7 CCLE: kronisk kutan LE (=DLE) - etterlater arr ! A 52 year old woman with a long history of photosensitivity and facial skin rashes treated by a dermatologist presented with proteinuria and facial skin lesions in a butterfly distribution as shown in the picture. The differential diagnosis consists of: 1. Acute butterfly skin rash of lupus erythematosus 2. Atrophic skin changes due to local corticosteroid treatment Wrong answer 3. Chronic lupus erythematosus with a butterfly appearance 4. Combination of skin lesion due to chronic lupus erythematosus and local treatment with corticosteroids.CCLE: kronisk kutan LE (=DLE) - etterlater arr !
8 Hypergammaglobulimeniske purpura ved prim Sjøgren (cf Waldenstrøm) A 58 year-old woman with a history of Sjögren’s syndrome (SS) for the last 10 years presented to Rheumatology Clinic with a skin rash involving the lower extremities. Clinical examination revealed bilateral parotid gland enlargement and palpable purpura over the extensor surfaces of the lower extremities. Antinuclear antibodies (ANA) were 1/1280 with a fine speckled pattern, IgM rheumatoid factor (RF) was 1/320 and antibodies to Ro (SSA) and La (SSB) were also positive. The differential diagnosis for the palpable purpura in this patient comprises: 1. Hypergammaglobulinemic purpura 2. Idiopathic thrombocytopenic purpura Wrong answer 3. Waldenström macroglobulinemia 4. T-cell lymphoma Hypergammaglobulimeniske purpura ved prim Sjøgren (cf Waldenstrøm)
9 Fig. 8.7 Tendon. (a) Micrograph of tendon showing the organized bundles of collagen. (b) Tendon insertion showing a population of chondrocytes at the bone–tendon interface and the collagen fibers crossing the interface. (Courtesy of Dr John Clark.)Enthesis
12 Fig The red hot joint. Septic arthritis of the ring finger metacarpophalangeal joint showing swelling and intense redness of the skin.Septisk artritt
13 Staphylococcus aureus septic arthritis of the left hip Staphylococcus aureus septic arthritis of the left hip. The patient presented with left hip pain and fever.The initial film was normal6 weeks later there is periarticular osteoporosis and cortical destruction of the femoral head.
15 A 55 year old woman with a history of “bronchial asthma”, recurrent iritis and articular chondritis presented with a high fever. Clinical examination revealed a dyspnoiec patient with a moon face and a saddle-shaped nose with diffuse wheezing bilaterally over the lungs. Laboratory evaluation showed leukocytosis with polymorphonuclearcytosis, high ESR, high CRP, and negative ANCA. The chest X-ray was normal. The differential diagnosis consists of: 1. Relapsing polychondritis 2. Cogan’s syndrome 3. Wegener's granulomatosis Wrong answer 4. Systemic vasculitis (Churg-Strauss syndrome) Saddle nose : trauma / polykondritt / Wegener’s granuolomatosis/ syfilis/ kokaine misbruk
18 A single 35 year-old woman with a history of Raynaud’s phenomenon since 1990 came to the Rheumatology outpatient clinic for evaluation. Her past medical and family history were unremarkable. Physical examination showed that the hands were cold with a bluish appearance and livedo reticularis was present on the lower extremities (figure). Immunological evaluation showed: positive antinuclear antibodies at low titer (1/80), positive anticardiolipin antibodies, both IgG and IgM (280 IU and 150 IU respectively, normal value <100 IU). The rest of the immunological evaluation was negative. What is the differential diagnosis? 