Scleroderma (Systemic Sclerosis, SSc)

Scleroderma (Systemic Sclerosis, SSc) is a chronic autoimmune connective tissue disease characterized by vascular dysfunction, immune activation, and progressive fibrosis of the skin and internal organs, most commonly affecting females with a 3:1 ratio between ages 30 and 50. Pathogenesis begins with endothelial injury and abnormal angiogenesis leading to increased vascular endothelial growth factor production and vascular leakage, followed by autoantibody formation including anticentromere antibodies, anti topoisomerase 1 antibodies, and anti RNA polymerase 3 antibodies. These processes drive autoantigen mediated T cell activation and cytokine release, particularly TGF beta, resulting in fibroblast activation and collagen deposition with progressive fibrosis. Diagnosis is based on weighted clinical criteria requiring a score of at least 9 and includes proximal finger skin thickening, digital edema progressing to induration and atrophy, non pitting sclerosis, sclerodactyly, digital ulcers and ischemic pitting scars, telangiectasias, abnormal nailfold capillaries, pulmonary arterial hypertension, interstitial lung disease, Raynaud’s phenomenon, and autoantibody positivity. Limited systemic sclerosis involves distal extremities and face with a better prognosis and includes the CREST variant, while diffuse systemic sclerosis involves proximal and distal extremities and trunk with severe visceral involvement and worse prognosis. Additional cutaneous features include microstomia, beaked nose, salt and pepper dyspigmentation, and pterygium inversum unguis.

Epidemiology

Female dragon slayer = Female predominance with 3:1 ratio
Ages 30 to 50 = Typical age of onset

Pathogenesis

Leaky vascular hose = Vascular dysfunction and abnormal angiogenesis
Bucket of VEGF veggies = Increased VEGF production
Leaking hose = Vascular leakage
Centromere shaped dragonfly = Anticentromere antibodies
Top hat = Anti SCL 70 antitopoisomerase 1 antibodies
Polymerase printer = Anti RNA polymerase 3 antibodies
Tree growth factor beta fertilizer = TGF beta cytokine production
Fibrotic tree = Fibrosis development

Clinical Diagnostic Criteria

Dragon’s thick claws = Skin thickening of fingers extending proximal to MCP joints
Wet hands = Edematous phase with pitting edema
Tight shiny dragon claws = Indurated phase
Stone claw statue = Non pitting skin thickening
Contracted claws = Atrophic phase with flexion contractures
Scaly finger skin = Sclerodactyly distal to MCP joints but proximal to PIP joints

Digital Ulcers and Pitting Scars

Ulcer in ground = Digital tip ulcers due to trauma
Blue pit = Fingertip pitting scars due to ischemia

Telangiectasias

Red mat = Matted telangiectasias of face lips and palms
Squared mat edges = Smooth squared off telangiectasia borders
Loop and dropout towel = Dilated capillary loops with dropout

Lung Findings

High tension vascular belt = Pulmonary arterial hypertension
Fibrotic lung tree = Interstitial lung disease

Raynaud’s Phenomenon

Blue gloves = Raynaud’s phenomenon and secondary Raynaud’s

Autoantibody Positivity

Centromere shaped dragonfly = Anticentromere antibodies
Top hat = Anti SCL 70 antibodies
Polymerase printer = Anti RNA polymerase 3 antibodies

Subtypes

Limited Systemic Sclerosis

Dragon mask and claws = Distal extremity and facial involvement
Thumbs up = Better prognosis without severe renal or pulmonary disease

CREST Syndrome

CREST shield = CREST syndrome
Glass of calcium milk = Calcinosis cutis
Blue gloves = Raynaud’s phenomenon
Esophagus shaped snake = Esophageal dysmotility
Dragon claws = Sclerodactyly
Telangiectasia mat = Telangiectasias

Diffuse Systemic Sclerosis

Sad dragon slayer in full body suit = Diffuse systemic sclerosis with worse prognosis

Additional Cutaneous Findings

Beaked bird with small mouth = Beaked nose and microstomia
Salt and pepper dragon = Salt and pepper dyspigmentation
Downward bending claws = Pterygium inversum unguis