* Erythema* TELANGIECTASES* Urticaria* PURPURA HENOCH – SCHÖNLEIN PURPURA By: Dr. Faraedon Kaftan College of Medicine Sulaimani University 2011 - 2012
1. Erythema:* is redness of the skin due to dilatation & an increase blood within the small skin blood vessels and leads to hyperemia in specific area of the skin in response to endogenous or exogenous factors* Fades on pressureFlushing:is Transient Erythema usually of the faceBlushing: is Flushing caused by Emotional FactorsErythema: is of 2 types1- Localized erythema2- Diffuse (Generalized) erythema
1- Localized erythematous eruptions are due to:a. Trauma, friction & sweating→ (Erythema intertrigo)b. Heat: (Erythema-Ab-Igni), (Erythema caloricum)c. Chemical irritantsd. Light: exposure to sun: (Erythema solare)e. Cold: Erythema pernio:(affects the acral parts )f. Neoplasmg. Reticulosis.h. Collagen diseasesi. Urticariaj. Pemphigoid (Pd)k. Liver cirrhosis:→ (Erythema palmare) Other types of erythema may occur in response to certain types of food, drugs, vaccines, stress, gastro-intestinal disturbances and vasomotor liability.
1- Localized erythemaErythema intertrigo: Erythema Ab Igne:friction & sweating (heat)Erythema solare : Erythema palmare Erythema pernio(exposure to sun: Light)
2- Diffuse erythematous eruptions due to systemic factors. a. Drugs b. Virus infection c. Bacterial infections d. Pregnancy.Types of Diffuse (Generalized) erythema E. Toxicum Neonatorum (ETN):(Toxic E. of the newborn) Exanthematous E. (EE) E. Infectiosum (EI): (Fifth disease):(Margarine disease) E. Annulare Centrifugum (EAC) E. Chronicum Migrans Erythema due to drug reaction (Corticosteroids) E. Multiforme (EM): (E. exudativum) E. Nodosum (EN) Erythroderma E. Annulare Rheumaticum E. Elevatum Diutinum (EED)
E. Toxicum Neonatorum (ETN): (Toxic E. of the newborn) appears in the 1st 3 or 4 days of life & disappears by the 2nd wk erythematous blotchy macules, papules or pustules mainly on the trunk , face and proximal parts of the limbs The papules may be surmounted by small pustules, 2-4 mm In more severe cases, urticarial papules arise within the erythematous areas, on the back and buttocks. The infant’s general health is good usually the lesions fade away without treatment .D. D. Toxic erythema of the newborn:1. Pustular miliaria,2. Herpes simplex virus infection .3. Incontinentia pigmenti .4. Neonatal pustular pyoderma
Exanthematous E. (EE): (Viral Exanthem)* Erythema is variably generalized and specific* Viral and bacterial diseases are common causes in infants & children* Types: Measles, erythema infectiosum (fifth disease), roseolaand scarlet fever
E. Infectiosum (EI):(Fifth disease):(Margarine disease)- Viral epidemic types ofgeneralized erythema, slightlyinfectious in spring & summer- The erythema appears on theproximal parts of the extremities,the face (butterfly appearance)and spread to cover the entire skinsurface.- Itching is severe,- on the fifth day erythemaregresses & itching becomes less.
Causes of EM:1- Virus infections: H. S. (frequently), atypical pneumonia, Mycoplasma infection, Lymphogranuloma ingunale (LGI), psittacosis, Variola, Vaccinia, Hepatitis, Milker’s nodules, Orf, Infectious mononucleosis (IM), Mumps & Poliomyelitis2- Histoplasmosis3- Bacterial infections4- X- ray therapy5- LE6- P. A. N. (Polyarteritis nodosa)7- Wegener’s granulomatosis8- Carcinoma, Reticulosis & Leukemia 9- Pregnancy, premenstrual.10- Drug reactions.11- Sarcoidosis12- Contact reactions13- Unknown.
