S E C T I O N
8 Dermatologic Disorders
83 ATOPIC DERMATITIS 691.8
(Atopic Eczema, Disseminated
Neurodermatitis)
Carsten Heinz, MD
Muenster, Germany
Arnd Heiligenhaus, MD
Muenster, Germany
INCIDENCE/ETIOLOGY
Atopic dermatitis (AD) is a chronic disease with a prevalence of 10–20% in children and 1–3% in adults. In acute AD a Th2 dominated immune process with production of cytokines as IL-4, IL-5 and IL-13 is found, while in chronic AD the mircomilieu comprises of both Th1 and Th2 cytokines. AD may also be complicated by rhinitis or asthma. The etiology seems to be multivariant. Familial disease increases the risk 2 to 3 fold. The hygiene hypothesis proposes that an in utero and postnatal immune stimulation with infectious agents with a subsequent predominantly Th1 immune response results in a lower risk of atopic manifestation. Food allergens, aeroallergens, bacteria and also emotional stress have been implicated to stimulate AD. Serum IgE is often elevated in patients with AD and sensitization against a variety of environmental allergens is frequent. However, manifestation or exacerbation of AD also occurs in the absence of exposure to environmental allergens. The high level of IgE autoantibodies against a broad variety of human proteins often correlates with disease severity and may contribute to pathogenesis.
Conjunctiva
●Conjunctival injection, chemosis, mucus discharge, papillary reaction, Trantas dots, squamous cell carcinoma.
Cornea
●Superficial punctate keratopathy (SPK), shield ulcer, scars, keratoconus, vascularization.
Lens
●Cataract formation.
Retina
●Detachment due to rubbing of the eyelids or traumatic slapping.
TREATMENT
Systemic
●Avoidance of identified allergens.
●Oral corticosteroids.
●Systemic tetracycline as blepharitis treatment.
●Immunosuppression in severe cases, e.g. with cyclosporin A.
Topical
Skin
Lid hygiene, topical corticosteroids, calcineurin inhibitors (e.g. tacrolimus or pimecrolimus), antibiotics.
Ocular
Unpreserved lubricants, mast cell stabilisators, antihistamines, cyclosporin A, topical corticosteroid, antibiotics, therapeutic contact lenses
Surgical
DIAGNOSIS
Atopic dermatitis is a chronic relapsing, inflammatory skin disease that is characterized by pruritic eczematous skin lesion. Predominant localization varies with the patient’s age, with predilection on the extensor surfaces in young children and on the flexor surfaces in adults. Ocular involvement is reported in about 32%.
●Tarsorhaphie or punctual occlusion in SPK.
●Amniotic membrane transplantation in corneal epithelial defects or shield ulcers.
●Cataract extraction with IOL implantation.
●Keratoplasty for keratoconus.
●Retinal detachment surgery.
OCULAR
Eyelid
COMMENTS
Atopic dermatitis is a chronic skin disease that also affects the eyelid leading to severe discomfort. Treatment of the skin disease is often satisfactory and should be managed coopera-
●Erythema, exudates, crusting, scaling, blepharitis, secondtively with dermatologists. The severity of eye affections varies
ary infection. |
extremely and may lead to visual loss. Treatment strategies |
153
Disorders8 SECTIONDermatologic •
include lubricants, topical mast cell stabilizers antihistamines or topical ciclosporin, and systemic immunosuppression or surgical interventions may be required to preserve vision.
REFERENCES
Heinz C, Fanihagh F, Steuhl KP: Squamous cell carcinoma of the conjunctiva in patients with atopic eczema. Cornea 22(2):135–137, 2003.
Hida T, Tano Y, Okinami S, et al: Multicenter retrospective study of retinal detachment associated with atopic dermatitis. Jpn J Ophthalmol 44(4):407–418, 2000.
Hingorani M, Moodaley L, Calder VL, et al: A randomized, placebo-control- led trial of topical cyclosporin A in steroid-dependent atopic keratoconjunctivitis. Ophthalmology 105(9):1715–1720, 1998.
