DOCSLIB.ORG

Sweet's Syndrome

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Sweet's Syndrome NEUTROPHILIC & EOSINOPHILIC DERMATOSES UNTHSC/TCOM Dermatology Residency Fort Worth, TX Bridget McIlwee, DO PGY4 Heather Reagin, DO PGY3 Michael Carletti, DO PGY3 DISCLOSURES Neither I, nor the members of our residency program, nor any of the contributors to this presentation have any relevant disclosures to declare. NEUTROPHIL BIOLOGY & NEUTROPHILIC DERMATOSES Neutrophil maturation and biology myeloblast • >5–10 × 1010 neutrophils produced daily in bone marrow promyelocyte • Differentiation from pluripotent stem cells requires 7–10 days myelocyte • Bone marrow upregulates neutrophil production in response to stress (e.g., metamyelocyte infection) • Mature neutrophils circulate in band peripheral bloodstream for 3–12 hours • They then migrate into tissues, surviving 2–3 days further segmented neutrophil Image available at: http://images.slideplayer.com/14/4463028/slides/slide_30.jpg • Acquired at promyelocyte stage Neutrophil biology • Myeloperoxidase, lysozyme, neutrophil Primary elastase, defensins, myeloblastin (azurophilic) • Neutrophils are a crucial granules defense against microbes • Granulocytes: like eos, basophils • Acquired at transition to myelocyte stage • Contain numerous granules – Secondary • Lactoferrin, neutrophil collagenase, oxidative and non-oxidative granules transcobalamin 1 • Enable destruction of target organisms • Acquired during later stages of maturation Tertiary • Neutrophil gelatinase granules Neutrophil biology • Neutrophils activated at inflamed site express • Inflammatory factors surface receptors – e.g. TLR, neutrophil for opsonins, CK activation b2 • Activation • Microbes killed by oxygen- ligand expression • degranulation dependent mechanisms • Binding to ICAM-1 • secretion of enzymes formation of ROS (endothelial cells) • Discharge of granule Tissue migration • oxidative burst Phagocytosis • Diapedesis = • production of contents degranulate neutrophil migration of arachidonic acid neutrophils towards • Neutrophils “crawl” metabolites • Neutrophils then rapidly site of injury or • Opsonisation enhances undergo apoptosis via use of • recognition, attachment inflammation secretion of cytokines ingested by macrophages lamellipodia targets particle for • Cells move along phagocytosis Activation, Destruction of chemical gradient, Activation • Binding of particle to adhesion, migration responding to neutrophil receptors ingested material chemical factors engulfment (FMLP, C5a, PAF, LTB4) • Particle fuses with lysosomal granule phagolysosome Neutrophilic pathophysiology • Neutrophils move at up to 30mm/min – fastest cell in the body • Among first cells to arrive at sites of inflammation • Neutrophils contain potent defense mechanisms • Destroy not only microbes and necrotic debris, but also normal tissue • Release of lysosomal enzymes, ROS, prostaglandins and leukotrienes into the extracellular space endothelial injury and tissue damage • Neutrophils contribute to many acute and chronic diseases of skin, body • Varied diseases • Similar pathogenesis and histopathology similar therapy Neutrophilic dermatoses Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Sweet’s syndrome • Acute febrile neutrophilic • Variants dermatosis • Classical: • Epidemiology • URI or GI infection • Occurs in all ages; average • Inflammatory bowel age of onset is 30-60 years disease • Female predominance (4:1) • Pregnancy • Especially drug-induced • Autoimmune disorders • Clinical presentation • Malignancy-associated: • AML • Tender papules or nodules coalesce into plaques • Vesiculobullous • May have vesicular, bullous, or • Drug-induced: pustular appearance • G-CSF (granulocyte • Favor head, neck, UE colony stimulating • Oral