Clinical features of EM:1- Papular form:2- Vesiculo- bullous form:3- Sever bullous form1- Papular form: * dull red, flat maculo- papules * reach a diameter of 1-2 cm in 48hs. * IRIS or (target lesion): is a characteristic sign A- The periphery of the papules remains red B- The centre becomes cyanotic or purpuric * These lesions fade in 1-2 wks * The sites affected are the: 1- Face 2- Backs of the hands, palms, wrists, forearms & elbows 3- Feet & Knees
2- Vesiculo- bullous form: * is intermediate in severity between: a. The papular type & b. Sever bullous type. * MMs are severely involved3- Sever bullous form(Stevens – Johnson syndrome):* is a very characteristic & sever type of E. M.* onset is usually sudden* numerous organs are affected: oral cavity, eyes, skin, male genitalia, anal mm, renal & Respiratory systems
D.D. of EM:1. Drug eruption2. L.E.: Lupus Erythematosus3. Pemphigoid4. TEN: (Toxic Epidermal Necrolysis)Treatment:*- In the papular type: Symptomatic*- In sever E.M.:1- Prednisolone (small) 30- 60mg daily & decreasing over a period of 2-4 wks.2- Systemic antibiotic to prevent 2ndary infections3- Zovirax tablet or syrup: when the cause is H. simplex
E. Nodosum (EN) most cases appear between 20 and 30 years of age 3-6 times more frequently in women than in men erythema nodosum is seen in younger patients than erythema induratum of Bazin. The typical eruption is quite characteristic and consists of: a sudden onset of symmetrical, tender (painful), erythematous, warm nodules and raised plaques usually located on the shins, ankles and knees. The eruption lasts from 3 to 6 weeksCauses: 3 S (streptococcal, Sulfa, Sarcoidosis) + Tb
The nodules in E. Nodosum (EN): Are bilateral 1 to 5 cm or more in diameter may become confluent resulting in erythematous plaques May involve thighs, extensor aspects of the arms, neck, and even the face. At first, show a bright red color and are raised above the skin. Within a few days, they become flat, with a livid red or purplish color. Finally, they exhibit a yellow or greenish appearance often taking on the look of a deep bruise, which is quite characteristic of erythema nodosum and allows a specific diagnosis in late stage lesions. Ulceration is never seen & heal without atrophy or scarring.Treatment Treatment of underlying associated condition Usually, nodules regress spontaneously within a few weeks, bed rest is often sufficient treatment. Aspirin and nonsteroidal anti-inflammatory drugs
Erythroderma Is intense and usually widespread reddening of the skin due to inflammatory skin disease often precedes or is associated with exfoliation (skin peeling off in scales or layers) known as exfoliative dermatitis (ED).Causes: Dermatitis especially atopic dermatitis, contact dermatitis (allergic or irritant) and stasis dermatitis (gravitational eczema) and in babies,seborrhoeic dermatitis, Psoriasis Pityriasis rubra pilaris Pemphigus and bullous pemphigoid Cutaneous T-cell lymphoma (Sezary syndrome) Drugs…. idiopathic called: red man syndromemay also be a symptom or sign of a systemic disease: Internal malignancies eg Ca. of rectum, lung, fallopian tubes, colon Haematological malignancies eg lymphoma, leukaemia Graft vs Host disease HIV infection
Management: Discontinue all unnecessary medications Monitor fluid balance and body temperature Maintain skin moisture with wet wraps, other types of wet dressings, emollients and mild topical steroids Antibiotics if secondary infection is present Antihistamines for severe itch If a cause can be identified then specific treatment should be started eg topical steroids for atopic dermatitis; acitretin or methotrexate for psoriasis.prognosis? depends on the underlying disease process. If the cause can be removed or corrected then prognosis is generally very good.
2. Telangiectasia:are Permanently Dilated Skin Small Vessels* Fades on pressure* Appears on the skin & mms as a small, dull red linear, stellate or punctate markings
Causes of Telangiectasia:1- Primary Telangiectasia:Vascular (Cherry angioma)→Angioma & AngiokeratomaAngiokeratoma Circumscriptum→Ataxia TelangiectasiaHHT: Hereditary Hemorrhagic T.