Leung DY, Bieber T: Atopic dermatitis. Lancet 361(9352):151–160, 2003.
Leung DY, Jain N, Leo HL: New concepts in the pathogenesis of atopic dermatitis. Curr Opin Immunol 15(6):634–638, 2003.
Novak N, Bieber T, Leung DY: Immune mechanisms leading to atopic dermatitis. J Allergy Clin Immunol 112(6 Suppl):S128–S139, 2003.
Schultz Larsen F, Diepgen T, Svensson A: The occurrence of atopic dermatitis in north Europe: an international questionnaire study. J Am Acad Dermatol 34(5 Pt 1):760–764, 1996.
Sturgill S, Bernard LA: Atopic dermatitis update. Curr Opin Pediatr 16(4):396–401, 2004.
Uchio E, Miyakawa K, Ikezawa Z, et al: Systemic and local immunological features of atopic dermatitis patients with ocular complications. Br J Ophthalmol 82(1):82–87, 1998.
84 CONTACT DERMATITIS 692.9
(Contact Allergy, Dermatitis Venenata)
Srini V. Goverdhan, MD, FRCSOphth
Southampton, England
ETIOLOGY/INCIDENCE
Contact dermatitis is a common immunologic skin condition which often presents to ophthalmologists as an eyelid or periorbital dermatitis. It results from exposure of the skin to a wide variety of substances with primary irritant properties commonly found in the environment, including drugs, dyes, plant resins, preservatives, cosmetics, resins, rubber derivatives and metals. Small incomplete hapten molecules bind to dermal protein, forming complete antigens. Initial exposure to haptens results in sensitization of CD8+ T lymphocytes, a second application triggers release of cytokines, which amplify the inflammatory response in the skin. There are three varieties of contact dermatitis:
1.Allergic, which is the common form;
2.Atopic; and
3.Irritant.
Allergic contact dermatitis (ACD), unlike the irritant variety, occurs only in sensitized individuals and involves the mechanism of cell-mediated immunity. In ACD, an individual becomes sensitized to a given chemical or other sensitizing substance, and with re-exposure to the same chemical, an erythematous delayed hypersensitivity skin reaction is elicited. In Glaucoma, ACD has been seen with use of brimonidine or betablockers (timolol, befunolol, and carteolol) where cross-
reactivity may occur as this is caused by a common aldehyde metabolite.
Atopic dermatitis (AD) is a common cause of chronic recurring eyelid dermatitis often developing as an atopic itchy red eye in childhood, but can also present in adulthood. Cell mediated immune defects within the skin and abnormal IgE levels have been described as possible mechanisms.
Irritant contact dermatitis (ICD) is caused by excessive moisture or by acids, alkalis, resins, or chemicals capable of injuring any person’s skin if persistent contact is allowed. Allergy or hypersensitivity plays no role in irritant contact dermatitis.
DIAGNOSIS
History is essential in making the diagnosis of contact dermatitis; a personal and family history of hay fever, asthma, eczema or childhood atopy supports the diagnosis of ACD. History may be positive for exposure to irritative or sensitizing substances including topical ocular medications like neomycin, atropine derivatives, preservatives (benzalkonium chloride, benzyl alcohol, thimerosal, etc.) and anti-glaucoma medications (betablockers). Patch testing is often required for an adequate diagnosis of ACD or ICD. Withdrawal of the offending substance often alleviates signs and symptoms.
Clinical signs and symptoms
Signs and symptoms include erythematous, edematous, exudative, and pruritic skin lesions; scaling, ulceration, and edema of the eyelids; conjunctivitis and keratitis, especially inferiorly; and punctate corneal staining or infiltrates, especially inferiorly; itching of the skin and of the eye itself. With chronicity, the periorbital skin, particularly the upper eyelid becomes eczematous and lichenified.
TREATMENT
Systemic
●Poison ivy or poison oak dermatitis may require a course of oral corticosteroids. Desensitization may also be helpful. Antihistamines and mast-cell stabilizers can help control atopic symptoms in AD. Local reactions rarely require systemic therapy.
