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521. Leprosy

and inflammation of the preexisting skin and peripheral nerve lesions, resulting in skin edema, erythema, and tenderness and worsening nerve function. These reactions, particularly if not treated early, contribute significantly to nerve damage. Because the immune response is increased, these reactions are termed reversal reactions, despite the apparent clinical worsening. Type 2 reactions (erythema nodosum leprosum, or ENL) are systemic inflammatory reactions that appear to be a vasculitis or panniculitis

2013 Merck Manual (19th Edition)

522. 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)

523. 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)

524. 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)

525. 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)

526. 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)

527. 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

528. Mutations in PSMB8 cause CANDLE syndrome with evidence of genetic and phenotypic heterogeneity. Full Text available with Trip Pro

, unlike in other autoinflammatory disorders.CANDLE syndrome is caused by mutations in PSMB8, a gene recently reported to cause "JMP" syndrome (joint contractures, muscle atrophy, microcytic anemia, and panniculitis-induced childhood-onset lipodystrophy) in adults. We extend the clinical and pathogenic description of this novel autoinflammatory syndrome, thereby expanding the clinical and genetic disease spectrum of PSMB8-associated disorders. IFN may be a key mediator of the inflammatory response

2011 Arthritis and Rheumatism

529. Lipodystrophies: Genetic and Acquired Body Fat Disorders. Full Text available with Trip Pro

are mainly autoimmune in origin and display complement abnormalities. Localized lipodystrophies occur due to drug or vaccine injections, pressure, panniculitis, and other unknown reasons. The current management includes cosmetic surgery and early identification and treatment of metabolic and other complications with diet, exercise, hypoglycemic drugs, and lipid-lowering agents.

2011 Journal of Clinical Endocrinology and Metabolism

530. 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

531. Protocol To Evaluate Patient Measurements After Ultrasonic Treatment

bruising disorder, anticoagulative / thromboembolic condition , hemorrhagic (bleeding) status or use of anti coagulants. Tissue ischemia in the area to be treated. Suffering from concurrent conditions such as liver or kidney disorders, Hypertension or abnormally high blood pressure. High cholesterol and/or diabetes. Active collagen vascular disease (e.g. fibromyalgia, panniculitis, lupus etc.). Epilepsy. Tuberculosis. Auxiliary electric organs (such as pacemakers), metal or myoelectric prosthesis

2011 Clinical Trials

532. A Phase 3 Trial of E7777 in Combination With CHOP Compared With CHOP Alone for the First-Line Treatment of Peripheral T-cell Lymphoma

lymphoma/leukemia, extranodal NK/TCL nasal type, enteropathy-associated TCL, hepatosplenic TCL, subcutaneous panniculitis-like TCL, and cutaneous T-cell lymphoma (CTCL) including mycosis fungoides and Sezary syndrome. Known central nervous system (CNS) involvement with lymphoma. Prior chemotherapy, immunotherapy, denileukin diftitox, or investigational agent(s) for this lymphoma, with the exception that a single cycle of CHOP (or CHOP-based therapy) is allowed if the last dose of CHOP (or CHOP-based

2011 Clinical Trials

533. 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

534. 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

535. 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

536. 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

537. 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

538. 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

539. Clinical Evaluation of the SNaP Wound Care System in Promoting Healing in Chronic Wounds

a thick eschar that persists after wound debridement. Patient has an HbA1C >12%. Patient has ulcers due to inflammatory conditions such as pyoderma gangrenosum, rheumatoid arthritis, vasculitis, cryoglobulinaemia, necrobiosis lipoidica, panniculitis, lupus erythematosus, scleroderma, or calcinosis. Patient has untreated osteomyelitis. Patient has any other condition that, in the opinion of the investigator, makes the patient inappropriate to take part in this study. Patient is allergic to the wound

2011 Clinical Trials

540. 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

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