Clinical Case, Connective Tissue Disease, Scleroderma, Teledermatology



  1. Agnetta, Vlatka
  2. Jacob, Sharon E.


ABSTRACT: Teledermatology is a term to describe the provision of dermatologic medical services through telecommunication technology. This is a teledermatology case of ulcerations on the hands and fingers.


Article Content

In the store-and-forward teledermatology modality, there is a transfer of patient medical information electronically (including history and visual data) obtained in one location to a provider who is in another location (Roman & Jacob, 2015). The construct of the TeleDerm-ViewPoint column is such that cases are presented in a standardized teledermatology reader format reflective of an actual teledermatology report.




Reason for consultation: presenting for diagnosis and management advice for a patient with ulcerations on the hands and fingers.


History of Present Illness

A 32-year-old woman presents for progressive tightening of her skin on her hands over the last year. The patient states that her skin has "hardened" over time and is tearing. Prior treatment: none. Her primary symptom: tight skin that is tender to the touch. Prior biopsy: none.



Fully satisfactory.



One image was provided noted as Problem A that shows round ulcerations over the dorsal surface of multiple proximal interphalangeal joints on bilateral hands as wells as the dorsal and ulnar surfaces of the right wrist. The fingers are notably tapered distally, and the skin appears tight and shiny with increased pigmentation and loss of normal wrinkling over the finger joints (see Figure 1).

Figure 1 - Click to enlarge in new windowFIGURE 1. Ulcerations over the hands and fingers.



Problem A

The history, morphology, and distribution of the lesions are most consistent with sclerodactyly. The ischemic ulcers are notably seen in systemic sclerosis.



Initial therapy includes patient education. Patients should be educated on the vasospastic and ischemic nature of the disease. The education should also focus on triggers and exacerbating factors like exposure to cold and smoking. Behavioral changes should be emphasized such as keeping the distal extremities warm. If the patient is a smoker, he or she should be advised to abstain from tobacco.



The patient should be referred to dermatology for confirmatory physical diagnosis and biopsy and triage care in the evaluation for systemic sclerosis.



Scleroderma is an autoimmune disease that primarily affects women. Skin disease manifestations include skin hardening and thickening, giving it a smooth appearance and impaired mobility.


Progressive systemic sclerosis (PSS) and CREST syndrome are both types of scleroderma. CREST syndrome describes a limited disease with the following features: calcinosis cutis (calcifications in the skin), Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. It generally carries a better prognosis (Yoon, 2014). A highly specific serologic marker for CREST syndrome is the presence of the autoantibodies to the centromere proteins, scleroderma-70 (topoisomerase I) and fibrillarin, which is positive in 50%-90% of patients with CREST syndrome (James, Berger, & Elston, 2011).


PSS is a widespread, generalized disease involving connective tissues affecting the skin, vasculature, and internal organs. Raynaud phenomenon is the first manifestation of the disease in over 50% of the cases. Classic criteria include at least two of the following: sclerodactyly, pitting scars or loss of fat padding on the fingertips, and bilateral basilar pulmonary fibrosis. Proximal sclerosis is the alternative criteria of diagnosing PSS. In addition, PSS can have cardiac, renal, and joint manifestations. Pulmonary disease is the most common cause of death.


Pulmonary fibrosis results in pulmonary hypertension and leads to right-sided heart failure. Other cardiac complications include pericarditis as well as myocardial sclerosis, which can lead to conduction abnormalities and arrhythmias. Renal disease can vary significantly and includes glomerulonephritis and scleroderma renal crisis. Joint swelling and pain indicate polyarthritis, which may be the first symptom of systemic sclerosis. Hand joints are the most commonly involved joints; cardiac involvement, scleroderma-70, and antinuclear antibody (ANA) are associated with poorer prognosis. Treatment is available and effective in many visceral complications of scleroderma. However, the treatment for skin manifestations is limited (James et al., 2011).


Smoking is absolutely contraindicated in this disease because it increases ischemia to the distal extremities and promotes development of peripheral vascular disease. Physical therapy is also an important therapeutic measure because it maintains range or motion and mobility. A variety of vasodilators, like calcium channel blockers and topical nitroglycerine paste, have been widely used in Raynaud phenomenon with significant efficacy. Possible side effects of vasodilators include hypotension. Calcium blockers can also exacerbate gastroesophageal reflux disease (Bolognia, Jorizzo, & Schaffer, 2012). L-arginine, botulism toxin, and gingko biloba have been reported to have some clinical efficacy in local ischemia (James et al., 2011). Systemic immunosuppressive therapies such as cyclophosphamide, methotrexate, corticosteroids, and phototherapy are utilized to aggressively treat this debilitating disease. Prompt multidisciplinary referral, including dermatology and physical therapy, is of utmost importance.




Bolognia J., Jorizzo J., Schaffer J. (2012). Dermatology. Philadelphia, PA: Elsevier Saunders. [Context Link]


James W., Berger T., Elston D.(2011). Andrews' diseases of the skin: Clinical dermatology. Philadelphia, PA: Elsevier Saunders. [Context Link]


Roman M., Jacob S. (2015). Teledermatology: Virtual access to quality dermatology care and beyond. Journal Dermatology Nurses Association, 6(6), 285-287. [Context Link]


Yoon J. (2014). CREST syndrome. Retrieved from[Context Link]


1The standardized teledermatology reader report format is available for authors on the submissions Web site and outlined in Table 1. [Context Link]