ulcers factor) • Exhibits pathergy • All-trans-retinoic acid Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Sweet’s syndrome DIAGNOSTIC CRITERIA • Major criteria – must demonstrate both: 1. Abrupt onset of cutaneous lesions consistent with typical Sweet’s syndrome 2. Histopathology consistent with Sweet’s syndrome • PLUS 2 minor criteria: • Preceded by associated systemic findings, such as infection, pregnancy, drugs, malignancy or other inflammatory conditions • Fever and constitutional symptoms • Leukocytosis • Excellent response to systemic corticosteroids • Associated laboratory abnormalities: • Elevated white blood cell count • Elevated erythrocyte sedimentation rate (ESR) or C- reactive protein (CRP) Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Sweet’s syndrome • Histopathology Treatment • Superficial dermal edema with diffuse • 30% will recur; 50% in patients with neutrophilic infiltrate hematologic disorders • Leukocytoclasia with minimal to no evidence of vasculitis • First line therapy: • Systemic corticosteroids for 4-6 weeks* • Intralesional or topical corticosteroids • Second line therapy: • Dapsone • Potassium iodide • Colchicine • Alternate therapies: • NSAIDs, clofazimine, cyclosporine, interferon-α, IVIg, thalidomide, TNF-α inhibitors, Anakinra Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Pyoderma gangrenosum • Epidemiology Common variants • Recurring, painful disease; unknown cause 1. Classical/ulcerative • Commonly affects women 20-50yo • Pretibial areas; frequently seen in conjunction with • IBD, RA 50% have underlying systemic disease • Resolves with cribriform scarring • IBD, arthritis; hematologic disorders 2. Atypical/bullous (IgA monoclonal gammopathy), other neutrophilic dermatosis • Vesicles bullae superficial ulcers/erosions • Face and upper extremities, especially dorsal hands • 25-50% of cases are idiopathic • Frequently occurs with AML, MDS, IgA gammopathy • 4% of cases occur in infants and children 3. Vegetative – pyostomatitis vegetans • Pathergy implicated in 20-30% of PG cases • Sterile pyoderma +/- sinus tracts • Clinical features • Labial and buccal mucosa • Frequently occurs with IBD • Classical: Tender papulopustule fibrinous ulcer, violaceous induration, undermined 4. Pustular • PAPA syndrome (AD): pyogenic sterile • Multiple pustules surrounded by erythematous halo • Extensor extremities and trunk; frequently occurs with arthritis, pyoderma gangrenosum and acne IBD • CD2 binding protein 1 gene mutation Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Pyoderma gangrenosum: proposed diagnostic criteria *Currently a diagnosis of exclusion. • Major criteria (must have all 2) 1. Rapid progression of painful, necrolytic ulcer with irregular, violaceous, undermined border 2. Already excluded: other causes of cutaneous ulceration • Minor criteria (must have ≥2) • History suggestive of pathergy or clinical finding of cribriform scar • History of systemic diseases associated w/PG • Histopathologic findings of PG • Rapid treatment response to systemic corticosteroids Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Pyoderma gangrenosum • Histopathology • Early: leukocytoclasia and sterile dermal neutrophilic infiltrate/abscess • Advanced: marked epidermal necrosis, leukocytoclasia, fibrosing inflammation at edge; thrombosis of vessels, extravasated RBCs • Treatments • None show consistent efficacy • Wound care • Avoid debriding tissue • Hyperbaric oxygen, biologic dressings, grafts • Corticosteroids • Systemic, topical or IL corticosteroids • TNF-α inhibitors • Infliximab, adalimumab, etanercept • Calcineurin inhibitors • Cyclosporine, oral or topical tacrolimus, topical pimecrolimus • Antimetabolites/cytotoxics • Azathioprine, cyclophosphamide, mycophenolate