Diagnostic Dilemma: Linear Scleroderma

Department Editor: Tania Phillips, MD, FRCPC Overall Learning Objective: The physician or podiatrist participant will develop a rational approach to the evaluation and treatment of a variety of uncommon wounds and will have an increased awareness of the differential diagnosis of cutaneous wounds and the systemic diseases associated with these wounds. Submissions: To submit a case for consideration in Diagnostic Dilemmas, e-mail or write to: Executive Editor, WOUNDS, 83 General Warren Blvd., Suite 100, Malvern, PA 19355, eklumpp@hmpcommunications.com Completion Time: The estimated time to completion for this activity is 1 hour. Target Audience: This CME/CPME activity is intended for dermatologists, surgeons, podiatrists, internists, and other physicians who treat wounds. At the conclusion of this activity, the participant should be able to: 1. Differentiate linear scleroderma from morphea 2. Recognize the histologic findings of scleroderma 3. Identify common laboratory abnormalities associated with linear scleroderma 4. Discuss the common treatment modalities for linear scleroderma. Disclosure: All faculty participating in Continuing Medical Education programs sponsored by HMP Communications are expected to disclose to the program audience any real or apparent conflict(s) of interest related to the content of their presentation. Drs. Hall, Stefanato, and Phillips disclose no financial conflicts. Accreditation: HMP Communications is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. HMP Communications is approved by the Council on Podiatric Medical Education as a sponsor of continuing education in podiatric medicine. Designation: HMP Communications designates this continuing medical education activity for 1 credit hour in Category 1 of the Physician’s Recognition Award of the American Medical Association. Each physician should claim only those hours he/she spent in the educational activity. HMP Communications designates this continuing medical activity for .1 CEUs available to participating podiatrists. Method of Participation: Read the article, take, submit, and pass post-test by January 15, 2005. This activity has been planned and produced in accordance with the ACCME Essential Areas and Policies. Release date: January 15, 2004 Expiration date: January 15, 2005 Presentation A 57-year-old African-American woman presented with a three-year-history of arthritis and changes in the quality of her skin. After an injury to the left leg in October, 2000, she developed rapidly progressive induration and pigmentary alteration of the skin of the left leg associated with limited movement of the knee and ankle. Over the past year, the skin on the medial aspect of the foot had ulcerated, and she was concerned about the large amount of drainage from the wound. She also complained of joint stiffness and pain, muscle cramps, and Raynaud’s phenomenon. She denied dysphagia or gastrointestinal symptoms. Her family history was significant for the presence of rheumatoid arthritis and systemic lupus erythematosus. Physical Examination Examination revealed mottled hyperpigmentation, induration, limb shortening, and muscle wasting of the left leg with extension to the left lower abdomen and back. On the medial aspect of the foot, there were three ulcers with granulating bases and serosanguinous drainage. Their size ranged from 1cm to 5cm. (Figures 1 and 2). There was depigmentation of the distal digits with ragged cuticles. The face was normal with no perioral puckering. Investigataions Three punch biopsies were submitted for histopathology examination. Biopsy sites were an ulcer border, sclerotic skin from the left anterior leg, and uninvolved skin from the left posterior leg. The ulcer border revealed epidermal hyperplasia, granulation tissue, and a mixed inflammatory infiltrate of lymphocytes, neutrophils, and eosinophils. The affected sclerotic skin from the anterior left leg revealed dermal sclerosis with appendageal atrophy and subadjacent septal panniculitis extending deep to the tissue margins and a mild superficial and deep perivascular lymphocytic infiltrate with plasma cells (Figure 3). The changes extended into the subcutaneous fat. The clinically unaffected skin from the posterior leg revealed a scant dermal perivascular lymphocytic infiltrate. Laboratory analysis included Scl-70Answers (Refer to questions on other side of page) Circle one letter for each answer: 1. A B C D 2. A B C D 3. A B C D 4. A B C D 5. A B C D Evaluation (circle one) Excellent (4) Good (3) Satisfactory (2) Poor (1) Accuracy and timeliness of content: 4 3 2 1 Relevance to your daily practice: 4 3 2 1 Impact on your professional effectiveness: 4 3 2 1 Relevance of the content to the learning objectives: 4 3 2 1 Effectiveness of the teaching/learning methods: 4 3 2 1 This activity avoided commercial bias or influence YES NO Now that you have read this article, can you: 1. Differentiate linear scleroderma from morphea? YES NO 2. Recognize the histologic findings of scleroderma? YES NO 3. Identify common laboratory abnormalities associated with linear scleroderma? YES NO 4. Discuss the common treatment modalities for linear scleroderma? YES NO What questions do you still have?_______________________________________________ How will you use what you have learned from this activity?____________________________ All tests must be received by 1/15/05.