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CUTANEOUS MANIFESTATIONS OF
RHEUMATOID ARTHRITIS
SUJITHA TAMILSELVAM
CRRI
PONDICHERRY INSTITUTE OF MEDICAL SCIENCES
RHEUMATOID ARTHRITIS
 Rheumatoid Arthritis (RA) is a immune-mediated,
chronic disease affecting selected joints
 Characterized by persistent inflammatory synovitis
leading to cartilage damage, bone erosions, joint
deformity and disability.
 In Extra -articular localizations, skin being the most
commonly affected organ, with polymorph clinical
manifestations
OVERVIEW
 Age: Any age, commonly 3rd to 6th decade
 Female: male 3:1
 pattern of joint involvement could be:-
1) Polyarticular : most common
2) Oligoarticular
3) Monoarticular
 Morning joint stiffness > 1 hour and easing with
physical activity is characteristic.
 Small joints of hand and feet are typically involved.
2010 ACR/EULAR CRITERIA
CLINICAL FEATURES
ARTICULAR
EXTRA – ARTICULAR
ARTICULAR MANIFESTATIONS
JOINT INVOLVEMENT PERCENTAGE
MCP, PIP 91
WRISTS 78
KNEES 64
SHOULDERS 65
ANKLES 50
FEET 43
ELBOWS 38
HIPS 17
TMJ 8
SPINE 4
PERI ARTICULAR SITES 27
EXTRA – ARTICULAR MANIFESTATIONS
EXTRA – ARTICULAR MANIFESTATIONS
EXTRAARTICULAR
MANIFESTATION
INCIDENCE
Pericarditis 2.6
Pleuritis 1.9
Major cutaneous vascultis 0.6
Vasculitis – related neuropathy 0.2
Felty syndrome 0.5
Scleritis 0
Episcleritis 0.7
Secondary sjogren syndrome 9.6
Pulmonary fibrosis 5.0
Bronchiolitis obliterans 0.7
Subcutaneous rheumatoid nodules 31.2
CUTANEOUS MANIFESTATIONS
SKIN MANIFESTATIONS OF RHEUMATOID ARTHRITIS
GENERAL
•Erythema , burning sensation (may precede joint changes)
•Nodules
•Atrophy
•Vitiligo (non – segmental )
•Possible association with pseudoxanthoma elasticum
VACULITIS
•Splinter haemmorhages and periungual infarcts
•Palpable purpura
•Livedo reticularis
•Atrophie blanche
NEUTROPHIL RELATED DISORDERS
•Pyoderma gangrenosum
•Rheumatoid neutrophilic dermatoses
MISCELLANEOUS
•Vasculitis , venous insufficiency, lymphoedema, inactivity, skin atrophy
•Interstitial granulomatous dermatitis
•Intralymphatic histiocytosis
CUTANEOUS MANIFESTATIONS
 SPECIFIC
 NON SPECIFIC
ATROPHIC SKIN WITH RA
 >60 Years
 F > M
 Skin – dorsa of hand – thin, loose, inelastic
transparent – tendons and veins clearly seen
 Histologically dermis is thinned
CLASSICAL RHEUMATOID NODULES
 Subcutaneous lesions, skin-like colored, with a
diameter generally ranging from >5mm to several
centimeters.
