Scleroderma is a disease characterized by fibrosis. An alteration occurs in the fibroblasts, whose cause is unknown. The most important affectation of the skin consists of the alteration of the number of fibroblasts protein producers, especially collagen. It is more frequent in women than in men, with a ratio of 3:1.
Is Scleroderma a Disability?
Patients with scleroderma disease can suffer significant changes in their physical appearance, as well as disability due to injuries to their internal organs with serious impairment of their health and also a functional limitation in their daily activity. It is worth mentioning that the disability generated by digital ulcers can be transient or permanent. It is better to consult a disability lawyer if you think that your scleroderma is affecting your daily life activities and quality of life.
Raynaud’s Phenomenon in Scleroderma
The Raynaud phenomenon is a transient, reversible vasospastic phenomenon, induced by cold or stress. It occurs in the fingers, toes and, less frequently, in the nose, ears, and nipples. It can be asymmetrical and not affect all the fingers.
Typically, skin color changes are 3: initial pallor, cyanosis and finally red fingers as an expression of the compensatory vasodilation phase. Analyzing the clinical manifestations of progressive systemic sclerosis, in both diffuse and limited variants, Raynaud is present in most patients.
Calcinosis in Scleroderma
Patients with the limited scleroderma variant develop calcinosis more frequently. These deposits of calcium are located in soft parts, without causing direct joint involvement; they can be small or extensive and are usually complicated by ulceration of the skin and over-aggregated infection. Patients with extensive calcinosis present significant deterioration of their functional capacity due to contractures inflection of the proximal joints. When the calcinosis bursts the skin, ulcerated lesions develop with the possibility of bacterial superinfection.
Digital Ulcers in Scleroderma
Despite the different criteria, digital ulcer in patients with scleroderma will be defined simply as a loss of continuity solution in the epidermis and adjacent layers and digital location.
On the contrary, the healing of the ulcer implies complete re-epithelialization of the ulcer, independently of residual pain.
Regarding digital ulcers, it should be considered certain characteristics of it: size, borders, bed, tissue exposure (bone, tendons) and presence of underlying calcinosis. An active ulcer is considered to be one whose evolution is less than 3 months, and chronic, if it is greater than 6 months.
Digital ulcers in patients with scleroderma are seen in the hands and feet. So-called non-digital ulcers have also been described. Non-digital ulcers in patients with scleroderma are located in the pretibial region of the legs, ankles, elbows, and forefoot. In legs, they are usually extensive and the differential diagnosis of vasculitis should be carried out.
The mechanism of development of digital ulcers in scleroderma is due to multiple factors including repeated microtrauma, thinning of the skin, dry skin and underlying calcinosis. It is estimated that 8 to 12% of ulcers have underlying calcinosis. However, prolonged digital ischemia as an expression of the unresolved Raynaud’s phenomenon is the most important risk factor.
The clinical course of ulcers will depend on the factors listed above. Around 30% of patients with scleroderma and digital ulcers have soft tissue and bone loss. When analyzing the complications of patients with ulcers during 7 years of follow-up, digital gangrene is observed in 11% of them, but if the treatment fails to reverse the situation of ischemia, practically 100% of patients with digital ulcers you will suffer from gangrene after this period of follow-up. 12% of patients with ulcers require hospitalization and surgery.
From the analysis of the different databases of patients with scleroderma, 58% will develop at least one digital ulcer at some point of the disease. In 32% of cases, they will become chronic and persistent ulcers. 25% of patients with scleroderma are presented to the rheumatological consultation with more than two digital ulcers. Ulcers are observed both in patients with the diffuse cutaneous variant and in patients with the limited cutaneous variant of scleroderma.
The impact on the quality of life and disability of patients with digital scleroderma ulcers can be objectified through the HAQ (Health Assessment Questionnaire) if the involved limb and the loss of joint function directly compromised by the ulcer.
- Mayo Clinic – Scleroderma: https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952
- American College of Rheumatology – Scleroderma: https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma
- National Organization for Rare Disorders (NORD) – Scleroderma: https://rarediseases.org/rare-diseases/scleroderma/
- Arthritis Foundation – Scleroderma: https://www.arthritis.org/diseases/scleroderma
- Cleveland Clinic – Scleroderma: https://my.clevelandclinic.org/health/diseases/17483-scleroderma
- Johns Hopkins Scleroderma Center – About Scleroderma: https://www.hopkinsscleroderma.org/scleroderma/
- Medscape – Scleroderma: https://emedicine.medscape.com/article/331864-overview
- Scleroderma Foundation – What is Scleroderma: https://www.scleroderma.org/site/SPageServer/?pagename=patients_whatis
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) – Handout on Health: Scleroderma: https://www.niams.nih.gov/health-topics/scleroderma
- NHS – Scleroderma: https://www.nhs.uk/conditions/scleroderma/