mofetil Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Behçet’s disease • Clinical features • Recurrent aphthous stomatitis and genital aphthae • Aphthous stomatitis generally first symptom • Epidemiology • Erythema nodosum-like lesions, superficial • Rare, multisystem; unknown etiology; unpredictable thrombophlebitis, palpable purpura relapses/remissions • Favor legs, buttocks of women • Triad: oral ulcers, genital ulcers, ocular inflammation • Other systems • Peak age of onset: 20-35 years • Ophtho: Anterior/posterior uveitis, vasculitis blindness • HLA*B51 association (80% of Asian patients) • MSK: Arthritis, arthralgias – non-erosive; occurs in 50% • Common in Japan, Middle East (Turkey) • GI: pain and hemorrhage; difficult to distinguish from IBD • Neurologic: impaired memory, CN palsy, brainstem • Related disorders lesions • MAGIC (mouth and genital ulcers with inflamed • Occurs later in disease course – portends poor prognosis cartilage) syndrome • Cardiac: myocarditis, coronary arteritis, aneurysms • Features of Behcet’s and relapsing polychondritis • Renal: glomerulonephritis Sweet’s Pyoderma Behcet’s SAPHO BADAS syndrome gangrenosum disease syndrome Behçet’s disease DIAGNOSIS OF BEHCET’S REQUIRES SCORE OF 3 POINTS 1 point Oral aphthosis 1 point Skin manifestations • psuedofolliculitis • skin aphthosis 1 point Vascular lesions • phlebitis • large vein thrombosis/aneurysm • arterial thrombosis SIMPLIFIED DIAGNOSTIC CRITERIA: 1 point Positive pathergy test • ≥3 episodes of oral ulceration in past 12mo 2 points Genital aphthosis • PLUS 2 of the following: 2 points Ocular lesions • Genital ulcers, eye lesions, skin lesions, positive pathergy test Behçet’s disease • Histopathology Treatment • Ulcerated epidermis or pustule • Difficult secondary to variable nature and • Angiocentric, neutrophilic infiltrates multi-organ involvement • Leukocytoclasia/LCV, RBC extravasation • Cutaneous lesions:
Load more

Recommended publications
  • Pyoderma Gangrenosum and Cobalamin
    Teoh et al. BMC Rheumatology (2021) 5:7 https://doi.org/10.1186/s41927-021-00177-4 BMC Rheumatology CASE REPORT Open Access Pyoderma gangrenosum and cobalamin deficiency in systemic lupus erythematosus: a rare but non fortuitous association Sing Chiek Teoh1, Chun Yang Sim2* , Seow Lin Chuah3, Victoria Kok1 and Cheng Lay Teh3 Abstract Background: Pyoderma gangrenosum (PG) is an uncommon, idiopathic, ulcerative neutrophilic dermatosis. In many cases, PG is associated with a wide variety of different disorders but SLE in association with PG is relatively uncommon. In this article we present the case of a middle aged patient with PG as the initial clinical presentation of SLE. We also provide a brief review of cobalamin deficiency which occurred in our patient and evidence-based management options. Case presentation: A 35 years old man presented with a 5 month history of debilitating painful lower limb and scrotal ulcers. This was associated with polyarthralgia and morning stiffness involving both hands. He also complained of swallowing difficulties. He had unintentional weight loss of 10 kg and fatigue. Physical examination revealed alopecia, multiple cervical lymphadenopathies, bilateral parotid gland enlargement and atrophic glossitis. There was Raynaud’s phenomenon noted over both hands and generalised hyper-pigmented fragile skin. Laboratory results disclosed anaemia, leukopenia, hyponatraemia and hypocortisolism. Detailed anaemic workup revealed low serum ferritin and cobalamin level. The autoimmune screen showed positive ANA, anti SmD1, anti SS- A/Ro 52, anti SSA/Ro 60, anti U1-snRNP with low complement levels. Upper gastrointestinal endoscopy with biopsies confirmed atrophic gastritis and duodenitis. Intrinsic factor antibodies and anti-tissue transglutaminase IgA were all negative.
