Scleroderma Cutaneous features Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir

Epidemiology: Annual incidence in USA: 1-2 / 100,000 Salehi I. Epidemiology: Annual incidence in USA: 1-2 / 100,000 Peak onset age: 30-50 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

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

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

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

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

Salehi I. Scleroderma

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

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

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

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

Hand involvement:

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

Neck sign

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