2- Secondary Telangiectasia: • Prolong vasodilatation as in: - Rosacea - Varicose Veins (V Vs) • Raynaud’s disease. • XP (Xeroderma pigmentosa) • Prolong exposure to sunlight or tar • Post – traumatic • Radio dermatitis • Atrophy e.g.: topical Cs. • L.E., Dermatomyositis, Scleroderma & Morphea. • Urticaria Pigmentosa (mastocytosis)
3. URTICARIA: Nettle rash or Hives Is an eruption of Transient Erythematous or edematous swelling of the Dermis or S.C. tissues for less than 48hs Fades on pressure While the similar but larger swellings of the S.C. tissues are called: - Angioedema or (Angioneurotic edema) - Quincke’s edema or (Giant Urticaria)* Urticaria & Angioedema are often associated
* Basic mechanism of Urticaria is Local permeabilityof capillaries & small venules by Mediators* Mediators are large No. of substances present in the body, they are:1- Histamine: is most important mediator & derived from mast cells2- Kinins3- Prostaglandin4- Leukotriens5- Complement6- Others (but not serotonin in man)Clinically there are 3 forms of Urticaria:1- Ordinary Urticarias: about 80% (last few hours)2- Physical Urticarias: 20% (last few minutes)3- Herediatory Angioedema* Sometimes Urticaria is classified in to:1- Acute Urticaria: less than 1.5 months2- Chronic Urticaria: more than 1.5 months
Provoking causes of Urticaria:1. Drugs:A- Histamine – releasing drugs as: * Codine, Curare, Dextran, Morphine & PolymyxinB- Drugs for unknown reason aggravate URTICARIA & Asthma: * Salicylates, Indomethacin & Benzoates2- Foods: eggs, nuts, chocolate, fish& shellfish & tomatoes3- Food additives: as a. The yellow azo dye tartarazine b. Benzoate4- Inhalants: as a. Grass pollens, b. Mould spores, c. Animal dander’s & d. House dust
5- Infections & Infestations.6- Psychological factors:7- General medical disorder asa. L. E. b. Reticulosis c. Polycythemiad. Carcinoma e. macroglobulinaemiaCl fs of Urticaria:The lesions are: Weals(Wheals) * may occur at any site, * of various sizes, * few or numerous, * characteristically intently itching, * have a white palpable centre of edema with a variable halo of erythema
* Weals are:round, irregular, annular or serpiginous* Weals last for a few hours in Ordinary Urticaria But in physical Urticaria Weals last for a few minutes onlyClinical patterns of Physical Urticarias: are AC DC PSH1- Aquagenic Urticaria (Water)2- Cold Urticaria3- Dermographism4- Cholinergic Urticaria5- Pressure Urticaria6- Solar Urticaria (Sun)7- Heat UrticariaIn general, Spontaneous improvement occurs evenin the absence of Diagnosis or TreatmentD.D. of Urticaria: PU (Insect bites), Pemphigoid, D.H., Atopic Eczema, Toxic erythema, E. M., Henoch- SchÖenlien Purpura or Follicular mucinosis
Treatment of Urticaria:1- Careful assessment of the cause2- Avoidance of salicylates & food additives3- Anti-candidia regime: Nystatin tablets4- Anti Histamines5- Systemic Corticosteroids (Cs):6- S.C. Adrenaline injection: in sever acute Urticaria7- Psychotherapy & sedative or tranquilizing drugs in psychogenic Urticarias
4. Purpura:- is longlasting appearance of red or purple discolorations on the skin or mms due to Extravasations of RBCs- is normally distinguished from Erythema when pressure by finger or Diascopy fails to blanch the lesion (does not fade on pressure)- Is a physical sign with many causes
* Extravasated blood is usually broken down to various other pigments derived from haem within 2 or 3wks* in 2 or 3 wks the red color of the blood changes to: purple, orange, brown, blue or green which occur in many purpuric lesions- Normal platelet count is: 150.000- 400.000 per microlitre of blood- Purpura due to Thrombocytopenic purpura occurs with a platelet count less than 50.000 per microlitre of blood
