chronic superficial dermatitis, digitate dermatosis or parapsoriasis granulomatous skin diseases [lymphomas.org.uk]

    […] and non skin disease at diagnosis, as well as skin only Excludes mycosis fungoides and peripheral T cell lymphoma All CD30 negative with medium / large cells or subcutaneous panniculitis-like (a) Subcutaneous panniculitis-like T cell lymphoma : Indurated [pathologyoutlines.com]

    & Blood Foundation — New Zealand lymphomainfo.net (facts, resources and community) Books about skin diseases See the DermNet NZ bookstore. [dermnetnz.org]

    These were minimal recurrences with limited skin disease responsive to topical steroids. Nine patients had major events/PD. These included 4 PD and 5 significant clinical relapses requiring other systemic therapies. [haematologica.org]

    Multi-agent chemotherapy is only indicated in patients presenting with or developing extracutaneous disease and rare patients with rapidly progressive skin disease [ 9 ]. [annonc.oxfordjournals.org]

  • Eruptions

    Most patients present with localized or disseminated eruptive papules, nodules and tumours with central ulceration and necrosis. [codes.iarc.fr]

    […] sunlight (photosensitivity), particularly on the exposed skin of the face; this is called ‘actinic reticuloid’ or ‘chronic actinic dermatitis’ lymphocytoma cutis, also known as 'pseudolymphoma', which can be triggered by: drugs (sometimes called a ‘drug eruption [lymphomas.org.uk]

    Clinically, they show localized or disseminated eruptive papules, nodules and tumors showing central ulceration or superficial, hyperkeratotic patches and plaques. [annonc.oxfordjournals.org]

    Bittencourt AL, da GraÇas Vieira M, Brites CR, et al: Adult T-cell leukemia/lymphoma in Bahia, Brazil: analysis of prognostic factors in a group of 70 patients, Am J Clin Pathol, 2007; 128: 875-882. 8) Sawada Y, Hino R, Hama K, et al: Type of skin eruption [minds.jcqhc.or.jp]

    Thirteen patients carried an initial diagnosis of ‘mycosis fungoides’, whereas many other cases were initially diagnosed with a benign condition including an eczematous process, contact dermatitis, viral or drug eruption, psoriasis (11 cases), lupus erythematosus [nature.com]

  • Erythema

    Erythema and pruritus of the affected areas can occur. Generally, the condition is considered benign. However, some researchers speculate this presentation an an unusual host reaction in particular body sites. [rarediseases.org]

    […] an initial diagnosis of ‘mycosis fungoides’, whereas many other cases were initially diagnosed with a benign condition including an eczematous process, contact dermatitis, viral or drug eruption, psoriasis (11 cases), lupus erythematosus (2 cases), erythema [nature.com]

    "Congenital telangiectatic erythema resembling lupus erythematosus in dwarfs; probably a syndrome entity". American Journal of Diseases of Children. 88 (6): 754–8. doi:10.1001/archpedi.1954.02050100756008. [wikipredia.net]

Workup

Thus, while this review provides an overview and guideline for the workup of lymphoid lesions of the skin, the practitioner should understand the importance of clinical correlation as well as appropriate utility of available resources (such as clonality [ncbi.nlm.nih.gov]

Distinguishing primary cutaneous anaplastic lymphoma (PC-ALCL) from its systemic counterpart requires a complete clinical and laboratory workup. [cancerjournal.net]

Follow-up Patients should be seen in an outpatient setting at least every 6 months for a clinical workup. They do not require any additional laboratory workup unless enlargement of the regional lymph nodes has occurred. [emedicine.medscape.com]

Other Pathologies

  • Lymphocytic Infiltrate

    The papillary dermis may have diffuse lymphocytic infiltration. [rarediseases.org]

  • Visceromegaly

    No other cutaneous manifestations were detected and there was no palpable locoregional lymphadenopathy or visceromegaly. Figure 1: Erythematous-violaceus and infiltrated plaque on posterolateral left leg with similar nodules in proximity. [omicsonline.org]