Local
●The allergen or irritant (determined by patch testing) should be removed from the patient’s environment.
●Topical corticosteroids may lead to the rapid relief of symptoms.
●Saline compresses and steroid lotions are helpful for skin lesions.
SUMMARY
Contact sensitivity is a common ophthalmologic problem because of exposure to chemicals in eye drops. The best form of treatment is discontinuation or avoiding the offending substance. Steroid therapy can reduce symptoms, but it is not recommended on a long-term basis. Patch testing can be very helpful in determining the cause of contact dermatitis and in selecting alternative drugs to which the patient is not sensitive.
154
REFERENCES
Friedlaender MH: Allergy and immunology of the eye. New York, Raven, 1993:79–82.
Guin JD: Eyelid dermatitis: a report of 215 patients. Contact Dermatitis 50:87–90, 2004.
Holdiness MR: Contact dermatitis to topical drugs for glaucoma. Am J Contact Dermatitis 12:217–219, 2001.
Manni G, Centofanti M, Sacchetti M, et al: Demographic and clinical factors associated with development of brimonidine tartrate 0.2%- induced ocular allergy. J Glaucoma 13:163–167, 2004.
Zug KA, Palay DA, Rock B, et al: Dermatologic diagnosis and treatment of itchy red eyelids. Surv Ophthalmol 40:293–306, 996.
85 ERYTHEMA MULTIFORME MAJOR
695.1
(Erythema Multiforme Exudativum, Stevens–Johnson Syndrome, Toxic Epidermal Necrolysis)
Sharon S. Lehman, MD
Wilmington, Delaware
ETIOLOGY/INCIDENCE
Erythema multiforme, Stevens–Johnson (SJS) and toxic epidermal necrolysis (TEN) represent a spectrum of disease characterized by an acute mucocutaneous reaction caused by hypersensitivity reaction to infection or drugs with activated T-lymphocytes acting destructively at the dermo-epidermal junction.
Erythema multiforme is a disease ranging in severity from mild (erythema multiforme minor), which is a benign condition without mucosal involvement, to severe (erythema multiforme major), which is characterized by bullous-erosive mucocutaneous reactions.
SJS, a form of erythema multiforme major, has limited full thickness epidermal necrosis, marked constitutional symptoms and low mortality rate. TEN is characterized by epidermal necrosis involving greater than 30% of the body surface with marked long term complications and mortality.
COURSE/PROGNOSIS
●The prodromal period before skin lesion usually includes fever, malaise, sore throat and arthralgias.
●Erythematous macules, papules, and the characteristic bull’s eye target skin lesions occur on the skin and ocular involvement includes:
●Eyelid: edema, erythema, and crusting occur initially with later entropion, scarring and trichiasis;
●Conjunctiva: initial chemosis and inflammatory pseudomembrane occur with later keratinization, scarring, symblepharon, ankyloblepharon, and a dry eye;
●Cornea: epithelial defects, limbal stem cell deficiency, corneal ulcers, vascularization, scarring and perforation occur.
DIAGNOSIS
Clinical signs and symptoms
The target, or bull’s eye, lesions of erythema multiforme are diagnostic and are recognized by the central, dark-purple area or a blister surrounded by a pale, edematous, circular zone, which in turn is surrounded by a peripheral rim of erythema. The characteristic involvement of the oral and conjunctival mucosa can also aid in the diagnosis of Stevens–Johnson syndrome.
TREATMENT
Systemic
●Multispecialty care, often in an ICU or burn unit, is required.
●Use of steroids is controversial.
●Use of antibiotics is based on clinical course and cultures.
●Immunosuppresive agents continue to be studied.
Ocular
●Gentle eyelid hygiene should be performed as necessary.
●Preservative free artificial tear ointment should be frequently and liberally applied.
●Use of antibiotics is based on clinical course and cultures.
●Use of steroids is controversial.
●Use of topical anesthetics should be limited because of ocular surface toxicity.