Petechiae: (pinpoint red spots)are small, purpuric lesions up to 2 mm across,occurring in crops (groups) Ecchymoses or bruises:are larger extravasations of bloodmore than 2 mm across
Predominantly in children, There is particular association of1- Skin rash (an erythematous-uriticarial & purpuric) with2- arthritis3- G. I. Symptoms or bothNephritis: Is particular complication of HSHPIn other word Nephritisgives HSHP a specialimportance.The cause is undetermined1. upper respiratory tract infections: - viruses, streptococcal infections food & drugs (may be important) but2. immune complex disease (ICD) (is at present most in favour)Diagnostic tests:1- Raised serum Ig A or2. Ig A deposition in the skin
Cl. f.: -* Prodromal symptoms of (headache, anorexia & fever) may precede an acute onset of1- Skin rash (Skin)2- Abdominal pain & (Abdomen)3- Arthralgia (Joints) These may occur together or all three may not be present in any one patient1- Skin Rashes:The sites of predilection(Skin Rashes) are:1- External aspects of the limbs2- Buttocks3- Face (occasionally)4- The oral mucosa (Rarely)
The eruption begins as a crop (group) ofErythematous Macules & most macules become1- Papular2- Uriticarial: Uriticarial component of the general picture is characteristic in HSHP3- Purpuric: A shower of purpura may be the presenting sign* Purpura may be the only symptom & always an outstanding (prominent) feature in HSHP* The diagnosis of HSHP must be suspected whenever Purpura occurs in children with a normal platelet count
2- Abdominal Symptoms: * In two- thirds or more: - colic, - vomiting & - diarrhea may be accompanied by hematamesis or (the passage of bloody stools)The onset may be sever enough to suggest an (acute abdomen)* A serosanguineous & Edematous involvement of a section of the bowel is more common than true Intussusceptions* There may be scrotal swelling due to torsion & subsequent hemorrhage.
3- Polyarthralgia* Occurs in most cases* Joints affected are : 1- The ankles 2- knees 3- Elbows & 4- Small joints of the hands & wrists are most frequently affectedRenal involvement:• Is the most serious feature of HSHP* Should receive the most careful attention because of its prognostic significance; 1- hematuria or 2- proteinuria (usually both)*Patients with renal involvement must be watched carefully for 5years.
D.D. of HSHP: * Skin lesions:1- Insect bites (Papular Urticaria)2- Drug eruptions3- E.M.4- Urticaria5- Gianotti-crosti syndrome: (Papular acrodermatitis):*Profuse eruption of a symmetrical red papules 5 -10 mm on the thighs & buttocks then on the extensor aspects of the arms, & finally on the face*Associated with A- Generalized lymphadenopathy mostly axillary & inguinal B- Hepatomegaly6. Transient recurring circinate erythema of Rheumatic fever
* Abdomen 1- Appendicitis 2-Mesenteric adenitis*Joints: Rheumatic fever* Meningococcal septicemia(if the systemic manifestations are sever)Course of HSHP:- Individual episodes usually last 3- 6 wks- but recurrences are common.Prognosis of HSHP:- Of each attack is good- For life depends on the 1- degrees & 2- severity of renal involvement
Treatment of HSHP:1- There is no specific treatment2- Rest is the best therapy3- Penicillin if there is evidence of concomitant streptococcal infection4- Cs: * decrease the symptoms * have no effect on the a- purpura or b- The nephropathy5- Dapsone if a- steroids fail or b- Cs: contraindicated
It is essential that aurine analysis be carried out:1. When the diagnosis is made2. Weekly during the attack3. At the end of the attack4. 1- 3 months after the attack has subsided* When persistent proteinuria is present, further regular tests are obligatory at intervals for at least 5 years.