 The extensor surfaces of the forearms, the back of
the hands, the occipital regions, the auricular
regions and the nasal pyramid
Other sites
 Larynx
 Heart , lungs
 Brain – leptomeninges > parenchyma
 Vertebral body – signs of myelopathy
RHEUMATOID NODULES – PATHOGENESIS
 Resident histiocytes and fibroblast proliferation to
an influx macrophages
 cytokine mediated – TNF, IL- 1 , IL- 12 , IL – 17
 Th1-mediated pathogenetic hypothesis
HISTOLOGY:
 Palisaded granulomas – deep dermis or
subcutaneous tissues
 Focal central fibrinoid necrosis – surrounding
fibroblasts
HISTOLOGY PIC
DIFFERENTIAL DIAGNOSIS – RHEUMATOID
NODULES
 Subcutaneous granuloma annulare
 Foreign body granuloma
 Infectious granuloma
 Sarcoid granuloma
 Myxoid cyst
 Traumatic epidermal cyst
 Xanthoma
 Epitheloid sarcoma
 Fibromatous nodules in lyme borreliosis
RHEUMATOID NODULOSIS
 Multiple nodules in the hand
 Positive RF
 Acute intermittent synovitis
 Subchondral cystic lesions of the small bones of
hands and feet
RHEUMATOID VASCULITIS
 Seen in 0.1 to 5.4 %
Can present as
 Distal arteritis - Splinter haemorrhages, periungaul
infarcts , gangrene
 Cutaneous ulceration – pyoderma gangrenosum
 Peripheral neuropathy – mononeuritis multiplex /
sensory stocking glove neuropathy
 Palpable purpura
 Arteritis of the viscera
RHEUMATOID VASCULITIS
 Livedo reticularis
High titres – RF & anti-CCP
Pathology :
 Panarteritis – all layers of vessel infiltrated with
mononuclear cells
 Fibrinoid necrosis
 Thrombosis , obliterative endarteritis
 Immune complex deposits
 Neurovascular disease manifest as
Mild distal sensory neuropathy
Severe sensorimotor neuropathy (mononeuritis
multiplex)
C/F –
Burning feet
Weakness ex: footdrop
Visceral lesions – claudication/ infarction
Gangrene of digits , extremities
Small vessel vasculitis involving
lower legs
LIVEDOID VASCULOPATHY
Periungual
granuloma
pyogenicum
NEUTROPHILIC DERMATOSIS
 Neutrophilic dermatoses are a wide group of
diseases characterized by neutrophil accumulation
in the skin and in extracutaneous sites.
 Include disorders like pyoderma gangrenosum and
Sweet syndrome – can co-exist in one patient
 Usually associated with Blood cell dyscracias , IBD,
Rheumatoid arthritis , systemic lupus
erythematosus
RHEUMATOID NEUTROPHILIC
DERMATOSIS
C/F
 Urticaria like papules and plaques – symmetrical on
the trunk and limbs
 Overlap with sweet syndrome
 Tense bullae can occur in lower limbs
 Histology :
 Heavy dermal infiltrates of neutrophils, no frank
vasculitis
PYODERMA GANGRENOSUM
 A type of neutrophilic dermatosis
 Pyoderma gangrenosum typically - sterile pustules
which rapidly evolve into painful ulcers,
 Wide and deep, characterized by well-defined and
raised edges of erythematous-purple coloring
 Overlap between – pyoderma gangrenosum and
sweets syndrome
 Between pyoderma gangrenosum and rheumatoid
neutrophilic vasculitis
SWEET’S SYNDROME
 “acute febrile neutrophilic dermatosis”
 onset of fever, neutrophil leukocytosis, and painful
plaque erythematous skin lesions
 Including bullous, pustular, subcutaneous and giant
cellulitis-like forms
 Histology :
 Dense neutrophilic infiltrate in the superficial
dermis, with considerable edema; another possible
variant is the “histioid” form, while the presence of
eosinophils in the infiltrate suggests a drug-induced
form
INTERSTITIAL GRANULAMATOUS
DERMATITIS
 Defined as interstitial granulomatous dermatitis
with arthritis
 Papules and erythematous plaques, erythematous-
purple lesions, in 10% of the cases with a typical
“cord-like” appearance,
 With symmetric distribution; the most affected sites
being the axillary area, the trunk and the medial
surface of thighs
 Interstitial histiocytic granulomas without vasculitis
and mucin deposit
DEFORMITIES IN RHEUMATOID ARTHRITIS
Patient with rheumatoid arthritis presenting with a radial
deviation
(right wrist), swan-neck deformity with hyperextension of the
proximal
interphalangeal (PIP) and fl exion of distal interphalangeal
(DIP) joints
Patient with rheumatoid arthritis presenting with volar
subluxationof the right hand with a visible sliding at the
radiocarpal joint and Z-deformity
(thumb) with flexion of the first metacarpophalangeal joint and
hyperextension of the interphalangeal joint.
Typical forefoot deformities in a patient with
rheumatoid arthritis with
clawing of the toes and dislocation of the
metatarsophalangeal (MTP) joints.
RADIOGRAPHIC FEATURES
 Peri-articular osteopenia
 Uniform symmetric joint space narrowing
 Marginal subchondral erosions
 Joint Subluxations
 Joint destruction
 Collapse
 Ultrasound detects early soft tissue lesions.
