Abstract: A 57-year-old African-American woman presented with a three-year-history of arthritis and changes in the quality of her skin. 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. Given the history of Raynaud’s syndrome and the presenting physical findings, the clinical diagnosis of linear scleroderma was felt to be most appropriate. In this article, the presentation, significant physical findings, etiology, and treatment plans for the patient are discussed.

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).

Figure 1

Shown here are the bilateral lower extremities of a 57-year-old African American woman. Notice the hemiatrophy of the left extremity.

Figure 2

Close-up view of the ulcerated lesions of the left foot pictured here.

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-70<1.00, anticentromere antibody negative, C-reactive protein 1.3, erythrocyte sedimentation rate (ESR) 36, anti-nuclear antibody (ANA) 1:40 with homogenous pattern, anti-Ro negative, anti-La negative, complete blood count (including eosinophil count) within normal limits, and cryoglobulins less than one percent. Rheumatoid factor was positive at 96 (normal 0–40).

Figure 3

The histopathology demonstrates dermal sclerosis with appendageal atrophy and subadjacent pannicular fibrosis with a deep dermal perivascular lymphocytic infiltrate with plasma cells.

Diagnosis

The histopathological diagnosis was consistent with morphea profunda. Given the history of Raynaud’s syndrome and the presenting physical findings, the clinical diagnosis of linear scleroderma was felt to be most appropriate.

Discussion

Scleroderma can be divided into systemic and localized forms, each of which can be further subdivided based on the extent of disease involvement.[1] Systemic involvement is subdivided into limited systemic sclerosis, defined by skin involvement limited to the face and distal extremities, and diffuse systemic sclerosis, characterized by the involvement of truncal skin or proximal extremities.[1] Both diffuse and limited systemic sclerosis display findings of systemic involvement (Raynaud’s phenomenon or organ involvement). Localized scleroderma, which is generally categorized by the presence of skin involvement only, is subdivided into several morphological variants, which include morphea and linear scleroderma.[2,3] Linear scleroderma (LS) generally presents in the first two decades of life and the lesions are arranged in a band-like distribution, most commonly affecting the lower extremities in a unilateral fashion. Other areas of involvement include the upper extremities, the face (in such location it is designated as coup de sabre), and the anterior thorax.[2] LS has two phases: acute erythematous and chronic sclerosis.[4] In the latter, the dermis is thickened by increased collagen bundles. Extension of sclerosis into the deeper layers of the skin and the underlying structures differentiates LS from morphea, and it may lead to severe orthopedic deformities, such as hemiatrophy or contractures.[2] Internal organ involvement is not a feature of localized scleroderma, and only rarely is Raynaud’s phenomenon present.[2]

LS has been associated with anomalies of the vertebral column, such as spina bifida occulta.[2] In coup de sabre, ocular, oral and neurologic abnormalities may occur.[1] Case reports have suggested a link between physical trauma and the development of LS, especially in the extremities.[5,6] Dystrophic calcinosis, with subsequent ulceration and calcium deposition in muscle, has also been reported.[7–9]

Laboratory analysis in LS often reveals the presence of antinuclear antibodies.[1,3,10] Less commonly eosinophilia, anti-single–stranded DNA antibodies, antihistone antibodies, and increased immunoglobulins (IgG) are present.[10,11] Rarely, rheumatoid factor and anti-centromere antibodies are detected.[2,12] ESR is usually normal.[3]

The mainstay treatment for LS is phototherapy, usually as psoralen plus ultraviolet A (PUVA) or UVA1.[3] PUVA is effective in treating not only the erythematous phase of LS but also the chronic sclerosis. LS may require significantly more phototherapy treatments than is required for morphea.[3] Oral corticosteroids or methotrexate are helpful in the acute inflammatory stages of LS or morphea, but do not improve the sclerosis. Several months of treatment with etretinate or acitretin also have proven beneficial in localized scleroderma.[3] Randomized, control trials have not supported the use of oral calcitriol, a vitamin D derivative. New treatment options, such as extracorporeal photophoresis and photodynamic therapy, lack evidence to support superiority over PUVA. Physiotherapy is critical in patients with impaired mobility.[3]

Patient Management

This patient was presented at the New England Dermatological Society meeting held December 6, 2003, in Boston, Massachusetts, for further treatment suggestions. The patient has commenced treatment with PUVA and physiotherapy with significant skin softening. Alginate dressings have been helpful for wound management.

How to obtain credits by reading this article:

Print off the answer and evaluation form at the end of this article. Fill it out completely, answer at least 70 percent of the questions correctly, and mail or fax completed tests and evaluations to:

Trish Levy, CME Director
HMP Communications
83 General Warren Blvd., Suite 100
Malvern, PA 19355
Fax (610) 560-0501

Questions

1. Which of the following is not a form of localized scleroderma?
A. Morphea
B. Generalized morphea
C. Limited systemic sclerosis
D. Linear scleroderma

2. What is the differentiating factor between morphea and linear scleroderma/morphea profunda?
A. Extension of the lesion into deeper layers of skin and underlying structures
B. Presence of ANA in serum
C. Systemic involvement
D. Presence of Raynaud’s phenomenon

3. Laboratory abnormalities associated with linear scleroderma may include which of the following?
A. Eosinophilia
B. ANA positive titer
C. Elevated immunoglobulins
D. All of the above

4. Which of the following is not a histologic feature of scleroderma?
A. Caseating granulomas
B. Excessive collagen deposition in the dermis and subcutaneous tissue
C. Entrapment of adnexal structures
D. Lymphoplamacytic infiltrate

5. What is the mainstay treatment for linear scleroderma?
A. Oral corticosteroids
B. Phototherapy
C. Oral calcitriol
D. Methotrexate

Linear Scleroderma Answer and Evaluation Form (Print this off)

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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.