Morphea/Scleroderma



Morphea/Scleroderma


Sudeep Gaudi, MD

Drazen M. Jukic, MD, PhD










A lesion of morphea en plaque is characteristically located on the trunk under an area of pressure (bra line). Note the violaceous peripheral rim (“lilac ring”) and white, sclerotic center.






Pictured are lesions of generalized morphea. This patient presented with indurated plaques image measuring at least 3 cm in greatest dimension over his trunk and upper and lower extremities.


TERMINOLOGY


Synonyms



  • Morphea: Localized scleroderma


Definitions



  • Most common subtype of scleroderma (group of fibrosing disorders that result from imbalance between collagen production and destruction)



    • Clinically heterogeneous disease process that is localized to the skin and subcutaneous tissues with possible involvement of underlying muscle and bone



      • Underlying central nervous system may also be affected in cases of face and head involvement


ETIOLOGY/PATHOGENESIS


Multifactorial



  • Precise etiology remains mystery


  • Evidence suggests microvascular injury induces profibrotic cytokines following environmental insult in genetically predisposed individual



    • These cytokines lead to increased collagen production and decreased collagen destruction


  • Genetic



    • Possible host factors include autoimmunity and microchimerism


  • Environmental



    • Possible insults include radiation, trauma, medications, and infection (potential link to certain Borrelia species, particularly in Europe)


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 0.4-2.7 per 100,000 people


  • Age



    • Prevalence is equal in adults and children



      • Approximately 90% of afflicted children present between 2 and 14 years of age


      • Mean age of presenting adults is mid-40s


  • Gender



    • About 2-4x more common in females than males


  • Ethnicity



    • Affects all races, but more common in whites



      • Approximately 70-80% of patients examined in ethnically heterogeneous clinical populations are white


Presentation



  • Circumscribed (a.k.a. morphea en plaque)



    • Most common subtype in adults


    • Patients typically present with less than 3 plaques, predominantly located on trunk in areas of pressure (e.g., around waist, under bra line)


    • Lesions tend to soften over period of 3-5 years


    • Superficial variant: More common; limited to epidermis and dermis


    • Deep variant (morphea profunda): Affects dermis and subcutaneous tissue, with variable involvement of underlying fascia and muscle; overlying epidermis may not be involved


  • Linear

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Morphea/Scleroderma

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