●Daily examination and disruption of symblepharon is required.
●Chronic dry eye requires continued use of preservative free artificial tear products and consideration may be given to the use of topical cyclosporin A.
Surgical
●Consider symblepharon ring for prevention of adhesions if tolerated by patient.
●Consider amniotic membrane transplantation (AMT) in acute phase in order to promote healing and inhibit inflammation, vascularization and scar.
●Treatment for chronic dry eye may require punctual occlusion and tarsarrhaphy.
●Treatment for trichiasis and entropion may require epilation, cryoablation, electolysis and eyelid reconstruction.
●Treatment of severely affected eyes requires consideration of AMT, limbal stem cell transplantation and corneal transplantation with possible immunosuppression.
COMMENTS
The treatment of erythema multiforme major, SJS and TEN remains challenging. New treatments such as immunomodulatory therapy offer new hope but require further testing for proof of therapeutic efficacy.
REFERENCES
Foulks GN: The evolving treatment of dry eye. Ophthalmol Clin N Am 16:29–35, 2003.
John T, Foulks GN, John ME, et al: Amniotic membrane in the surgical management of acute toxic epidermal necrolysis. Ophthalmology 109:351–360, 2002.
Major Multiforme Erythema • 85 CHAPTER
155
Disorders8 SECTIONDermatologic •
Lehman SL: Long-term ocular complication of Stevens-Johnson syndrome. Clin Pediatr 38:425–427, 1999.
McKenna JK, Leiferman KM: Dermatologic drug reactions. Immunol Allergy Clin North Am 24:399–423, 2004.
Metry DW, Jung P, Levy ML: Use of intravenous immunoglobulin in children with Stevens-Johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics 112:1430–1436, 2003.
Samson CM, Nduaguba C, Baltatzis S, et al: Limbal stem cell transplantation in chronic inflammatory eye disease. Ophthalmology 109:862– 868, 2002.
●Conjunctiva: interpalpebral or diffuse hyperemia, papillary and/or follicular reaction, pinguecula, scarring;
●Cornea: punctate erosions, pannus, neovascularization, lipid deposition, spade-shaped infiltrates, scarring, thinning, ulceration, perforation, phlyctenule;
●Sclera: episcleritis, scleritis.
The prevalence of ocular complaints in patients with acne rosacea is estimated at between 45% and 85%. The primary symptoms include burning, itching, tearing, foreign body sensation, redness and pain; however, the symptoms may be out of proportion to the clinical signs of the disease.
86 OCULAR ROSACEA 695.3
Vinicius Coral Ghanem, MD
Joinville, Santa Catarina, Brazil
Mark J. Mannis, MD, FACS
Sacramento, California
ETIOLOGY/INCIDENCE
Acne rosacea is a chronic cutaneous disease of unknown etiology. It affects up to 10% of the population, most notably fairskinned women from 30 to 60 years of age. Theories of its pathogenesis have included gastrointestinal, psychological, infectious, climatic, pharmacological and immunological causes.
Laboratory findings
None.
Differential diagnosis
●Blepharitis, meibomitis, recurrent chalazion, sebaceous gland carcinoma.
●Staphylococcal and seborrheic blepharokeratoconjunctivitis.
●Dry eye syndrome.
●Medication toxicity.
●Interstitial keratitis.
●Skin disease:
●Acne vulgaris;
●Seborrheic dermatitis;
●Chronic topical corticosteroid therapy;
●Alcohol abuse;
●Phototherapy;
●Lupus erythematosus, perioral dermatitis, essential telangiectasia, tuberculosis, syphilis, dermatomyositis, etc.
COURSE/PROGNOSIS
Rosacea is most commonly seen in adults although it has been reported in young children. The course may be characterized by recurrent inflammatory attacks of decreasing severity and frequency, or more commonly by chronic disease that lasts for more than 10 years, presenting chronic complications such as persistent facial edema, keratitis and rhinophyma. The prognosis of ocular rosacea is generally good. Nevertheless, corneal involvement may be as high as 30% in cutaneous rosacea and up to 85% in frank ocular rosacea.