1. Systemic lupus erythematosus 2. Progressive systemic sclerosis Wrong answer 3. Primary antiphospholipid syndrome 4. Atrophic blanche A number of conditions may cause the appearance of livedo reticularis:Cutis marmorata telangiectatica congenita a rare congenital conditionSneddon syndrome - association of Livedoid vasculitis and systemic vascular disorders, such as strokes, due to underlying genetic cause.Idiopathic livedo reticularis - is the commonest cause of livedo reticularis appearance and is a completely benign condition of unknown cause affecting mostly young women during the winter. May be mild, but ulceration may occur later in the summer.Secondary livedo reticularis:Vasculitis autoimmune conditions:Livedoid vasculitis - with painful ulceration occurring in the lower legsPolyarteritis nodosaSystemic lupus erythematosusDermatomyositisRheumatoid arthritisLymphomaPancreatitisTuberculosisAmantadine (drug)Obstruction of capillaries:Cryoglobulinaemia - proteins in the blood that clump together in cold conditions.Antiphospholipid syndrome due to small blood clotsHypercalcaemia (raised blood calcium levels which may be deposited in the capillaries)Haematological disorders of Polycythaemia rubra vera or Thrombocytosis (excessive red cells or platelets)Infections (syphilis and tuberculosis)Arteriosclerosis (cholesterol emboli) and homocystinuria (due to Chromosome 21 autosomal recessive Cystathionine beta synthase deficiency)Intra-arterial injection (especially in drug addictsLivedo reticularis
19 Primaer Raynaud prevalens 5-10% , ANA neg, Norm kapillaroskopi
21 Positiv ANA immunofluoresence technique Fig ANA patterns on mouse liver. (a) Peripheral (rim) pattern; (b) homogeneous (diffuse) pattern; (c) speckled; (d) nucleolar. (Provided by Peter H Schur MD for this publication as well to UpToDate.)Positiv ANA immunofluoresence technique
22 Table 20.1 Sensitivity of the ANA in autoimmune and non-rheumatic disease
28 Fig Acute gout. The first MTP joint is involved at some time in approximately 75% of patients. Desquamation of the skin often occurs.
29 Fig. 26. 7 Annular variety of subacute cutaneous lupus Fig Annular variety of subacute cutaneous lupus. (With permission from Sontheimer et al.27)Subakutt kutan lupus
30 Hydrops/synovia-hyperplasi i kneledd (UL) Fig Ultrasound monitoring of joint effusion. Images of the knee joint obtained in a patient with SLE arthritis demonstrate a large joint effusion (large arrows) and nodular areas of synovial hypertrophy (small arrows). (Courtesy of Dr Carol Benson, Brigham and Women's Hospital, Harvard Medical School.)
31 Rheumatoid arthritis with hyperplastic synovial villi eroding and replacing cartilageat the joint margin
33 Pølsefingre-tåer (dactylitt) ved PsA Fig Sausage fingers and toes. (a) Psoriatic arthropathy – dactylitis at the third finger. (b) Reactive arthritis – dactylitis more evident at the second and third left toes and nail dystrophy.Pølsefingre-tåer (dactylitt) ved PsA
35 Lupus Erythematosus - Malar eminences and nose - Malar erythema and atrophy. Fig Acne rosacea.
36 Kapillæroskopi - scleroderma Fig Nailfold capillary pattern in a patient with scleroderma. Notice the avascular areas. (Courtesy of Dr Hildegard Maricq.)Kapillæroskopi - scleroderma
37 Nailfold capillaroscopy in patients with systemic sclerosis showing changes from normal (top left): irregular vessels, loss of some, dilation of others
38 Fig cANCA pattern. Demonstration of cytoplasmic antineutrophil cytoplasmic antibodies (cANCAs) by indirect immunofluorescence with normal neutrophils. There is heavy staining in the cytoplasm while the multilobular nuclei (clear zones) are non-reactive. These antibodies are usually directed against proteinase 3 and most patients have Wegener's granulomatosis. (Courtesy of Dr Helmut Rennke.)Positive C-ANCA
39 Table 20.4 Significance of cANCA directed against proteinase 3
49 Libman Sacks endokarditt (SLE) Fig Transesophageal echocardiogram in a patient with systemic lupus erythematosus. There is thickening of the anterior (aml) and posterior (pml) mitral leaflets and a Libman–Sacks vegetation (arrow) on the pml. (Courtesy of CA Roldan.)Libman Sacks endokarditt (SLE)