    [Show full text]
  • Sarcoidosis and Pyoderma Gangrenosum, an Unusual
    Open Journal of Clinical & Medical Volume 4 (2018) Issue 19 Case Reports ISSN 2379-1039 Sarcoidosis and pyoderma gangrenosum, an unusual combination Naval Mendiratta*; Muzafar Bindroo Ahmed; Shruti Bajad; Rajiva Gupta *Naval Mendiratta Department of Rheumatology and Clinical Immunology, Medanta Hospital Gurgaon, Haryana, India Email: [email protected] Abstract We present a case of unusual combination of Sarcoidosis with refractory Pyoderma Gangrenosum. Pyoderma Gangrenosum has been a reported with other autoimmune disorders like Rheumatoid Arthritis and Ulcerative colitis but it has hardly ever been reported with another rare disease like Sarcoidosis. Here we present a case of 39 year old female, who was diagnosed and treated for Sarcoidosis but later presented to our OPD with worsening skin lesions which were biopsied and diagnosed as Pyoderma Gangrenosum. Not only were they unusual, but they were even refractory to standard medical therapy. Keywords pyoderma; sarcoidosis; refractory pyoderma; cyclophosphamide Abbreviations ESR : Erythrocyte sedimentation rat; CRP: C- Reactive protein; CT: Computerized tomography; EUS: Endoscopic ultrasound; FNAC: Fine needle aspiration cytology; AFB: Acid fast bacilli; OPD: Out patient department; ACE : Angiotensin converting enzyme Case Report 39 year old female, was referred to our OPD for further management once the diagnosis of Sarcoidosis was conirmed. Symptoms started in December 2014, when she had complaints of fever along with cough. Further evaluation was done and a chest CT revealed multiple mediastinal lymph nodes. Patient was started on empiricalanti tubercular drugs (4 drug regimen ) ,which was given for 6 months. She felt better during the treatment course but after a period of 2 months, she started having again low grade fever with myalgia's and arthalgias.
    [Show full text]
  • Pyoderma Gangrenosum with Positive Antinuclear Antibody, in the Absence of Systemic Association
    Case Report http://dx.doi.org/10.3126/njdvl.v16i1.19418 Pyoderma Gangrenosum with Positive Antinuclear Antibody, in the Absence of Systemic Association Shrestha S1, Aryal A2 1Lecturer, 2Second Year Resident, Department of Dermatology, Dhulikhel Hospital, Kathmandu University-Teaching Hospital, Dhulikhel, Kavre, Nepal. Abstract Pyoderma gangrenosum is an uncommon neutrophilic dermatosis, seen on legs, and infrequently on hands and other anatomical sites. It is associated with systemic diseases in 50-70% of the cases. Antinuclear antibody (ANA) seropositivity has been reported in pyoderma gangrenosum associated with connective tissue disorders. However, there are very few case reports of pyoderma gangrenosum in patients of systemic lupus erythematosus, while we did not find any reports of ANA seropositivity in isolated pyoderma gangrenosum. Hence, we report this unique case of pyoderma gangrenosum with classical clinicohistopathology, positive ANA but no systemic association. As anticipated, our patient responded promptly to steroids. Key words: Antibodies; connective tissue diseases; lupus erythematosus; vasculitis, leukocytoclastic Introduction systemic comorbidi es were elicited from history. She was a nonsmoker. yoderma gangrenosum (PG) is a rare necro zing, Pulcera ve neutrophilic dermatosis.1 It is usually On examina on, pa ent was afebrile with normal associated with various systemic illnesses, but vital signs (BP 100/60 mm of mercury, Pulse 84/m, rarely described in associa on with systemic lupus RR 18/min). Cutaneous examina on revealed fi ve erythematosus (SLE) or an nuclear an body (ANA) annular lesions on sun-exposed sites of hands and seroposi vity. We report this case of PG on sunexposed feet (Figure 1). Among them, only one lesion on right sites, with posi ve ANA and no internal disease.