DIAGNOSIS
Clinical signs and symptoms
Primary features include flushing (transient erythema), nontransient (persistent) erythema, papules, pustules and telangiectasias. The presence of one or more of these features with a central facial distribution is suggestive of rosacea. Secondary features include burning or stinging, plaques, dry appearance, edema, phymatous changes and involvement of more peripheral locations (neck, chest, scalp, ears and back). Persistent erythema and telangiectasia between acute episodes of inflammation are typical signs.
Ocular involvement, commonly referred to as ocular rosacea, occurs in more than 50% of patients, usually affecting both eyes simultaneously. Clinical findings may be confined to the eyes and may include:
TREATMENT
Systemic
The use of systemic antibiotics is the mainstay of treatment in most cases. We would stress that the treatment goal is to reduce the signs and symptoms rather than effecting a cure. Tetracycline is the most common initial regimen, 250 mg orally every 6 hours for a time period of 3 to 4 weeks, tapering to a maintenance dosage with improvement of the clinical condition. Alternatively, one can use 100 mg of doxycycline taken orally two times daily for 3–4 weeks with a subsequent taper. Symptomatic improvement commonly occurs before the clinical signs ameliorate and generally occurs 2 to 4 weeks after initiation of treatment. Patients who have recurrent disease or potentially sight-threatening complications may require a maintenance dose of up to 250 mg of tetracycline or 50 mg of doxycycline daily or every other day indefinitely. Abrupt withdrawal of treatment may cause an exacerbation of the disease. Recently, a sub-antimicrobial-dose doxycycline (doxycycline hyclate, 20mg twice daily) has demonstrated results comparable to conventional doses of tetracyclines, but with significant fewer side effects. The effectiveness of this therapy has yet to be evaluated for ocular rosacea. Supplements rich in Omega 3 (flaxseed oil
— 500 to 1000 mg 3 times per day) may also be helpful in reducing ocular symptoms.
For dermatologic disease, oral metronidazole or erythromycin can also be employed. These treatment methods are less
●Eyelid: telangiectasias and erythema of the lid margin, meieffective for cases of persistent lymphedema and rhinophyma,
bomian gland dysfunction, anterior blepharitis, recurrent |
while oral isotretinoin has been used successfully in resistant |
chalazion/hordeolum, madarosis, trichiasis; |
cases and in rhinophyma. |
156
Ocular
Proper lid hygiene is fundamental in the successful treatment of ocular rosacea. Warm compresses, lid margin massage (meibomian gland expression), and careful cleaning of the cilia and lid margin with dilute baby shampoo or commercially available lid scrubs are very helpful. The frequency of lid hygiene depends on the severity of the disease and is generally performed 2–4 times per day.
Approximately 40% of rosacea patients have signs of keratoconjunctivitis sicca. For this reason, artificial tears, preferentially without preservatives, should be used frequently. Punctal plugs may be helpful. Systemic antibiotics, lid hygiene and artificial tears in combination, may aid in restoration of the ocular surface by reduction of Staphylococcus aureus toxins and toxic free fatty acids present on the tear film.
In cases of severe lid margin disease, topical antibiotic and corticosteroid ointment may be used cautiously for short periods of time to control the acute exacerbations. Corticosteroid eye drops may be used, but with caution and preferably only for severe ocular inflammatory signs and symptoms.
Topical
Topical medications are usually combined with systemic treatment for successful control of symptoms and signs, especially in the case of ocular disease. The use of metronidazole 0.75% or 1% gel, sodium sulfacetamide 10%/sulfa 5% as a topical formulation or azelaic acid have shown good results. Topical corticosteroids must be used cautiously and for short periods, since their chronic usage can cause worsening of vasodilatation and telangiectasias.
