Scleroderma

Question 1

systemic sclerosis

Answer 1

autoimmune disorder -

• fibrosis + microvascular injuries in affected organs
• disorder of small vessels and connective tissue
• thickness and tightness of the skin and subcutaneous tissues

special subform: CREST

C - calcinosis

R - raynaud phenomenon

E - esophageal dysmobility

S - sclerodactyly

T - telangiektasie

epidemology:

prevalence diffus, f:m - 3:1 - 8:1

classification criteria:

1 - skin thickening proximal MCP - 9 points

2 - puffy fingers - 2 points

3 - sclerodactyly - 4 points

4 - digital tip ulcers - 2 points

5 - finger tip pitting scars - 3 points

6 - telangiektasie - 2 points

7 - abnormal nail fold capillaries - 2 points

8 - pulmonary hypertension - 2 points

9 - ACA - anticentrome antibodies - 3 points

more than 9 points is the diagnosis of SS

affected the skin, gastrointestinal tract, kidneys, lungs, hearts and often the hands

overlap syndrome:

Scleroderma patient with associated findings of LE, Dermatomyositis or RA

Question 2

forms of SS

Answer 2

limited or localized skin involvement (not common)

diffuse type: common

finger joint contractures and digital deformities

subcutaneous and intracutanous calcinosis - skin can break down and discharged a white tooth-paste like or chalky material

sclerodactyly

slender fingers with thin, shiny and sclerotic skin

Question 3

Raynaud’s phenomenon

Answer 3

intermittent vasospasm and reduced digital perfusion in 3 phases - fingers become white, than blue and than reddish

leads to skin ulcers and gangrene of the finger tips

Question 4

manifestation

Answer 4

fingers, hand, face

microstomia and microcheilia - (fish mouth)

calcinosis cutis - 30%

Raynaud Phaenomenon:

capillaroscopic abnormalities

• enlarged capillaries
• capillary loss
• capillary haemorrhages

musculoskeletal: 50%, mostly MCP and PIP, acro-osteolysis with resuption of the distal phalanx
pulmonary: limited Sclerosis

pulmonary arterial hypertension

laboratory:

• ACA - anticentromer AB (limited SCL)
• Antitopoisomerase - 1 (SCL-70)
• ANA AB positive
• Anti-polymerase III (diffuse SCL)
• U3 / ribonucleoprotein

Question 5

digital deformity

Answer 5

PIP flexion contracture - loose the ability active to extend the PIP joint - fixed contracture

extensor mechanism thins and ruptures

skin becomes blanches and eventually breaks down and exposing underlying tendons and joints

often compensate through MCP joint hyperextension - MCP flexion initially maintain active - with contract of the colleteral ligaments, joint capsule and overlying skin contract develop limit MCP joint flexion

skin and muscles in first web space contractured - reduced thumb mobility - disability to grasp large objects

ulceration best treated local with silver sulfazadine - cultured mostly S. aureus - maybe minimal bone resection to detense the skin

calcinosis leads to skin break down - debulking is enough for pain relief of patiens - led it heal by second intention because sutures can compromise the skin

contracture PIP:

arthrodesis with bone an skin resection can achieve good healing - perfusion at PIP level is better than at the tip of the finger - in combination with MCP joint release to get a good function of the finger

contracture MCP:

normal circulation - no prosthesis - high risk of infection - in severe cases the metacarpal head can be resected through a palmar approach - MCP flexion contracture a standard dorsal approach is uses

first web space contracture

carefully opened and release of the adductor muscle and maybe skin graft addition - maybe IP or MP joint arthrodesis - in some patients resektion of the trapezium is necesary to restore the metacarpal abduction

vascular insufficiency:

sympathectomy is the best surgey - with stripping the adventitia of the common vessels in the palm

maybe segmental occlusion of the palmar arch - than vene grafts interposition

botolinum toxin A can be injected to the vessels with a good recovery of perfusion

Question 6

therapy

Answer 6

vascular:

• calcium channel blocker
• endothelial inhibitors like “bosentan”
• 5-phosphodiesterase inhibitors like “sildanafil”
• prostanoid analogeous - prostacyclin analogeous

systemic:

• corticosteroids
• methotrexate
• mycophenolate “mofetil”
• cyclophosphamid

atrophy blanche as result from vascular deficiency

typical is the prayer sign in hand holding

surgery:

botox or operative sympathectomy, vene bypass if arteria is occluded or vene bypass for the constricted arteries - surgery should be performed very critical - ulcers of the finger tips normally heal in second condition - no normal wound healing after surgery