Treatment

Skin-directed treatments: For early stages of MF, treatments are aimed at the skin. [cancer.org]

Based on this classification we will provide a summary of the current medical literature in diagnosis, treatment, and prognosis for primary cutaneous lymphomas with emphasis on new treatment strategies. [scholars.northwestern.edu]

Five of six patients relapsed following initial treatment. Four patients were treated with primary radiation therapy, three of which had relapses outside the treatment area. [ncbi.nlm.nih.gov]

Treatments for cutaneous lymphoma Treatment for skin lymphomas depends on how widespread the disease is. [cityofhope.org]

Recurring tumors have worse prognosis compared to tumors that do not recur Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment Progression of the condition makes the outcome [dovemed.com]

Prognosis

(associated with a poor prognosis). [orpha.net]

Primary cutaneous T- and B-cell lymphomas are a heterogenous group of diseases with varied clinical presentations and prognosis. [scholars.northwestern.edu]

Primary cutaneous diffuse large B-cell lymphoma, other Large B-cell lymphoma has an excellent prognosis. Blastic plasmacytoid dendritic cell neoplasm and in travascular large B-cell lymphoma have a poor prognosis. [dermnetnz.org]

The prognosis of patients with MF is above all dependent on the stage of the disease [ 3 ]. [annonc.oxfordjournals.org]

Our patients showed poor prognosis ; the present cases may belong to lethal subtypes, and their poor prognosis may not be simply due to a delay in diagnosis. [ci.nii.ac.jp]

Etiology

These two cases, in consideration of the rarity of nodular lymphoma in children, promote speculative considerations, especially in regard to etiology and therapy. [ncbi.nlm.nih.gov]

Cutaneous T-Cell Lymphoma: Epidemiology, Etiology, and Classification. Leukemia & Lymphoma, Nov2003 Supplement 3, Vol. 44 Issue s3, pS49-S54, Burg G y otros: WHO/EORTC classification of cutaneous lymphomas 2005. Histological and molecular aspects. [iqb.es]

(Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. [dovemed.com]

Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. The disease likely begins as a hyperreactive inflammatory process. [emedicine.medscape.com]

Epidemiology

Epidemiological data on PCLs according to the World Health Organization/European Organization for Research and Treatment of Cancer classification (WHO-EORTC) has not been investigated in Korea to date. [ncbi.nlm.nih.gov]

Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma. [atlasgeneticsoncology.org]

Herein, we review primary CTCLs, including epidemiological data, a brief summary of clinical presentations, immunophenotype, molecular signatures and differential diagnoses. [jcp.bmj.com]

Pathophysiology

The average age of cancer diagnoses in the cohort is approximately 26 years old.[23] Pathophysiology[edit] When a cell prepares to divide to form two cells, the chromosomes are duplicated so that each new cell will get a complete set of chromosomes. [wikipredia.net]

Pathophysiology The development of cutaneous lymphoma is only partially understood. Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. [emedicine.medscape.com]

Prevention

[…] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer. [nccn.org]

United States Preventive Services Task Force Overstates Cosmetic Harms of Skin Cancer Screening by Joseph F. Sobanko, MD; Kimberly Shao, BS; Rebecca L. Pearl, PhD; and Sancy Leachman, MD PhD Dr. [jcadonline.com]

Residency University of Pa Medical Ctr - Dermatology Other Education University of Pa Medical Ctr - Dermatology Internship Sinai Hospital of Baltimore - Internal Medicine Medical School McGill University - Medical Doctor Member Board American Board of Preventive [uvmhealth.org]

How can Primary Cutaneous, Small-Medium, CD4-Positive, T-Cell Lymphoma be Prevented? Currently, it is not possible to prevent Primary Cutaneous, Small-Medium, CD4-Positive, T-Cell Lymphoma. [dovemed.com]

This will prevent premature loss. The aspiration of food and fluid attenuated inversion recovery images is more echogenic than hydrocele fluid. [miketnelson.com]

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2.1
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