Surgical
Surgical treatment is reserved for the most severe cases of ocular rosacea that develop significant corneal thinning or impending or actual perforation. Surgical options may include: conjunctival flap, cyanoacrylate adhesive application, or lamellar or penetrating keratoplasty. Before considering a transplant for optical purposes, it is essential that any inflammatory signs be controlled. Even so, these corneas are usually vascularized and transplant surgery is high-risk. Maintenance of systemic and ocular treatment is vital.
Dermatological surgical treatment is reserved for selected cases of telangiectasias and rhinophyma.
COMPLICATIONS
The absorption of tetracycline may be hindered by food intake. Therefore, tetracycline should not be taken during meals or along with dairy products, antacids, or iron preparations. Doxycycline absorption, on the other hand, is not affected by food intake. Neither tetracycline nor doxycycline should be used in children under 12 years of age or in pregnant or nursing women. Patients using these drugs should be advised of gastrointestinal side-effects and photosensitivity.
COMMENTS
Rosacea is a relatively common and frequently under-diagnosed disease by ophthalmologists, who may not carefully examine the patient’s face. Considering that there are no objective diagnostic tests currently available, both the ophthalmologist as well as the dermatologist must rely on clinical findings, which
may be subtle. A careful facial examination with adequate illumination is essential. In 20% of the cases, ocular symptoms appear before skin disease, and since the ocular signs may be non-specific, the clinical diagnosis may be difficult. Nevertheless, if the ocular signs are diagnostic, empirical treatment should be initiated. Patient follow-up and management must be multidisciplinary, including not only the dermatologist but also the ophthalmologist.
REFERENCES
Akpek EK, Merchant A, Pinar V, et al: Ocular rosacea: patient characteristics and follow-up. Ophthalmology 104:1863–1867, 1997.
Alvarenga LS, Mannis MJ: Ocular rosacea. Ocul Surf 3(1):41–58, 2005.
Brown SI, Shahinian J, Jr: Diagnosis and treatment of ocular rosacea. Ophthalmology 85:779–786, 1978.
Browning DJ, Proia AD: Ocular rosacea. Surv Ophthalmol 31:145–158, 1986.
Frucht-Pery J, Chayet AS, Feldman ST, et al: The effect of doxycycline on ocular rosacea. Am J Ophthalmol 107:434–436, 1989.
Frucht-Pery J, Sagi E, Hemo I, et al: Efficacy of doxycycline and tetracycline in ocular rosacea. Am J Ophthalmol 116:88–92, 1993.
Ghanem VC, Mehra N, Wong S, et al: The prevalence of ocular signs in acne rosacea: comparing patients from ophthalmology and dermatology clinics. Cornea 22(3):230–233, 2003.
Macsai MS, Mannis MJ, Huntley AC: Acne rosacea. In: Mannis MJ, Macsai MS, Huntley AC, eds. Eye and skin disease. Philadelphia, LippincottRaven, 1996:335–341.
Quaterman MJ, Johnson DW, Abele DC, et al: Ocular rosacea: signs, symptoms, and tear studies before and after treatment with doxycyline. Arch Dermatol 133:49–54, 1997.
Sneddon IB: A clinical trial of tetracycline in acne rosacea. Br J Dermatol 78:649–652, 1966.
Wilkin J, Dahl M, Detmar M, et al: Standard grading system for rosacea: report of the national rosacea society expert committee on the classification and staging of rosacea. J Am Acad Dermatol 50(6):907–912, 2004.
87 PSORIASIS 696.1
Rubén Queiro, MD
Oviedo-Asturias, Spain
Juan J Barbón, MD
Avilés-Asturias, Spain
Daniel de la Mano, MD
Avilés-Asturias, Spain
ETIOLOGY/INCIDENCE
Psoriasis is a chronic skin disorder characterized by excessive proliferation of the epidermis. Epidermal cells in psoriatic patches have lost regulatory control and exhibit accelerated growth. It can begin at any age but most commonly arises in the third decade. It is more common in white people and its prevalence is similar in both sexes. Psoriasis affects 1% to 2% of general population, and 7% to 40% of these patients develop arthritis as the main systemic complication. Most patients have minimal disease, but 15% have severe generalized disease. Psoriasis typically lasts for life, and infections, psychological distress, trauma, and some therapies are common causes of exacerbation. The etiology is unknown, but genetical and immunological factors are relevant in the etiopathogenesis.