    [Show full text]
  • The Inpatient Burden and Comorbidities of Pyoderma Gangrenosum in Adults in the United States
    Henry Ford Health System Henry Ford Health System Scholarly Commons Dermatology Articles Dermatology 7-3-2020 The inpatient burden and comorbidities of pyoderma gangrenosum in adults in the United States Shanthi Narla Henry Ford Health System, [email protected] Jonathan I. Silverberg Follow this and additional works at: https://scholarlycommons.henryford.com/dermatology_articles Recommended Citation Narla S, and Silverberg JI. The inpatient burden and comorbidities of pyoderma gangrenosum in adults in the United States. Arch Dermatol Res 2020. This Article is brought to you for free and open access by the Dermatology at Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Dermatology Articles by an authorized administrator of Henry Ford Health System Scholarly Commons. Archives of Dermatological Research https://doi.org/10.1007/s00403-020-02098-7 ORIGINAL PAPER The inpatient burden and comorbidities of pyoderma gangrenosum in adults in the United States Shanthi Narla1 · Jonathan I. Silverberg2 Received: 24 April 2020 / Accepted: 17 June 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020 Abstract Hospital admission is often necessary for management of pyoderma gangrenosum (PG), including wound care and pain con- trol. No large-scale controlled studies examined the burden of hospitalization for PG. The objective of this study is to deter- mine the prevalence, predictors, outcomes, and costs of hospitalization for PG in United States adults. Data were analyzed from the 2002 to 2012 National Inpatient Sample, including a 20% representative sample of United States hospitalizations. The prevalence of hospitalization for PG increased between 2002 and 2012. Primary admission for PG was associated with age 40–59 years, female sex, black race/ethnicity, second-quartile household income, public or no insurance, and multiple chronic conditions.
    [Show full text]
  • Clinical Features of the SAPHO Syndrome and Their Role in Choosing the Therapeutic Approach: Report of Four Patients and Review of the Literature
    Acta Dermatovenerol Croat 2014;22(3):180-188 CLINICAL ARTICLE Clinical Features of the SAPHO Syndrome and their Role in Choosing the Therapeutic Approach: Report of Four Patients and Review of the Literature Branimir Anić, Ivan Padjen, Miroslav Mayer, Dubravka Bosnić, Mislav Cerovec Division of Clinical Immunology and Rheumatology, Department of Internal Medicine, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Croatia Corresponding author: SUMMarY Although the SAPHO (synovitis, acne, pustulosis, hyper- Ivan Padjen, MD ostosis, osteitis) syndrome was defined as a distinct entity more than 20 years ago, its classification within the spectrum of inflammatory Department of Internal Medicine rheumatic diseases and the proper therapeutic approach are still a Division of Clinical Immunology and matter of debate. We present four patients diagnosed with the SAPHO Rheumatology syndrome treated and followed-up in our Department, demonstrating the diversity of their clinical courses and their responses to different University of Zagreb School of Medicine therapeutic approaches. We also review the clinical, laboratory, and University Hospital Centre zagreb imaging features of the SAPHO syndrome described in the relevant Kišpatićeva 12 literature. Despite the growing quantity of published data on the clini- 10000 Zagreb, Croatia cal features of the syndrome and the recognition of two disease pat- terns (inflammatory and bone remodeling disease), it is still not clear [email protected] whether these possible disease subsets require different therapeutic strategies. Tumor necrosis factor-alpha (TNF-α) inhibitors have been Received: April 8, 2014 suggested to be effective in patients with the inflammatory pattern, whereas bisphosphonates seem to be effective in patients with bone Accepted: July 10, 2014 remodeling disease; however, this is still a hypothesis not yet confirmed by adequately designed clinical studies.
