Skin manifestations in rheumatic diseases
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Rheumatic diseases are usually associated with cutaneous manifestations.This ppt will present common cutaneous manifestations in rheumatology
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Skin manifestations in rheumatic diseases
- 1. Samar Tharwat Lecturer of Internal Medicine (Rheumatology & Immunology) Mansoura University SkinManifestations in Rheumatic Diseases
- 2. AGENDA ✗ Presentation of Skin Manifestations ✗ Inflammatory Joint Diseases ✗ Connective Tissue Diseases ✗ Vasculitides ✗ Methodology 2
- 3. Presentation of Skin Manifestations Malar erythema Purpura Livedo reticularis Telangectasia Ulceration Panniculitis Puffy fingers
- 4. 4 Rosacea
- 5. 5
- 6. Livedo Reticularis ✗ Mottled, reticulated vascular pattern ✗ Due to alterations in blood flow through the cutaneous microvasculature ✗ AE: physiologic, rheumatic disease ✗ Livedo racemosa 6
- 7. Purpura 7 Macular petechiae (thrombocytopenia) Macular ecchymoses (corticosteroid, amyloidosis) Palpable purpura (leukocytoclastic vasculitis)
- 8. 8 Telangiectasias • Dilatation of the capillaries, arterioles, and/or venules • Erythematous • Blanchable • Anywhere on the skin & mucous membranes • Cosmetic • May be primary as Hereditary hemorrhagic telangiectasia • Or secondary
- 9. Secondary Telangiectasias 9 subacute cutaneous erythematosus Postinflammatory telangiectasia in a patient with dermatomyositis Telangiectatic and avascular patches typical of dermatomyositis Matted telangiectasias of systemic sclerosis Telangiectasias on PIPJ in a patient with systemic sclerosis Typical periungual telangiectasias in this patient with SLE
- 10. Ulceration in Rheumatic Diseases 10 Aphthous ulcer on the lower lip shows a fibrinous base (Behçet’s disease) pyoderma gangrenosum with an undermined ulcer (Behçet’s disease Lupus ulcer Systemic sclerosis ulceration on the digit pulp and the lateral erythema ( Dermatomyositis) Left leg with ulcers in a patient with rheumatoid arthritis Ulcers in vasculitis
- 11. Panniculitis Inflammation of the SC adipose tissue Associations: ✗ infections ✗ oral contraceptives ✗ antibiotics ✗ Sarcoidosis ✗ Behçet’s disease ✗ SLE ✗ Crohn’s disease ✗ primary biliary cirrhosis ✗ idiopathic 11
- 12. Puffy Fingers No clear definition Differential Diagnosis ✗ Systemic Sclerosis ✗ limited cutaneous SSc with ACA ✗ limited cutaneous SSc with Anti-Th/To Antibody ✗ Early SSc with Anti-Topoisomerase I Antibody ✗ Sclerodactyly in dcSSc with Anti-RNA Polymerase Antibody ✗ Mixed connective tissue disease ✗ SSc-Like Disorders ✗ Anti Synthetase Antibody Syndrome ✗ Rheumatoid Arthritis ✗ Overlapping Syndromes 12
- 13. Skin in Rheumatic Diseases • Inflammatory Joint Diseases • Connective Tissue Diseases • Vasculitides
- 14. Nodules in Rheumatoid Arthritis 14 • 1 in 4 patients with long-standing RA will have rheumatoid nodules • Seropositive disease • Non-tender, firm , extensor surfaces • Benign nodules: rheumatoid nodules in the absence of RA Palisaded neutrophilic & granulomatous dermatitisRheumatoid nodules
- 15. Psoriasis and Psoriatic Arthritis 15 Psoriatic plaques Guttate psoriasis Inverse psoriasis Sebopsoriasis Erythrodermic psoriasis Pustular psoriasis Psoriatic nail with onycholysis, pitting and discoloration
- 16. Inflammatory Bowel-Associated Spondyloarthropathy 16 Erythema nodosum Pyoderma gangrenosum Pyostomatitis vegetans Cutaneous polyarteritis nodosa Metastatic Crohn’s Sweet’s syndrome
- 17. Reactive Arthritis 17 Circinate balanitis Erosions on tongue Nail changes include nail dystrophy, subungual debris, and periungual pustules Keratoderma Blennorrhagica