Psoriasis • 87 CHAPTER
157
Disorders8 SECTIONDermatologic •
DIAGNOSIS
Clinical signs and symptoms
Systemic
●Characterized by sharply demarcated , geographically shaped, erythematous patches of skin covered with coarse, dry, silvery scales.
●When hyperkeratotic scales are removed, small blood droplets develop within seconds (Auspitz’s sign).
●Scalp, nails, elbows, knees, extensor surfaces of the extremities, and sacral region most commonly affected.
Ocular
●Affects about 10% of patients with psoriasis.
●Lids:
●May develop plaques.
●Lid margins can be affected by scaling, with trichiasis and madarosis in severe cases.
●An obstructive type of meibomian gland dysfunction has been described in psoriatic patients.
●Conjunctiva:
●Nonspecific conjunctivitis.
●Yellow plaque-like lesions.
●Limbal phlyctenule-like lesions.
●Cornea:
●Superficial and deep opacities.
●Vascularization, infiltration, and melting can occur, especially peripherally.
●Usually corneal involvement when there is active lid disease.
●Uveitis:
●The risk of uveitis is about 7% to 18%, especially in those affected by advanced spondylitis.
●Uveal inflammation may be anterior, posterior, and sometimes bilateral.
●Oral retinoids, particularly in association with UV therapy.
Ocular
●Topical corticosteroids for lid lesions, conjunctivitis, and uveitis.
●Adhesive dressings and detergent scrubs may be also used for skin lesions.
●Topical tar, anthralin derivates, and UV therapy probably not appropriate for lid lesions.
●Corneal melting: lubrication, bandage contact lenses, tarsorrhaphy, and treatment of systemic disease; high doses of oral corticosteroids (1 to 2 mg/kg of prednisone or equivalent) and oral CsA appear to provide systemic relief most rapidly, but corticosteroids are not recommended because of the high doses necessary and the severe exacerbations that often occur after esteroids are stopped. Corneal involvement can be slow to respond and can result in vascularization and scarring.
●Severe cases of uveitis may need systemic corticosteroids and immunosupresants such as CsA, azathioprine, alfainterferon or anti-TNF therapies.
COMPLICATIONS
Retinoids have been reported to cause dry eyes, blepharitis, corneal opacities, cataracts, and decreased night vision.
Conjunctival hyperemia, decreased lacrimation, and cataract have been described with psoralens and UVA, but the relationship is unclear. The use of adequate UV-blocking sunglasses after treatment is recommended.
Methotrexate-treated patients may show reduced full-field electroretinogram and decreased vision.
TREATMENT
Systemic
Systemic treatment is undertaken in coordination with a dermatologist, and in cases of psoriatic arthritis with a rheumatologist.
Local
Topical treatment
●Corticosteroids (most commonly fluorinated preparations such as halcinonide, fluocinonide, betamethasone, and triamcinolone) twice daily.
●Coal tar (2% to 5%) or tar derivate, with subsequent exposure to ultraviolet (UV) B light (Goeckerman regimen).
●Anthralin derivates (0.1% to 0.8%) with salicylic acid in a paste or ointment form and used in combination with tar and UV therapy (Ingram regimen).
● Waterproof adhesive dressings left in place for at least
1 week.
●Phototherapy through presensitization with psoralens and exposure to UVA light (320 to 400 nm).
●Intralesional injections of corticosteroids, such as 5 mg/mL triamcinolone.
●In severe cases, methotrexate 7.5 to 25 mg/week or cyclosporin A (CsA) 2.5 to 5 mg/kg/day.
REFERENCES
Catsarou-Catsari A, Katambus A, Tkheodorpoulos P, et al: Ophthalmological manifestations in patients with psoriasis. Acta Derm Venereol (Stock) 64:557–559, 1984.
