1. Scleroderma Cutaneous features Iraj Salehi-Abari MD., Internist
Rheumatologist

2. Epidemiology: Annual incidence in USA: 1-2 / 100,000
Salehi I.
Epidemiology:
Annual incidence in USA: 1-2 / 100,000
Peak onset age: y/o
100 RA, 10 SLE, 1 Scl
F/M ratio: 3/1
Black > other race
Rare in children
Rare in men < 30 y/o
Scleroderma

3. General manifestations:
Salehi I.
General manifestations:
Most frequent:
Fatigue, Stiff joints
Loss of strength, Pain
Sleep difficulties
Skin discoloration
Less frequent:
Breathlessness, depression, sore eyes
Upset stomach, nausea, weight loss
Scleroderma

4. Skin involvement: Universal feature of SSc Characterized by:
Salehi I.
Skin involvement:
Universal feature of SSc Characterized by:
Skin thickening, hardness, and fixation
The fingers, hands and face are at first
Prominent skin manifestation:
. Pruritus and edema in the early stages
. Sclerodactyly
. Digital ulcers & pitting of fingertips
. Telangiectasia
. Calcinosis cutis
Scleroderma

5. Skin involvement: Diffuse tanning in the absence of sun exposure
Salehi I.
Skin involvement:
Diffuse tanning in the absence of sun exposure
Vitiligo-like hypopigmentation
“Salt-and-pepper” appearance:
In scalp, upper back, chest
Pigment loss spares the perifollicular areas
Dry skin, hair loss:
Collagen accumulation causes oblitration of hair follicles, sweat & sebaceous glands
Scleroderma

6. The assessment of skin involvement:
Salehi I.
The assessment of skin involvement:
Estimation of:
Skin thickness
Pliability (hardness)
Fixation to underling structures (tethering)
The modified Rodnan skin score:
In 17 distinct areas of the body
Score from 0 (nl) to 3 (most severe)
Gold standard is skin palpation by Rheumatologist
Ultrasonography & Durometer ?
Scleroderma

7. Salehi I.
Scleroderma

8. Face: “Mauskopf” face Microstomia (fish mouth)
Salehi I.
Face:
“Mauskopf” face
Shiny taut skin
Masklike & expressionless
Decreased skin folds
Microstomia (fish mouth)
Perioral radial furrowing
“Beak-like” nose
Small sharp nose:
Omega sign
Matlike telangiectases
Scleroderma

10. Hand involvement: Sclerodactyly : Tapered fingers
Salehi I.
Hand involvement:
Sclerodactyly :
Thickening of finger skin
Fibrosis of tendons
Tapered fingers
Transverse creases on the dorsum of the fingers disappear
Scleroderma

11. Hand involvement: Digital ulcerations “Digital pits” = Pitting ulcers
Salehi I.
Hand involvement:
Digital ulcerations
Superinfection
Osteomyelitis
“Digital pits” = Pitting ulcers
Healing of ischemic fingertip ulcerations
Scleroderma

12. Hand involvement: Dissolution of terminal phalanges
Salehi I.
Hand involvement:
Dissolution of terminal phalanges
Loss of volar pads of the fingertips 
Resorption of the terminal phalangs:
Acro-osteolysis
Periungual telangiectasia
Scleroderma

13. Hand involvement:

16. Calcinosis cutis: Most common in patients with lcSSc & positive ACA
Salehi I.
Skin involvement:
Calcinosis cutis:
Most common in patients with lcSSc & positive ACA
CPPD deposition
Visualized on plain X-Rays
Typical locations: finger pads, palms, extensor surfaces of the forearms, olecranon & prepatellar bursae
Persistent firm, nontender subcutaneous lumps
If ulcerate  drainage of chalky white material
Scleroderma

17. Neck sign

18. Barnett’s sign: Vertical striations in the skin with neck extension
Salehi I.
Barnett’s sign: Vertical striations in the skin with neck extension
Scleroderma