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521. Sarcoidosis

of Sarcoidosis This image shows dacryocystitis manifesting as inflammation of the palpebral lobe of the lacrimal glands in both upper eyelids in a patient with sarcoidosis. This photo shows erythematous facial plaques in a patient with sarcoidosis. Erythema nodosum is a form of panniculitis characterized by palpable, tender, red or violet subcutaneous nodules most typically appearing on the shins. It most often accompanies systemic diseases, including streptococcal infection, sarcoidosis, and inflammatory

2013 Merck Manual (19th Edition)

522. Alpha-1 Antitrypsin Deficiency

with panniculitis Neonates with jaundice or liver enzyme elevations Patients with unexplained bronchiectasis or liver disease Diagnosis is made by identifying serum alpha-1 antitrypsin levels < 80 mg/dL ( < 15 mcmol/L) if measured by the radial immunodiffusion method or levels < 50 mg/dL ( < 9 mcmol/L) if measured by nephelometry. Patients with low levels should have confirmation by genotyping. Prognosis As a group, people with severe alpha-1 antitrypsin deficiency who have never smoked have a normal life (...) processing rather than by enzyme deficiency. may be used for patients with liver failure. Treatment of panniculitis is not well defined. Corticosteroids, antimalarials, and tetracyclines have been used. Key Points Suspect alpha-1 antitrypsin deficiency if patients have unexplained emphysema, liver disease (particularly in neonates), panniculitis, or bronchiectasis. Diagnose using serum alpha-1 antitrypsin levels < 80 mg/dL ( < 15 mcmol/L) and confirm by genotyping. Treat selected patients (nonsmoking

2013 Merck Manual (19th Edition)

523. Stasis Dermatitis

irritants and to potentially sensitizing topical agents (eg, antibiotics; anesthetics; vehicles of topical drugs, especially lanolin or wool alcohols). Key Points Stasis dermatitis results from chronic venous insufficiency. Early signs include erythema, scaling, weeping, and crusting. Eventual results can include hyperpigmentation, ulceration, fibrotic skin, chronic edema, and lipodermatosclerosis (a painful induration resulting from panniculitis). Treat chronic venous insufficiency with elevation

2013 Merck Manual (19th Edition)

524. Chronic Venous Insufficiency and Postphlebitic Syndrome

, lipodermatosclerosis) 5 Skin changes due to venous stasis and healed ulceration 6 Skin changes due to venous stasis and active ulceration *May occur idiopathically without chronic venous insufficiency. Venous stasis dermatitis consists of reddish brown hyperpigmentation, induration, venous ectasia, lipodermatosclerosis (fibrosing subcutaneous panniculitis), and venous stasis ulcers. Stasis Dermatitis (Chronic Changes) Images provided by Thomas Habif, MD. Venous stasis ulcers may develop spontaneously or after

2013 Merck Manual (19th Edition)

525. Systemic Lupus Erythematosus (SLE)

blistering and ulceration are rare, although recurrent ulcers on mucous membranes (particularly the central portion of the hard palate near the junction of the hard and soft palate, the buccal and gum mucosa, and the anterior nasal septum) are common (sometimes called mucosal lupus); findings can sometimes mimic toxic epidermal necrolysis. Generalized or focal alopecia is common during active phases of SLE. Panniculitis can cause subcutaneous nodular lesions (sometimes called lupus panniculitis (...) , maculopapular lupus rash, photosensitive lupus rash (in the absence of dermatomyositis) or Subacute cutaneous lupus (nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, sometimes with postinflammatory dyspigmentation or telangiectasias) Chronic cutaneous lupus Classic discoid rash, localized (above the neck) discoid rash, generalized (above and below the neck) discoid rash, hypertrophic (verrucous) lupus, lupus panniculitis (profundus), mucosal lupus, lupus erythematosus

2013 Merck Manual (19th Edition)

526. Vitamin D Status, Disease Specific and Quality of Life Outcomes in Patients With Cutaneous Lupus

and Severity Index) is a validated CLE disease severity measure that has been in use in clinical trials since 2005. This scale captures cutaneous, mucosal membrane, and alopetic disease activity (erythema and scale/hypertrophy) as well as damage (dyspigmentation and scarring/ atrophy/ panniculitis). Secondary Outcome Measures : Quality of life as measured by the Skindex 29 [ Time Frame: 1 year ] No cutaneous lupus specific quality of life (QOL) measure exists, but the Skindex 29, a validated skin-specific