Cram DL: Corneal melting in psoriasis. J Am Acad Dermatol 5:617, 1981.
Eustace P, Pierse D: Ocular psoriasis. Br J Ophthalmol 54:810–813, 1970.
McClure SL, Valentine J, Gordon KB: Comparative tolerability of systemic treatments for plaque-type psoriasis. Drug Saf 25:913–927, 2002.
Ponjavic V, Granse L, Stigmar EB, et al: Reduced full-field electroretinogram (ERG) in a patient treated with methotrexate. Acta Ophthalmol Scand 82:96–99, 2004.
Queiro R, Torre JC, Belzunegui J, et al: Clinical features and predictive factors in psoriatic arthritis-related uveitis. Semin Arthritis Rheum 31:264–270, 2002.
See JA, Weller P: Ocular complications of PUVA therapy. Aust J Dermatol 34:1–4, 1993.
Zengin N, Tol H, Balevi S, et al: Tear film and meibomian gland functions in psoriasis. Acta Ophthalmol Scand 74:358–360, 1996.
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88 URTICARIA AND HEREDITARY
ANGIOEDEMA 708.9
(Angioneurotic Edema, Giant Edema,
Giant Urticaria, Hives, Nettle Rash,
Quincke’s Disease)
Mitchell H. Friedlaender, MD
La Jolla, California
ETIOLOGY/INCIDENCE
Urticaria is a cutaneous eruption with multiple pathogenic mechanisms that may be immunologic or nonimmunologic. Its prevalence in the general population is estimated to be between 10% and 25%. No specific cause can be found in 50% of patients with chronic urticaria; in others, psychogenic, allergic and physical factors may play a role. The pathogenesis of urticaria is poorly understood. It is associated with uncontrolled mast cell degranulation and the release of mediators. Hereditary angioedema is an inherited deficiency of C1-esterase inhibitor.
Hereditary angioedema (HAE) is an autosomal dominant disease, and characterized by repeated attacks of epithelial edema involving the skin, respiratory tract, and gastrointestinal tract. Repeated episodes of angioedema involving the skin and respiratory tract may lead to death from pharyngeal edema and asphyxiation.
DIAGNOSIS
Clinical signs and symptoms
Erythematous, elevated, multiple, pruritic skin lesions may be localized or widespread. Hereditary angioedema is characterized by repeated attacks of epithelial edema involving the skin, respiratory tract and gastrointestinal tract. Pharyngeal edema may be life threatening. Abdominal attacks may mimic intraabdominal crises.
TREATMENT
Systemic
●Antihistamines for urticaria or angioedema.
●β-Adrenergic drugs.
●Systemic corticosteroids.
●Cyclosporin A.
●Antifibrinolytic agents for hereditary angioedema (aminocaproic acid or tranexamic acid).
●Anabolic steroids and impeded androgens and fresh frozen plasma.
●C1 esterase inhibitor concentrate (for HAE).
Local
Subcutaneous injection or application of cotton pads soaked in 1 : 1000 epinephrine may be useful to treat severe conjunctival edema.
Surgical
If orbital edema develops to such an extent that the globe or optic nerve is threatened, relief from pressure should be provided. This may involve decompression of the lateral orbital wall. Surgery is rarely necessary.
SUMMARY
Urticaria has numerous causes, and its pathogenesis is poorly understood. Basically, it is associated with uncontrolled mast cell degranulation and release of mediators. Therapy is designed to prevent mast cell release or inhibit the mediators of inflammation.
Hereditary angioedema is an inherited deficiency of C1-este- rase inhibitor and represents a chronic condition that is life threatening. Patients must be counseled intensively and emergency therapy must be available to them.
REFERENCES
Bielory L, Noble KG, Frohman LP: Urticarial vasculitis and visual loss. J Allergy Clin Immunol 88:819–821, 1991.
Bowen T, Cicardi M: Canadian 2003 international consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J Allergy Clin Immunol 114:629–637, 2004.
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