    [Show full text]
  • Extrafacial Granuloma Faciale
    Journal of the American Osteopathic College of Dermatology Volume 11, Number 1 SPONSORS: ',/"!,0!4(/,/'9,!"/2!4/29s-%$)#)3 July 2008 34)%&%,,!"/2!4/2)%3s'!,$%2-! www.aocd.org Journal of the American Osteopathic College of Dermatology Journal of the American Osteopathic College of Dermatology 2007-2008 Officers President: Jay Gottlieb, DO President Elect: Donald Tillman, DO First Vice President: Marc Epstein, DO Second Vice President: Leslie Kramer, DO Third Vice President: Bradley Glick, DO Secretary-Treasurer: Jere Mammino, DO (2007-2010) Immediate Past President: Bill Way, DO Trustees: James Towry, DO (2006-2008) Mark Kuriata, DO (2007-2010) Karen Neubauer, DO (2006-2008) David Grice, DO (2007-2010) Sponsors: Global Pathology Laboratory Editors Stiefel Laboratories Jay S. Gottlieb, D.O., F.O.C.O.O. Medicis Stanley E. Skopit, D.O., F.A.O.C.D. James Q. Del Rosso, D.O., F.A.O.C.D. Galderma Editorial Review Board Ronald Miller, D.O. JAOCD Eugene Conte, D.O. Founding Sponsor Evangelos Poulos, M.D. Stephen Purcell, D.O. Darrel Rigel, M.D. !/#$s%)LLINOISs+IRKSVILLE -/ s&!8 Robert Schwarze, D.O. WWWAOCDORG Andrew Hanly, M.D. #/092)'(4!.$0%2-)33)/.WRITTENPERMISSIONMUSTBEOBTAINED Michael Scott, D.O. FROMTHE*OURNALOFTHE!MERICAN/STEOPATHIC#OLLEGEOF$ERMATOLOGY FORCOPYINGORREPRINTINGTEXTOFMORETHANHALFPAGE TABLESORlGURES Cindy Hoffman, D.O. 0ERMISSIONSARENORMALLYGRANTEDCONTINGENTUPONSIMILARPERMISSION Charles Hughes, D.O. FROMTHEAUTHORS INCLUSIONOFACKNOWLEDGEMENTOFTHEORIGINALSOURCE ANDAPAYMENTOFPERPAGE TABLEORlGUREOFREPRODUCEDMATERIAL Bill Way, D.O. 0ERMISSIONFEESAREWAIVEDFORAUTHORSWISHINGTOREPRODUCETHEIROWN Daniel Hurd, D.O. ARTICLES2EQUESTFORPERMISSIONSHOULDBEDIRECTEDTO*!/#$CO!/#$ 0/"OX+IRKSVILLE -/ Mark Lebwohl, M.D. #OPYRIGHTBYTHE*OURNALOFTHE!MERICAN/STEOPATHIC#OLLEGEOF Edward Yob, D.O. $ERMATOLOGY Jere Mammino, D.O. Printed by: Stoyles Graphics Services, Mason City, IA 50401 Schield M.
    [Show full text]
  • Eosinophilic Cellulitis (Wells Syndrome): a Case Report
    International Journal of Research in Dermatology Baabdullah AM et al. Int J Res Dermatol. 2021 May;7(3):450-453 http://www.ijord.com DOI: https://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20211708 Case Report Eosinophilic cellulitis (wells syndrome): a case report Ahmad Mohammad Baabdullah1, Khalid Ali Al Hawsawi2, Bashayr Saad Alhubayshi3*, Marwa Rashed Gammash4 1Department of Dermatology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia 2 Department of Dermatology, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia 3Taibah College of Medicine, Taibah University, Almadinah Almunawwarah, Saudi Arabia 4Collage of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia Received: 14 January 2021 Accepted: 08 February 2021 *Correspondence: Dr. Bashayr Saad Alhubayshi, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Eosinophilic Cellulitis is also known as Wells syndrome is uncommon dermatitis, characterized by the infiltration of eosinophils in the dermis. The exact etiology of the disease is unknown. Clinically, it is highly varied but commonly the presentation is pruritic erythematous plaque. We report a case of one and half years old healthy boy who developed itchy bullae on the dorsum of his hand with multiple erythematous papules over his extremities that started immediately after his vaccines. Histopathological examination of the lesion showed infiltrate eosinophils with typical flame figures. The case was successfully treated with corticosteroid course.