- 19. Skin Manifestations of Juvenile Idiopathic Arthritis 19 Maculopapular rash of sJIA Nail pitting in PsA Dactylitis in PsA The classic lesions of psoriasis
- 20. Skin in Rheumatic Diseases • Inflammatory Joint Diseases • Connective Tissue Diseases • Vasculitides
- 21. Systemic Lupus Erythematosus ✗ Acute ✗ Subacu te ✗ Chronic Lupus non specific 21 Butterfly rash Oral ulcers Photosensitivity Annular type Psoriasiform Discoid LE lupus panniculitis LE tumidus Raynaud’s phenomenon Livedo reticularis Leukocytoclastic vasculitis
- 22. Neonatal Lupus Erythematosus 22 Periorbital and inguinal distribution pattern of skin lesions of neonatal SCLE in a 2- month- old female baby ( left ). The sternal scar results from the implantation of a cardiac pacemaker for atrioventricular block grade III
- 23. Systemic Sclerosis 23 Skin sclerosis Skin sclerosis (and hyperpigmentation) of trunk Expressionless face Diffuse hyperpigmentation hypopigmentation Salt and pepper pattern Furrowing of the mouth Extensive subcutaneous calcinosis
- 24. Localized Scleroderma ✗ Limited type ✗ Generalized type ✗ Linear type 24 Morphea Guttate morphea Generalized localized scleroderma (3 or more anatomic sites) Disabling pansclerotic morphea Linear localized scleroderma En coup de sabre Parry Romberg syndrome
- 25. Dermatomyositis 25 Gottron’s papules Gottron’s papule Heliotrope rash and periorbital edema V sign Periungual telangiectasia
- 26. Sjögren’s Syndrome 26 Xerosis Angular Cheilitis Erythema annulare Vasculitis Urticarial vasculitis
- 27. Skin in Rheumatic Diseases • Inflammatory Joint Diseases • Connective Tissue Diseases • Vasculitides
- 28. Giant Cell Arteritis 28 Scalp necrosis with loss of surrounding hair Ischemic necrosis of tongue Two months after treatment. There has been sloughing of skin of tip of tongue and healing visible temporal artery thickening or nodularityand erythema over the superfi cial temporal arteries
- 29. Kawasaki Disease 29 Macular, morbilliform rash Strawberry tongue Edema of dorsum of both hands Palmar erythema Peeling, exfoliation of hands
- 30. Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) 30 Necrotic purpura Papulonecrotic lesions Livedo reticularis Necrotic lesions Gangrene Oral ulcers Gingivitis Xanthelasmas
- 31. Henoch-Schönlein Purpura 31 Palpable purpura palpable purpura involving the upper and lower Typical palpable purpura on the buttocks Ecchymoses and purpura of the scrotum; the penis is swollen and ecchymotic Bullous evolution of purpuric lesions
- 32. Behçet’s Disease 32 Oral ulcers Genital ulcers Erythema nodosum Superficial thrombophlebitis Pathergy Reaction
- 34. Capillaroscopy 34 Secondary Raynaud’s phenomenon The three scleroderma patters ( early , active , late )
- 35. Skin Biopsy ✗ Site Selection: Anatomical Region, Type of Lesion ✗ Indications: Rheumatoid Arthritis Lupus Erythematosus (LE) Scleroderma Dermatomyositis Psoriasis with Psoriatic Arthritis Vasculitis Panniculitis 35
- 36. 36
- 37. 37
Editor's Notes
- Symmetrical,erythematous,tender,rounded nodules on the extensors of lower extremities Red, purplish, yellow Heal without atrophy
- Many other conditions present with skin fi brosis and may be confused with SSc. SSc-like disorders include immune-mediated diseases (eosinophilic fasciitis, graftversus- host disease), deposition disorders (scleromyxedema, nephrogenic systemic fi brosis, systemic amyloidosis), toxic exposures (toxic oil, l -tryptophan, bleomycin, polyvinyl chloride), and genetic syndromes (progeria, stiff skin syndrome) [ 8 ]. Some of the above patients may seldom complain of puffy fi ngers.
