5th June 2025

Diagnosis

LcSSc and dcSSc both show the same signs and symptoms, but the skin distribution and course of the disease is different. Two thirds of patients with scleroderma have dcSSc, which has widespread skin involvement including proximal limbs. This is often rapidly progressive, has significant internal organ involvement and has a worse prognosis. One third of patients with scleroderma have lcSSc, where the sclerosis is limited to the digits, face and distal limbs (does not spread further than elbows or knees). It has a slower progression and less organ involvement, but carries a risk of pulmonary artery hypertension. Up to 20% of patients have an overlap syndrome with another connective tissue disease.

Systemic features

Gastrointestinal: dysmotility, gastroesophageal reflux (GORD), dysphagia, bloating, nausea and vomiting, alternating diarrhoea/constipation
Cardiopulmonary: interstitial lung disease (ILD), pulmonary artery hypertension, cardiomyopathy
Renal dysfunction: patients may present with abrupt onset of moderate/marked hypertension, which is a sign of scleroderma renal crisis
Musculoskeletal: friction rubs over joints and tendons, synovitis, weakness and limited movement causing contractures
Ophthalmological: tight eyelids, reduced tear secretion, retinopathy
Fatigue, arthralgia, myalgia
Sicca symptoms and Sjogren syndrome

Cutaneous features

Skin sclerosis, commonly sclerodactyly (hardening of the skin causing the fingers to curl inward) or microstomia Image Source: Wikimedia Commons (2015). Crest 1, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:CREST1.JPG (Accessed 19 May 2025).
Puffy hands, often one of the first symptoms
Raynaud’s phenomenon Image Source: Wikimedia Commons (2014). Raynaud Phenomenon, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/w/index.php?search=Raynaud%E2%80%99s+phenomenon&title=Special:MediaSearch&go=Go&type=image (Accessed 19 May 2025).
Digital pits and/or ulceration
Abnormal nail fold capillaries
Telangiectases – often located on the face, hands and mucous membranes
Subcutaneous calcinosis – small, hard masses on pressure points (eg. Fingers, forearms) Image Source: Wikimedia Commons (2018). Calcinosis Cutis, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:Calcinosis_cutis_new_image.jpg (Accessed 19 May 2025).
Image Source: Wikimedia Commons (2018). Calcinosis of Crest Syndrome, Licensed under a creative commons licence. Available for free at: https://commons.wikimedia.org/wiki/File:Calcinosis_of_CREST_syndrome.jpg (Accessed 19 May 2025).
Salt and pepper dyspigmentation – hyperpigmentation with vitiligo-like depigmentation