2011 Clinical Trials

527. A Japanese Phase 1/2 Study to Assess the Efficacy, Safety and Pharmacokinetics of Romidepsin in Patients With Peripheral T-cell Lymphoma (PTCL)

for study participation and have: Histologically confirmed Peripheral T cell Lymphoma (PTCL) Not Otherwise Specified (NOS), Angioimmunoblastic T-cell Lymphoma, enteropathy- type T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, cutaneous T-cell lymphoma (excludes mycosis fungoides or Sezary syndrome) , hepatosplenic T-cell lymphoma, Anaplastic Large cell lymphoma (ALCL) [anaplastic lymphoma kinase-1 (ALK-1) negative], patients with ALK 1 expressing ALCL (ALK-1 positive) who have relapsed

2011 Clinical Trials

528. Clinical Usefulness and Prognostic Significance of Interim 18F-FDG PET/CT for the Treatment of Peripheral T Cell Lymphomas

, first analysis of clinical data will be performed at the time of 80 patients enrolled or after a median follow-up of more than 12 months. Criteria Inclusion Criteria: Age above 18 years Histologically proven PTCLs except primary cutaneous/subcutaneous panniculitis ECOG performance status ≤ 2 Patients who are available to be performed PET/CT at diagnosis No severe concomitant disease Exclusion Criteria: Patients who did not undergo PET/CT scans at diagnosis Patients who have a primary CNS lymphoma

2011 Clinical Trials

529. Study of Pralatrexate Versus Observation Following CHOP-based Chemotherapy in Previously Undiagnosed Peripheral T-cell Lymphoma Patients

T-cell leukemia virus 1+) Angioimmunoblastic TCL Anaplastic large cell lymphoma (ALCL), primary systemic type, excluding anaplastic lymphoma kinase positive (ALK+) with International Prognostic Index (IPI) score less than 2 at initial diagnosis and complete response (CR) after CHOP-based therapy PTCL-unspecified Enteropathy-type intestinal lymphoma Hepatosplenic TCL Subcutaneous panniculitis TCL Transformed mycosis fungoides (tMF) Extranodal T/NK-cell lymphoma nasal or nasal type Primary

2011 Clinical Trials

530. Phase 1 Trial of Siplizumab and Dose-Adjusted EPOCH-Rituximab in T- and NK-Cell Lymphomas

the fourth and sixth treatment cycles (approximately weeks 12 and 18) of siplizumab-EPOCH-R, study researchers will perform blood tests and CT/MRI scans on all patients to assess their response to the treatment. Condition or disease Intervention/treatment Phase T-Cell Peripheral Lymphoma Gamma Delta Hepatosplenic T-Cell Lymphoma Subcutaneous Panniculitis-Like T-Cell Lymphoma NK T-Cell Lymphoma Biological: Rituximab Drug: EPOCH Biological: Siplizumab Phase 1 Detailed Description: Background: The clinical (...) (nos), gamma-delta hepatosplenic T cell lymphoma, subcutaneous panniculitis-like T cell, NK-T cell lymphoma confirmed by pathology or flow cytometry staff of the Hematopathology Section, Laboratory of Pathology, NCI. Patients with alk-positive anaplastic large cell lymphoma and patients with T cell precursor disease are not eligible. Age greater than or equal to 18 years. Laboratory tests: Creatinine less than or equal to 1.5 mg/dL or creatinine clearance greater than or equal to 60 ml/min

2011 Clinical Trials

531. Alisertib in Treating Patients With Relapsed or Refractory Peripheral T-Cell Non-Hodgkin Lymphoma

or negative Angioimmunoblastic T-cell NHL Subcutaneous panniculitis-like T-cell lymphoma Enteropathy-associated T-cell NHL Hepatosplenic T-cell lymphomas Extranodal natural killer (NK)/T-cell lymphoma, nasal type Adult T-cell leukemia/lymphoma Unclassifiable PTCL Transformed cutaneous T-cell lymphoma (CTCL) to PTCL with systemic involvement (not local skin transformation) No other histologies are eligible; examples of ineligible histologies include: T-cell prolymphocytic leukemia, T-cell large granular

2011 Clinical Trials

532. Evaluation of disease activity and damage in different subtypes of cutaneous lupus erythematosus using the CLASI. (Abstract)

than with ACLE (P<0.05), SCLE (P<0.001) and CCLE (P<0.001). The erythema score and the scale/hypertrophy score were significantly lower in LET than in ACLE (P<0.05, both) and CCLE (P<0.05 and P < 0.001, respectively). The dyspigmentation score was lowest in patients with LET, differing significantly from ACLE (P<0.05), SCLE (P<0.05) and CCLE (P<0.001). The scarring/atrophy/panniculitis score was significantly higher in patients with CCLE in contrast to SCLE and LET (P<0.05 and P<0.001, respectively