    [Show full text]
  • 12.2% 116000 120M Top 1% 154 3800
    We are IntechOpen, the world’s leading publisher of Open Access books Built by scientists, for scientists 3,800 116,000 120M Open access books available International authors and editors Downloads Our authors are among the 154 TOP 1% 12.2% Countries delivered to most cited scientists Contributors from top 500 universities Selection of our books indexed in the Book Citation Index in Web of Science™ Core Collection (BKCI) Interested in publishing with us? Contact [email protected] Numbers displayed above are based on latest data collected. For more information visit www.intechopen.com 5 Expression of Tumor Necrosis Factor-Alpha (TNF-TNF-Converting Enzyme and Matrix Metalloproteinase-3 in SAPHO Syndrome Synovium - A Rare Case Accompanied by Acrodermatitis Continua of Hallopeau: A Case Report and Review of Anti-TNF-Therapy Koichiro Komiya1, Nobuki Terada1, Yoshikazu Mizoguchi2 and Harumoto Yamada3 1Department of Orthopaedic Surgery, Fujita Health University Second Hospital 2Department of Pathology, Fujita Health University Second Hospital 3Department of Orthopaedic Surgery, Fujita Health University Japan 1. Introduction Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disorder characterized by osteoarticular and dermatological manifestations. The denotation was first proposed by Chamot et al. in 1987 after investigation of 85 cases (Chamot et al., 1987). The most common site of SAPHO syndrome is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions and hyperostosis. The axial skeleton and peripheral bones can be involved. Peripheral synovitis is also common. Skin manifestations include palmoplantar pustulosis (PPP), severe acne and various patterns of psoriasis. The pathogenesis of SAPHO syndrome has not been determined.
    [Show full text]
  • Urticaria from Wikipedia, the Free Encyclopedia Jump To: Navigation, Search "Hives" Redirects Here
    Urticaria From Wikipedia, the free encyclopedia Jump to: navigation, search "Hives" redirects here. For other uses, see Hive. Urticaria Classification and external resourcesICD-10L50.ICD- 9708DiseasesDB13606MedlinePlus000845eMedicineemerg/628 MeSHD014581Urtic aria (or hives) is a skin condition, commonly caused by an allergic reaction, that is characterized by raised red skin wheals (welts). It is also known as nettle rash or uredo. Wheals from urticaria can appear anywhere on the body, including the face, lips, tongue, throat, and ears. The wheals may vary in size from about 5 mm (0.2 inches) in diameter to the size of a dinner plate; they typically itch severely, sting, or burn, and often have a pale border. Urticaria is generally caused by direct contact with an allergenic substance, or an immune response to food or some other allergen, but can also appear for other reasons, notably emotional stress. The rash can be triggered by quite innocent events, such as mere rubbing or exposure to cold. Contents [hide] * 1 Pathophysiology * 2 Differential diagnosis * 3 Types * 4 Related conditions * 5 Treatment and management o 5.1 Histamine antagonists o 5.2 Other o 5.3 Dietary * 6 See also * 7 References * 8 External links [edit] Pathophysiology Allergic urticaria on the shin induced by an antibiotic The skin lesions of urticarial disease are caused by an inflammatory reaction in the skin, causing leakage of capillaries in the dermis, and resulting in an edema which persists until the interstitial fluid is absorbed into the surrounding cells. Urticarial disease is thought to be caused by the release of histamine and other mediators of inflammation (cytokines) from cells in the skin.
    [Show full text]
  • Eosinophilic Gastroenteritis Presenting with Severe Anemia and Near Syncope
    J Am Board Fam Med: first published as 10.3122/jabfm.2012.06.110269 on 7 November 2012. Downloaded from BRIEF REPORT Eosinophilic Gastroenteritis Presenting with Severe Anemia and Near Syncope Nneka Ekunno, DO, MPH, Kirk Munsayac, DO, Allen Pelletier, MD, and Thad Wilkins, MD Eosinophilic gastrointestinal disorders or eosinophilic digestive disorders encompass a spectrum of rare gastrointestinal disorders that includes eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Eosinophilic gastroenteritis is a rare inflammatory disease characterized by eosino- philic infiltration of the gastrointestinal tract. The clinical manifestations include anemia, dyspepsia, and diarrhea. Endoscopy with biopsy showing histologic evidence of eosinophilic infiltration is consid- ered definitive for diagnosis. Corticosteroid therapy, food allergen testing, elimination diets, and ele- mental diets are considered effective treatments for eosinophilic gastroenteritis. The treatment and prognosis of eosinophilic gastroenteritis is determined by the severity of the clinical manifestations. We describe a 24-year-old woman with eosinophilic gastroenteritis presenting as epigastric pain with a history of severe iron deficiency anemia, asthma, eczema, and allergic rhinitis, and we review the litera- ture regarding presentation, diagnostic testing, pathophysiology, predisposing factors, and treatment recommendations. (J Am Board Fam Med 2012;25:913–918.) Keywords: Case Reports, Eosinophilic Gastroenteritis, Gastrointestinal Disorders copyright. A 24-year-old nulliparous African-American woman During examination, her height was 62 inches, was admitted after an episode of near syncope asso- weight 117 lb, and body mass index 21.44 kg/m2. Her ciated with 2 days of fatigue and dizziness. She re- heart rate was 111 beats per minute, blood pressure ported gradual onset of dyspepsia over 2 to 3 121/57 mm Hg, respiratory rate 20 breaths per minute, months.
