Systemic Sclerosis and Scleroderma in Patients with Darker Skin

00:01 Welcome to our lecture on systemic sclerosis.

00:05 Systemic sclerosis is a chronic multisystem disease characterized by widespread vascular dysfunction and progressive fibrosis of the skin and internal organs.

00:18 Scleroderma is an umbrella term for skin manifestations of disease.

00:23 Skleros, meaning hard or indurated, and derma, meaning skin. The majority of patients are female, with a female to male ratio ranging from 3:1 to 8 :1 .

00:41 Patients of African descent tend to have earlier onset disease and more severe disease phenotype. The exact cause of scleroderma is unknown, but genetic predisposition and environmental triggers like infections have been implicated. Exposure to certain chemicals like silica or solvents, drugs, for example bleomycin, and radiation exposure, are just some of the factors that have been implicated in systemic sclerosis.

01:22 Clinical manifestations of scleroderma.

01:25 It is usually a condition which starts with swelling and puffiness that does not respond to diuretic therapy, and this is usually the edematous stage of the condition.

01:39 After that, the skin tightens, and you get induration.

01:46 The fingers are first affected, and we call this or refer to this as sclerodactyly. And then the lesions extend proximally.

01:59 A beak-like facies and paucity of wrinkles are some of the cutaneous manifestations of systemic sclerosis. Macrostomia has also been documented as a manifestation of systemic sclerosis due to skin tightness and induration.

02:19 Hyper and hypopigmentation of the skin, referred to as salt and pepper lesions.

02:25 Salt being the hypopigmented patches or macules, and pepper being the hyperpigmented, macules or patches.

02:35 Cutaneous calcinosis is also seen, which is deposition of calcium into the skin and subcutaneous tissue.

02:45 The Raynaud's phenomenon is a phenomenon where you get recurrent vasospasm of the fingers and toes. It is part of the initial presentation about 70% of patients.

02:58 95% will develop it at some point of the disease occurrence.

03:05 Other clinical manifestations include arthralgia, arthritis, tendinitis, etc..

03:13 Gastrointestinal involvement may lead to fibrosis and scarring in the esophagus, and this can result in strictures causing dysphagia and difficulty in swallowing.

03:29 Progressive lung fibrosis, resulting in dyspnea and chest pain, may also be seen in patients with scleroderma, and fibrosis of the heart can lead to chest pain, dyspnea, palpitations, fatigue, and other symptoms.

03:45 Fibrosis and inflammation of the glomeruli can result in hypertension and scleroderma renal crisis. So there are four types of systemic sclerosis: has limited cutaneous type, diffuse, systemic, as well as overlap syndromes.

04:09 Looking at limited cutaneous systemic sclerosis, this usually has a long preceding history of Raynaud's phenomenon.

04:18 The slower development of limited cutaneous sclerosis involving distal extremities and face, later onset of organ involvement, that's okay, and more favorable long-term prognosis happens in this condition.

04:35 Diffuse cutaneous systemic sclerosis.

04:37 Here you have sudden onset of Raynaud's phenomenon, rapidly progressive, more widespread cutaneous sclerosis within 12 to 18 months.

04:48 This early onset of organ involvement, particularly including the kidneys.

04:54 This has less favorable long-term prognosis compared to the previous subtype.

05:01 Systemic sclerosis sine scleroderma.

05:05 In this type, there is no skin involvement.

05:08 There's presentation with pulmonary fibrosis, renal, cardiac or gastrointestinal disease.

05:16 So talking about the overlap syndromes.

05:19 Patients with features of other autoimmune rheumatic disease for example SLE, polymyositis can occur with systemic sclerosis or scleroderma.

05:33 Morphea which is a localized type of scleroderma.

05:36 It is characterized by an area of inflammation and fibrosis, which causes thickening and hardening of the skin due to the increased collagen deposition which is produced by fibroblasts.

05:52 The diagnosis of this condition includes physical examination and clinical history.

05:59 Laboratory studies as listed, and the Anti-scl 70 antibodies specific.

06:08 One needs to do also pulmonary function tests, ECG, rarely a skin biopsy may be indicated if the clinical and laboratory tests are not useful. The following table shows the criteria based on the American College of Rheumatology and European League Against Rheumatism.

06:29 A score of nine or more confirms the diagnosis.

06:34 The differential diagnosis includes eosinophilic fasciitis, graft versus host disease, morphea, and linear scleroderma, which is a linear localized form of scleroderma.

06:50 The management involves a multidisciplinary management involvement.

06:57 The gastroenterologist, rheumatologists, and dermatologists.

07:02 The therapeutic approach depends on the presentation of the disease and the complexity of the symptoms.

07:10 Pruritus may be treated by preventing dryness and ensuring that the skin is well lubricated, systemic antihistamines may be used and sometimes low-dose prednisone .

07:24 For skin sclerosis, the following drugs are used to treat the condition.

07:30 Lastly, phototherapy or PUVA may also be used.

07:35 Additional treatment requirements may vary based on the involvement of other accounts and the specific clinical context.

07:44 Kidneys , esophageal reflux, pulmonary hypertension can be addressed using the following drugs that are listed.