2011 Journal of the European Academy of Dermatology and Venereology

533. Dermal infiltrates of cutaneous T-cell lymphomas with epidermotropism but not other cutaneous lymphomas are abundant with langerin⁺ dendritic cells. (Abstract)

infiltrates. No direct co-localization of CD3 and langerin could be resolved. Dermal langerin(+) cells were detected only in one of six primary cutaneous anaplastic large cell lymphomas (C-ALCL), characterized by epidermotropism. In other C-ALCL cases (five of six), in lymphomatoid papulosis (n = 3), subcutaneous panniculitis-like T-cell lymphoma (n = 2), and all variants of CBCL no dermal langerin(+) DCs could be found.Langerin(+) DCs are abundant in the dermal infiltrates of T-cell lymphomas

2011 Journal of the European Academy of Dermatology and Venereology

534. High expression of Dicer reveals a negative prognostic influence in certain subtypes of primary cutaneous T cell lymphomas. (Abstract)

of 50 consecutive patients with primary CTCL were studied, with the majority having mycosis fungoides (n=34). Five patients had primary cutaneous CD 30+ anaplastic large cell lymphoma, four patients each had lymphomatoid papulosis and primary cutaneous CD4-positive small/medium T-cell lymphoma, one primary cutaneous γδ T cell lymphoma, one Sézary syndrome and another subcutaneous panniculitis-like T cell lymphoma of αβ-phenotype. Immunohistochemistry was performed on paraffin sections using

2011 Journal of dermatological science

535. A Case of Assisted Reproductive Therapy-induced Erythema Nodosum Full Text available with Trip Pro

A Case of Assisted Reproductive Therapy-induced Erythema Nodosum Erythema nodosum is a common variant of panniculitis. It is characterized by tender erythematous nodule and plaque on the anterior aspect of the leg. The etiology is not fully understood. It may be associated with a variety of disorders, including infection, medication, autoimmune disorders, pregnancy, and malignancy. A 33-year-old Korean woman presented with 1 week history of painful erythematous plaques on both knees. She was 7 (...) weeks pregnant with assisted reproductive therapy, and had been maintained on daily intramuscular progesterone injection for 4 weeks. Histological examination of the lesions revealed septal panniculitis without vasculitis. Two days after discontinuing progesterone injection, the symptoms and lesions started to resolve. Herein we present a case of erythema nodosum caused by progesterone injection for endometrial preparation.

2011 Annals of dermatology

536. Calcinosis Cutis Occurring in Association With Autoimmune Connective Tissue Disease: The Mayo Clinic Experience With 78 Patients, 1996-2009. Full Text available with Trip Pro

in the clinical setting of ACTD.Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n = 30) with classic (n = 15), juvenile (n = 14), and amyopathic (n = 1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n = 24); lupus panniculitis (n = 4); systemic lupus erythematosus (n = 2); mixed connective tissue disease (n = 4); overlap connective tissue disease (n = 6); undifferentiated

2011 Archives of Dermatology

537. Cutaneous simulants of infectious disease. (Abstract)

, and panniculitis, have been mistaken for infection. This review emphasizes the clinical presentation, physical exam, and diagnostic workup of many of these conditions to assist the clinician in ascertaining the correct diagnosis. In addition, general treatment options are provided for each disease category.© 2011 The International Society of Dermatology.

2011 International Journal of Dermatology

538. Nonbullous neutrophilic lupus erythematosus: A newly recognized variant of cutaneous lupus erythematosus. (Abstract)

. One patient also presented with neutrophil-rich lupus panniculitis. All clinical lesions resolved with immunomodulating/immunosuppressive agents.This study was limited by the small number of cases.Nonbullous neutrophilic LE is an important entity to consider in the differential diagnosis of neutrophil-mediated eruptions. In addition, the histologic finding of neutrophils in the setting of lupus should alert one to the possibility of systemic disease.Copyright © 2009 American Academy of Dermatology

2011 Journal of American Academy of Dermatology

539. Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma. (Abstract)

Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma. Lymphoma-associated hemophagocytic syndrome (LAHS) is a rare clinicopathological entity. It has been described with primary cutaneous lymphomas, mostly of the subcutaneous panniculitis-like T-cell type, and only once with cutaneous T-cell lymphoma (CTCL).We report the cases of 5 patients with epidermotropic CTCL who developed LAHS and died shortly thereafter. Unlike

2011 Journal of American Academy of Dermatology

540. Breast infarction due to calciphylaxis after coronary artery bypass grafting. (Abstract)

-sized to medium-sized vessels. This causes ischemic necrosis of the skin and septal panniculitis. We believe that this is the first case report of breast necrosis after coronary artery bypass grafting, due to calciphylaxis in a patient with known chronic renal insufficiency, without renal replacement therapy.Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

2011 Annals of Thoracic Surgery

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