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    Skin Signs of Rheumatic Disease - DermNet

    Uploaded by

    Anonymous 9lmlWQoDm8

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    © All Rights Reserved
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    Skin signs of rheumatic disease Author: Dr Jenny Chung, House Officer, Auckland City Hospital, New Zealand. DermNet NZ Editor in Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell/Maria McGivern, November 20 What is a rheumatic disease? ‘Many autoimmune connective tissue diseases and vascular conditions in rheumatology have cutaneous manifestations, “+ Skin signs are useful in the diagnosis of rheumatic disease, as they often precede systemic symptoms. ‘+ They can also act as a prognostic marker and predict systemic involvement. Rheumatic diseases described on this page are: ‘© Cutaneous lupus erythematosus Dermatomyositis systemic sclerosis Scleroedema Scleromyxoedema Rheumatoid arthritis systeric-onset juvenile arthritis Relapsing polyehondrits Psorlatic arthritis Osteoarthritis = vascullts, Cutaneous lupus erythematosus Alzout 25% of patients with systemic lupus eryshermatosus (SLE) intally present with skin involvement. Iti important to correctly classify {cutaneous lupus erythematosus (CLE), as It helps determine the underlying type and severity of SLE. About 5~10% of patients with CLE develop SLE, and CLE Is associated with less severe forms of SLE ‘Skin manifestations of lupus erythematosus are commonly divided into lupus erythematosus-speciic and non-specific disease, Note that four ofthe rine American College of Rheumatology criteria for SiF are skin signs (ie, malar/outterfly rash, discoid plaques, photosensitivity, and oral ulcers). Lupus erythematosus-specific disease Acute cutaneous lupus erythematosus Forms of acute CLE include the following: ‘+ Localised acute CLE — this presents with malar or butterfly’ rash (symmetrical erythema and oedema ofthe cheeks, forehead, chin, and V ofthe neck but sparing the nasolabial folds or'smile lines) 1+ Generalised acute CLE — this presents with a widespread exanthematous eruption on the extensor surfaces, trunk, sun-exposed areas, ard hands but sparing the knuckles) + Toxic epidermal necrolysissike acute CLE — this sa fe-hreatening variant of acute CLE that presents with a massive epidermal Injury, eccurs predominantly on sun-exposed skin and has a gradual, insidious onset, unlike toxic epidermal necrolysis. ‘Acute CLE typically triggered or exacerbated by exposure to ultraviolet (UV) radiation. On recovery, there may be postin ammatory hyperpigmentation without scarring. Malar rash of systemic lupus erythematosus Inips:idermnetnz orgopics/skin-signs-of moumatie-isease ana Subacute cutaneous lupus erythematosus Subacute cutaneous lupus erythematosus (SCLE) starts as macules or papules that progress to hyperkeratotic plaques. SCLE is photosensitive so plaques usually occur on sun-exposed skin; these plaques do not lead to scarring but can result in postinvlarmatory hyperpigmentation or hypopgmentation. SCLE should be monitored to exclude any progression co SLE. Forms of SCLE include: ‘© Annular SCLE— this subtype presents with slightly cased red lesions with central clearing + Papulosquamous SCLE — this subtype presents with eczematous or psoriass-like lesions on sun-exposed skin Subacute cutaneous lupus erythematosus Chronic cutaneous lupus erythematosus CChronic CLE is not as photosensitive as acute CLE or SCLE. Forms of chronic CLE include: ‘+ iscoid lupus erythematosus (DLE)— this affects the face, outer ears, neck, sun-exposed areas and lips, and presents with discoid plaques (erythematous, well-demarcated plaques covered by scale) that become hyperkeratotic, leading to atrophy and scarring: there is folicuar involvement, causing both reversible and ireversible (scarring) alopecia (hair loss}; dyspygmentaton of the per pheries is also comman in certain ethnicities (Asian, Indian) ‘+ Hypertrophic or verrucous lupus erythematosus — this is arare form of CLE presenting with severe hyperkeratosis ofthe extensor surfaces ofthe arms, upper back and face; It has overlapping features with lichen plans, ‘+ Mucosal lupus erythematosus — this affects 25% of patients with CLE; most commonly, painless erythematous patches on the oral mucosa develop into chronic plaques that can centrally uleerate ard also affect nasal, conjunctival and genital mucosa; oral lupus erythematasus rarely degrades to oral cancer (squamous cel carcinoma, Discoid lupus erythematosus Inips:idermnetnz orgopics/skin-signs-of moumatie-isease ang Drug-induced lupus erythematosus ‘Many drugs are thought to induce SLE and dvus-incuced lupus erythematosus often includes cutaneous signs. Drugs that induce lupus cerytrematosus include: © Hydralazine Isoniazid + Chlorpromazine + procainamide + Phenytoin © Minacyeline ‘© Anti-tumour necrosis factor medications. Drug-induced cutaneous lupus Drug-induced subacute lupus Drug-induced cutaneous lupus Druguinduced cutaneous lupus erythematosus erythematosus erythematosus Rarer types of lupus