    [Show full text]
  • Dermatopathology
    Dermatopathology Clay Cockerell • Martin C. Mihm Jr. • Brian J. Hall Cary Chisholm • Chad Jessup • Margaret Merola With contributions from: Jerad M. Gardner • Talley Whang Dermatopathology Clinicopathological Correlations Clay Cockerell Cary Chisholm Department of Dermatology Department of Pathology and Dermatopathology University of Texas Southwestern Medical Center Central Texas Pathology Laboratory Dallas , TX Waco , TX USA USA Martin C. Mihm Jr. Chad Jessup Department of Dermatology Department of Dermatology Brigham and Women’s Hospital Tufts Medical Center Boston , MA Boston , MA USA USA Brian J. Hall Margaret Merola Department of Dermatology Department of Pathology University of Texas Southwestern Medical Center Brigham and Women’s Hospital Dallas , TX Boston , MA USA USA With contributions from: Jerad M. Gardner Talley Whang Department of Pathology and Dermatology Harvard Vanguard Medical Associates University of Arkansas for Medical Sciences Boston, MA Little Rock, AR USA USA ISBN 978-1-4471-5447-1 ISBN 978-1-4471-5448-8 (eBook) DOI 10.1007/978-1-4471-5448-8 Springer London Heidelberg New York Dordrecht Library of Congress Control Number: 2013956345 © Springer-Verlag London 2014 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfi lms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifi cally for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work.
    [Show full text]
  • Basics of Hematology and Patho-Histology
    Basics of Hematology and Patho-histology Practical Course in Molecular Pathology Winter Term 2015 Ernst Müllner MFPL (Max F Perutz Laboratories) Department of Medical Biochemistry Medical University of Vienna [email protected] www.mfpl.ac.at/mfpl-group/group/muellner.html (Müllner homepage / research) E. coli + macrophages medicalschool.tumblr.com/post/43914024728/sem-image-of-e-coli-bacteria-and-macrophages medicalschool.tumblr.com/post/18256087351/r ed-blood-cells-erythrocytes-trapped-by-fibrin Overview on main white blood cell (WBC) types – (Wikipedia) Mature white blood cell types I White Blood cells (WBCs) are frequently also referred to as peripheral blood mononuclear cells (PBMCs). Granulocytes in general are part of the innate immune system. Names derive from staining with hematoxylin and eosin. Whereas basophils stain dark blue and eosinophils are bright red, neutrophils stain neutral to pink. Basophil granulocytes Eosinophil granulocytes Neutrophil granulocytes Least common granulocyte type About 1-6% of WBCs; component Most abundant WBC type (40- (0.01- 0.3% of WBCs. Large of innate immune system to com- 75%) and essential part of the cytoplasmic granules obscure the bat parasites and certain infec- innate immune system. A patho- nucleus under the microscope. tions; also associated with allergy gen is likely to first encounter a When unstained, the nucleus is and asthma. Following activation, neutrophil. Normally contain a nu- visible and usually has 2 lobes. eosinophils effector functions in- cleus of 2-5 lobes. Neutrophils Basophils appear in inflammatory clude production and release (de- quickly congregate at a infection reactions, particularly those granulation) of cytotoxic substan- site, attracted by cytokines from causing allergies, mainly via the ces (granule proteins, reactive activated endothelium, mast cells, vasodilator histamine (antihistami- oxygen species …) and production or macrophages.
    [Show full text]