 MRI has greatest sensitivity to detect synovitis and
marrow changes
 Thank you

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Cutaneous manifestations of rheumatoid arthritis

  • 1. CUTANEOUS MANIFESTATIONS OF RHEUMATOID ARTHRITIS SUJITHA TAMILSELVAM CRRI PONDICHERRY INSTITUTE OF MEDICAL SCIENCES
  • 2. RHEUMATOID ARTHRITIS  Rheumatoid Arthritis (RA) is a immune-mediated, chronic disease affecting selected joints  Characterized by persistent inflammatory synovitis leading to cartilage damage, bone erosions, joint deformity and disability.  In Extra -articular localizations, skin being the most commonly affected organ, with polymorph clinical manifestations
  • 3. OVERVIEW  Age: Any age, commonly 3rd to 6th decade  Female: male 3:1  pattern of joint involvement could be:- 1) Polyarticular : most common 2) Oligoarticular 3) Monoarticular  Morning joint stiffness > 1 hour and easing with physical activity is characteristic.  Small joints of hand and feet are typically involved.
  • 6. ARTICULAR MANIFESTATIONS JOINT INVOLVEMENT PERCENTAGE MCP, PIP 91 WRISTS 78 KNEES 64 SHOULDERS 65 ANKLES 50 FEET 43 ELBOWS 38 HIPS 17 TMJ 8 SPINE 4 PERI ARTICULAR SITES 27
  • 7. EXTRA – ARTICULAR MANIFESTATIONS
  • 8. EXTRA – ARTICULAR MANIFESTATIONS EXTRAARTICULAR MANIFESTATION INCIDENCE Pericarditis 2.6 Pleuritis 1.9 Major cutaneous vascultis 0.6 Vasculitis – related neuropathy 0.2 Felty syndrome 0.5 Scleritis 0 Episcleritis 0.7 Secondary sjogren syndrome 9.6 Pulmonary fibrosis 5.0 Bronchiolitis obliterans 0.7 Subcutaneous rheumatoid nodules 31.2
  • 9. CUTANEOUS MANIFESTATIONS SKIN MANIFESTATIONS OF RHEUMATOID ARTHRITIS GENERAL •Erythema , burning sensation (may precede joint changes) •Nodules •Atrophy •Vitiligo (non – segmental ) •Possible association with pseudoxanthoma elasticum VACULITIS •Splinter haemmorhages and periungual infarcts •Palpable purpura •Livedo reticularis •Atrophie blanche NEUTROPHIL RELATED DISORDERS •Pyoderma gangrenosum •Rheumatoid neutrophilic dermatoses MISCELLANEOUS •Vasculitis , venous insufficiency, lymphoedema, inactivity, skin atrophy •Interstitial granulomatous dermatitis •Intralymphatic histiocytosis
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  • 13. ATROPHIC SKIN WITH RA  >60 Years  F > M  Skin – dorsa of hand – thin, loose, inelastic transparent – tendons and veins clearly seen  Histologically dermis is thinned
  • 14.
  • 15. CLASSICAL RHEUMATOID NODULES  Subcutaneous lesions, skin-like colored, with a diameter generally ranging from >5mm to several centimeters.  The extensor surfaces of the forearms, the back of the hands, the occipital regions, the auricular regions and the nasal pyramid Other sites  Larynx  Heart , lungs  Brain – leptomeninges > parenchyma  Vertebral body – signs of myelopathy
  • 16. RHEUMATOID NODULES – PATHOGENESIS  Resident histiocytes and fibroblast proliferation to an influx macrophages  cytokine mediated – TNF, IL- 1 , IL- 12 , IL – 17  Th1-mediated pathogenetic hypothesis HISTOLOGY:  Palisaded granulomas – deep dermis or subcutaneous tissues  Focal central fibrinoid necrosis – surrounding fibroblasts
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  • 21. DIFFERENTIAL DIAGNOSIS – RHEUMATOID NODULES  Subcutaneous granuloma annulare  Foreign body granuloma  Infectious granuloma  Sarcoid granuloma  Myxoid cyst  Traumatic epidermal cyst  Xanthoma  Epitheloid sarcoma  Fibromatous nodules in lyme borreliosis
  • 22. RHEUMATOID NODULOSIS  Multiple nodules in the hand  Positive RF  Acute intermittent synovitis  Subchondral cystic lesions of the small bones of hands and feet
  • 23. RHEUMATOID VASCULITIS  Seen in 0.1 to 5.4 % Can present as  Distal arteritis - Splinter haemorrhages, periungaul infarcts , gangrene  Cutaneous ulceration – pyoderma gangrenosum  Peripheral neuropathy – mononeuritis multiplex / sensory stocking glove neuropathy  Palpable purpura  Arteritis of the viscera
  • 24.