erythematosus ‘The rarer types of lupus erythematosus include: ‘+ Lupus profundus/luous panniculitis — this is arare form of chronic CLE with frm nodules In the lower dermis and subcutaneous, tissue that causes ipodystrophy; some use the term lupus panniculitis to refer to subcutaneous involvement only, and lupus profundus wnen there is a combination of lupus pannicults with DLE, ‘+ Chillin lupus erythematosus — this presents with purple-red patches, papules and plaques on toes, fingers and face, andis, associated with nail fold telangiectasia; itis precipitated by exposure to tre cold, so often presents in winter. “+ Lupus erythematosus tumidus —this sa variant of chronic CLE with succulent or indurated erythematous plaques without surface change, Rare forms of chronic cutaneous lupus erythematosus Lupus profundus CChilblain pus Lupus tumidus Inips:idermnetnz-orgopies/skin-signs-of eumati ise ang Lupus erythematosus — non-specific disease Lupus erythematosus-nonspecific disease can relate to SLE or another autoimmune disease, but nonspeciic cutaneous features are most often associated with SLE. Common cutaneaus features seen include + Photosensitivty — this an abnormal response to UV radiation that is present in 50-93% of patients with SLE + Mouth ulcers — these are present in 25-459 of patients with SLE + Non-scarring hairloss in SLE — presenting as coarse, dry hair with increased fragility (also referred to as lupus hair) Cutaneous vascular disease is also common. Forms of cutaneous vascular disease include: + Raynaud phenomenon — this presents with focal ulceration in the fingertips and periurgual areas that can cause pitted scarring, haemorrhage and other nail fold complications + Vascultis — leukocytoclastic vascul ts: urticarial vasculltis presenting with tender papules and plaques over bony prominences; and ‘megium or large vessel vascultis can occur, presenting with purpuric plaques with stellate borders, often with necrosis and Ulceration or subcutaneous nodules + Thromboembolic vasculopathies — clots + Lived reticularis — characterised by netike blanching rec-purple rings that commonly arise on the lower limbs thromelalgia — characterised by burning pain in the feet and hands, and with macular erythema; ts associated with heat 1ese may have a similar clinical presentatlon to vascullts, but vessel occlusion s due to blood Vascular disease in lupus erythematosus Raynaud phenomenon Livedo reticularis (other symptoms include: + Cutaneous mucinosis — characterised by induratee papules, nodules, or plaques on the trunk or arms + Lupus nail dystrophy — presenting as ral pitting, ridging, leukonychia, onycholsis, and ed lunula + Spontaneous chronic urticaria Lichen planus + acanthosis nigricans + Sclerodactyly (spindle-shaped fingers) thema multiforme * Cutis axa + Rheumatoid nodules. Dermatomyositis Dermatomyositis (alse known as idiopathic inflammatory myopathies) is a heterogeneous group af autoimmune disorders affecting the skin and musculature. ‘Skin signs are critical in diagnosing and classifying dermatomyositis: + Amyopathic dermatomyositis — this involves skin changes without muscle or other systemic involvement for more than 6 months. + Classic dermatomyositis — this is more common and results in skin and muscle disease nipesidermnetne.orgopleslekin-signs-ot eumatie-isease ang Two hallmark skin signs of dermatomyositis help differentiate it from CLE; these are: “© Gottron papules — these are violaceous macules over the knuckles and in periungual areas; they often have a depressed centre that Is white or atrophic ‘+ Gottron sign — this refers to symmetrical violaceous macules, sometimes with oedema, over the knuckles, elbows, or knees. Classic skin signs of dermatomyositis include + Periorbital macules — these are violaceous, erythematous (hellotrope’) macules ofthe eyelids and periorbital tissue, presenting with fr without oedema + Periungual telangiectasia ‘+ Macules over the hands and fingers — these present with symmetric erythema on the dorsum af hands and fingers, the extensor surface of arms, deltoid, posterior shoulders, Varea of the neck, central forehead, face and scalp ‘+ Mechanic's hand (a sign of antisynthetase syndrome) — this is characterised symmetrical chronic eczerna-tke scaling affecting the hands, especially along the ulnar side of the thumb and radial side ofthe fingers. ‘other skin signs of dermatomyosits include: ‘+ Polkloderma — this isa combination of violaceous erythema, dysplgmentation, telangiectasia and atrophy ofthe skin; most commonly aver the shoulders, back, buttocks, V of neck and chest ‘+ Caleinosis cutis — presenting as painful irregular nodules at sites of friction and trauma, such as the elbows, knees and fingers. Dermatomyositis Gottron papules Macular violaceous rash Calcinosis cutis Systemic sclerosis ‘system'c sclerosis is a multisystem form of scleroderma with hallmark ski signs. itis associated with a high morsality and morbidity rate, The clinical features of systemic sclerosis are diverse and affect multiple organs in the body. ‘Two important skin signs of systemic sclerosis