- can occur in other tissues such as the eye, larynx, lung, and nervous system Methotrexate therapy is associated with rheumatoid nodulosis with rapid onset of multiple smaller lesions over the hands Less commonly, smaller papules and plaques, occasionally linear, with smooth, umbilicated, or crusted surfaces occur symmetrically over the joints or extensor surfaces in patients with RA or systemic lupus erythematosus
- Psoriatic arthritis occurs in approximately 5–30 % of patients affected by psoriasis characteristic lesion of psoriasis vulgaris is the psoriatic plaque, which is a welldemarcated, erythematous, round or oval, sharply bordered lesion with a noncoherent, silvery-white scale Guttate psoriasis:‘drop-like’, 0.5–1 cm, well-defined psoriatic papules Inverse psoriasis:intertriginous areas of the body – axilla, groin and neck. Sebopsoriasis:erythematous plaques and greasy, yellow scale in the scalp, nasolabial folds and presternal areas Erythrodermic psoriasis is an acute, generalized form of psoriasis characterized by erythema and superfi cial scale that covers more than 90 % of the total body surface area
- Between 4 % and 11 % of patients with infl amatory bowel disease can develop spondyloarthropathy 36 % of patients with infl ammatory bowel disease, with or without SpA, will develop extra-intestinal manifestations
- This exanthem of KD initially begins as a clear vesicle on an erythematosus base that progresses to macules and/or waxy papules that progress and develop into a hyperkeratotic, scaly lesion that very much resembles pustular psoriasis. Individual lesions coalesce into larger areas of thick, keratotic scales. The lesions may remain small and appear as discrete, erythematosus, hyperkeratotic papules or coalesce into quite large plaques which resemble a more generalized hyperkeratosis. These lesions can be found on the scrotum, toes, plantar aspect of the feet, palms, trunk, and scalp. Coloration is that of yellow to brown
- Erythema marginatum (5 %) is a macular, non-pruritic rash with serpiginous erythematosus spots located on the trunk and rarely on the face, commonly associated with arthritis or carditis
- It begins before the age of 16 years and by defi - nition lasts longer than 6 weeks. Skin manifestation is evident only in systemiconset disease and in psoriatic arthritis The rash is typically a pink, evanescent, salmon-colored macular rash The classic lesions of psoriasis consist of round, well-demarcated, red areas of skin infl ammation with a characteristic grayish or silvery scales
- ACLE is a non-scarring subtype and does usually not lead to depigmentation SCLE is associated with autoantibodies to Ro/SSA and La/SSB Lesions can be triggered by UV light and several drugs, among them terbinafi ne, thiazide diuretics and several calcium channel blockers. lupus panniculitis is characterised by indurated nodules or plaques that will result in deep lipoatrophy Therefore, suffi cient experience and an open, interdisciplinary approach will be essential to optimise care for SLE patients. Even under these circumstances, and despite all due caution, the disease will sometimes not adhere to any textbook, and all unexpected reactions should prompt rethinking and reinvestigation
- The specifi c skin lesions occur in 2–10 % of children after delivery in anti-SSA/Roor anti-SSB/La-positive mothers. They are a consequence of the transplacental transfer of the antibodies. The lesions are similar to those seen in adult subacute cutaneous lupus erythematosus – scaly erythematous infl ammatory lesions with a predominant annular or plaque-type appearance transient, disappearing within weeks Periorbital and inguinal distribution pattern of skin lesions of neonatal SCLE in a 2-month- old female baby ( left ). Single effl orescences appear as annular erythematous and scaly plaques ( right ). The sternal scar results from the implantation of a cardiac pacemaker for atrioventricular block grade III
- GCA rarely presents with cutaneous manifestations because it spares the small vessels which are typically associated with skin lesions [ 1 ]. The most common visible “skin involvement” in GCA is related to the infl ammation of the cranial arteries, especially the temporal arteries he temporal arteries are often tender, while their pulses may be decreased or absent vasculitis mimicker (e.g. amyloidosis)
- KD is the leading cause of acquired heart disease in children in developed countries. Coronary aneurysms (CAA) develop in about 25 % of untreated children but also in 4 % of those who received IVIG
- Dermatologic lesions of granulomatosis with polyangiitis (Wegener’s granulomatosis; GPA) are frequently encountered and occasionally may be the initial manifestations (8–13 %) Purpura may evolve to necrosis, leading to vesicles, blisters, erosions, ulcerations, and ulcers Xanthelasmas are yellow cholesterol plaques on the eyelids, predominantly on the nasal side. They do not have any distinctive histological features. When they appear suddenly in a GPA patient, they are usually adjacent to a granulomatous infi ltration into the underlying orbit
- Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in childhood of small-sized blood vessels, resulting from an IgA-mediated infl ammation palpable purpura, the typical skin lesions, involve the lower extremities and buttocks and rarely the upper extremities, face and trunk
- assessment of the microcirculation
- diagnostic and prognostic marker Not all skin lesions require biopsy the variability of the cutaneous clinical manifestations of rheumatologic diseases, a properly performed skin biopsy can be a powerful diagnostic tool an untreated, well-formed skin lesion in an early stage should be chosen for biopsy