  • 25. RHEUMATOID VASCULITIS  Livedo reticularis High titres – RF & anti-CCP Pathology :  Panarteritis – all layers of vessel infiltrated with mononuclear cells  Fibrinoid necrosis  Thrombosis , obliterative endarteritis  Immune complex deposits
  • 26.  Neurovascular disease manifest as Mild distal sensory neuropathy Severe sensorimotor neuropathy (mononeuritis multiplex) C/F – Burning feet Weakness ex: footdrop Visceral lesions – claudication/ infarction Gangrene of digits , extremities
  • 27. Small vessel vasculitis involving lower legs
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  • 31.
  • 32. NEUTROPHILIC DERMATOSIS  Neutrophilic dermatoses are a wide group of diseases characterized by neutrophil accumulation in the skin and in extracutaneous sites.  Include disorders like pyoderma gangrenosum and Sweet syndrome – can co-exist in one patient  Usually associated with Blood cell dyscracias , IBD, Rheumatoid arthritis , systemic lupus erythematosus
  • 33.
  • 34. RHEUMATOID NEUTROPHILIC DERMATOSIS C/F  Urticaria like papules and plaques – symmetrical on the trunk and limbs  Overlap with sweet syndrome  Tense bullae can occur in lower limbs  Histology :  Heavy dermal infiltrates of neutrophils, no frank vasculitis
  • 35.
  • 36. PYODERMA GANGRENOSUM  A type of neutrophilic dermatosis  Pyoderma gangrenosum typically - sterile pustules which rapidly evolve into painful ulcers,  Wide and deep, characterized by well-defined and raised edges of erythematous-purple coloring
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  • 38.
  • 39.  Overlap between – pyoderma gangrenosum and sweets syndrome  Between pyoderma gangrenosum and rheumatoid neutrophilic vasculitis
  • 40. SWEET’S SYNDROME  “acute febrile neutrophilic dermatosis”  onset of fever, neutrophil leukocytosis, and painful plaque erythematous skin lesions  Including bullous, pustular, subcutaneous and giant cellulitis-like forms  Histology :  Dense neutrophilic infiltrate in the superficial dermis, with considerable edema; another possible variant is the “histioid” form, while the presence of eosinophils in the infiltrate suggests a drug-induced form
  • 41. INTERSTITIAL GRANULAMATOUS DERMATITIS  Defined as interstitial granulomatous dermatitis with arthritis  Papules and erythematous plaques, erythematous- purple lesions, in 10% of the cases with a typical “cord-like” appearance,  With symmetric distribution; the most affected sites being the axillary area, the trunk and the medial surface of thighs  Interstitial histiocytic granulomas without vasculitis and mucin deposit
  • 42. DEFORMITIES IN RHEUMATOID ARTHRITIS Patient with rheumatoid arthritis presenting with a radial deviation (right wrist), swan-neck deformity with hyperextension of the proximal interphalangeal (PIP) and fl exion of distal interphalangeal (DIP) joints
  • 43. Patient with rheumatoid arthritis presenting with volar subluxationof the right hand with a visible sliding at the radiocarpal joint and Z-deformity (thumb) with flexion of the first metacarpophalangeal joint and hyperextension of the interphalangeal joint.
  • 44. Typical forefoot deformities in a patient with rheumatoid arthritis with clawing of the toes and dislocation of the metatarsophalangeal (MTP) joints.
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  • 47. RADIOGRAPHIC FEATURES  Peri-articular osteopenia  Uniform symmetric joint space narrowing  Marginal subchondral erosions  Joint Subluxations  Joint destruction  Collapse  Ultrasound detects early soft tissue lesions.  MRI has greatest sensitivity to detect synovitis and marrow changes
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  • 49.