are Raynaud phenomenon and skin sclerosis; these signs help classily the disease into the following subsets. ‘+ Localised systemic sclerosis — features of which involve pre-existing chronic Raynaud phenomenen for a period of years and skin sclerosis (scleroderma) affecting the face, neck, and extremities, while sparing proximal body sites 1+ CREST syndrome — this involves: * Calcinosis + Raynaud phenomenon + (o}esophageal symotoms + Sclerodactyly + Telangiectasia + Visceral cisease — this may begin after a long period of Raynaud disease and can involve pulmonary arterial hypertension, pulmonary fibrosis, ard gastraintestinal tract involvement + Diffuse systemic sclerosis — features of which include Raynaud phenomenon of recent onset and proximal scleroderma affecting Upper arms, thighs, chest and abdomen; there is an acute onset with inflammatory features in the first 24 months of disease; pulmonary fibrosis, caralac disease ane renal crisis are also common “© Overlapping systemic sclerasis — these patients have features of scleroderma with another autolmmune theumat disease, such as dermatomyosttis or polymyositis, SLE, §)6gren cisease, vasculitis, or polyarterit's nips idermnetnz orgopics/skin-signs-of moumatie- 6 months, hypocomplementaemia, and leukocytoclastic vasculitis on blopsy + Hendch-Schonlein purpura — this i an immune-complex mediates form of small vessel vasculitis that normally occurs in children, usually after a respiratory tract infection, and presents with purpura (leukocytoclastc vascultis) and urticaria with local oedema; it Can also cause intestinal vasculis, intussusception (where one portion of an intestine protrudes into another), glomeruionephitis (inflammation of the kidneys), and joint symptoms (which are more common in adults than in children) hema elevatur diutinum — this fsa chronic leukocytoclastc vascults that causes symmetrical red, violaceous, and yellow brown papules, nodules, or plaques commonly on the extensor surfaces ofthe hands, knees, legs, and Achilles tendon + Malignant atrophic papulosis (Degas disease) — this sa type of small vessel vasculapathy thac presents with papules with a central white depression; the papules heal with a white scar nipesidermnetne.orgopleslekin-signs-ot meumatie-isease wns Rare forms of small vessel vasculitis Urticarial vasculitis Henoch Schonlein purpura Erythema elevatum diutinum, Medium vessel vasculitis Forms of medium vessel vascullts and their associated skin signs include: ‘+ Polyartertis nodosa — this s a rare form of vascults involving small and medium-sized arteries ofthe dermis and subcutaneous tissue; skin signs (seen in 1586 of cases) include red, blue, or vilet subcutaneous nodules that become confluent and paintul, vedo reticulars, leg ulcers, and postinflammatory hyperpigmentation ‘+ ‘Thromboangits obliterans (Buerger disease) — thisis 2 form of vasculopathy due to thrombosis of small or medium vessels that is. associated with smoking and is often asymmetrical and associated with cold climate or exercise: its skin signs are painful purple or blue fingers or toes (see blue toe syndrome) that may ulcerate or become gangrenous leading to amputation ofa limb. ‘+ Kawasaki disease — this is an acute multisystem vascultls with fever,and systemic and cardiac manifestations; it is the most comnen ‘cause of heart disease in children; its skin signs are polymorphous exanther (90% of which are found on the trunk and proximal limbs), perineal eruption (macular or plaque-type erythema that desquamates within 48 hours}; oedema and erythema of palms and Soles; oral signs (including ‘cherry’ Ips, dry and cracked ‘strawberry tongue with hypertrophied papillae, anc alated blood vessels), and periungual desquamation, Medium-sized vessel vascul Ulcer due to palyarteritis nodosa Livedo reticularis in polyartertis Berger disease nodosa Large vessel vasculitis Forms of large vessel vasculitis and thelr associated skin signs include: ‘+ Takayasu arteritis — this is a rare, systemic inflammatory large-vessel vascults usually Involving the aorta and its branches and arteries going to the brain limbs, heart, ana kidneys that affects women aged *0-30 years; there are no associated stn signs. ‘+ Giant cel arteritis (temporal arteritis) —this is an inflammatory condition mainly involving the blood vessels ofthe head and affects those $5 years of age or greater its skin signs are cyanosis and pallor of exiremities, scalp tenderness and sometimes, necrosss. ‘+ Cogan syndrome — ths is characterised by inflammation, predominantly ofthe aorta and its branches, and causes hearing loss, vertigo and eye symptoms; itnas no associated skin signs. Behcet disease Inips:idermnetnz orgopics/skin-signs-of moumatie-isease vans Behet disease isa mukisystem inflammatory disease affecting the skin, eyes joints, and other systems. ls skin signs include: ‘© Recurrent oral aphthous ulcers> In 80¥ of cases< Genital ulcers in 64-88% of cases rythema nodosurn pustules supe I thrombophlebitis, nips idermnetnz orgopics/skin-signs-of moumatie-

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