What Code Should You Assign for Scleroderma? Well, it Depends…

Becky Buegel, RHIA, CDIP, CHP Medical Coding Leave a Comment

What Code Should You Assign for Scleroderma? Well, it Depends…

Your next chart has the diagnosis “scleroderma.” Being familiar with medical terminology, you know “sclero” means “hard”. Derma, of course, means skin, so the diagnosis means hard/hardened skin. If you’re using coding books instead of an encoder, you’re probably thinking skin codes fall under Chapter 12 – Diseases of the Skin and Subcutaneous Tissue as you flip through the Alphabetic Index to the letters “Scle….” Not so fast, Sherlock! You may be surprised to find not only codes from the aforementioned Chapter 12, but also codes from Chapter 13 – Diseases of the Musculoskeletal System and Connective Tissue. Huh?

There’s more than one type of scleroderma, and while coders always review the patient’s entire chart instead of relying on just the physician’s final diagnosis statement, knowing more about scleroderma will (obviously) lead to more accurate code assignment(s).

There are two main “categories” of scleroderma:

  • Localized
  • Systemic

LOCALIZED SCLERODERMA, which falls under ICD-10-CM category L94 – Other Localized Connective Tissue Disorders, affects only the skin. This autoimmune disease is characterized by morphea – which are discolored, leather-like patches of skin, Morphea can, on occasion, affect underlying fat, muscle, and bone. There are three types of localized scleroderma:

  • Circumscribed Morphea/Scleroderma (aka “localized.”) Patients may have from one to three oval-shaped morphea. The morphea characteristically vary in size and usually have a red border with a yellow center. Morphea that extend into the subcutaneous tissue are labeled “deep circumscribed” morphea.
  • Generalized Morphea is the term used when four or more areas of skin (and other tissue) are involved. Morphea can appear on areas such as arms, head, neck, and/or the torso. “Pansclerotic” morphea is diagnosed if skin, subq fat, and underlying muscle and bone are involved.
  • Linear Scleroderma occurs more often in children 10 and younger. It is characterized by thick, tight bands on the arms, legs, buttocks, anterior and posterior torso, and/or the face. If morphea are on the head, the brain can be affected. If on the limbs, it can cause underdevelopment leading to disability.

SYSTEMIC SCLEROSIS (SCLERODERMA) falls under ICD-10-CM category M34 – Systemic Sclerosis (Scleroderma). Sometimes documented as “SS” or “SSc”, this type of scleroderma affects more than just the patient’s skin. More often than not, it affects what’s under the skin – muscles, blood vessels, joints, GI tract, kidneys, lungs, and heart. While facial involvement is commonplace; while it can be mild, it has been known to affect a patient’s ability to open their mouth. Most patients with SS also have Raynaud’s phenomenon. Like localized scleroderma, there are three types of SSc:

  • Limited Scleroderma, which is the most common type of SSc, is characterized by tight, hard skin, usually restricted to the fingers. However, the hands, forearms and the face can also be involved. Damage to internal organs is less common than other forms of SSc. Patients generally have normal life expectancies. Some do have issues with heartburn, Raynaud’s, and muscle pain. Life-threatening pulmonary hypertension does develop in a limited number of patients.
    • CREST Syndrome is a subtype of limited scleroderma. CREST stands for:
      • Calcinosis
      • Raynaud’s phenomenon
      • Esophageal dysmotility
      • Sclerodactyly
      • Telangiectasias
    • Diffuse Scleroderma is diagnosed when skin is thickened over large areas of the body, and there is significant related organ damage. Lung and or heart damage can be life-threatening. Severe HTN can damage the kidneys.
    • Sine Sclerosis is SSc that includes systemic organ involvement, including Raynaud’s, but curiously does NOT involve skin thickening or other skin-related symptoms.

There’s more to know about both localized and systemic scleroderma and any accompanying complications. The length of this blog doesn’t allow for more information, but additional information can be found by consulting the references noted below.


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