Morphea is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • A benign inflammatory skin disorder leading to excessive collagen deposition with thickening and sclerosis of the skin and SC tissues and characteristic plaque formation
    • Lesions are well-circumscribed, flat, firm to touch, and have a waxy feel. They may be more easily felt than seen. They are ivory-colored and have a lilac halo border.
    • Lesions are not associated with generalized scleroderma/systemic sclerosis based on lack of sclerodactyly, Raynaud phenomenon, and nail fold capillary changes.
  • Three phases: active/edematous, inactive/sclerotic/fibrotic, and atrophic lesions
  • Morphea is classified into five types (Mayo Classification):
    • Plaque: most common subtype especially in adults, well-circumscribed, and confined to dermis
    • Generalized: 4+ plaques involving 2+ body sites
    • Bullous: rare
    • Linear: most common subtype in children
    • Deep: more severe and involving all layers from skin to bone, causing limb contractures and muscle atrophy
  • Synonym(s): localized scleroderma (LS); scleroderma circumscripta

Epidemiology

Incidence
  • Predominant sex: female > male (2.4 to 5:1)
  • Peak incidence of plaque subtype in 3rd to 4th decade
  • 50% of morphea cases present during childhood.
  • Linear morphea can develop before age 10 years in 20% of patients.
  • 0.3 to 3 cases per 100,000/year

Prevalence
  • Onset in children is between ages 2 and 14 years.
  • 50 cases per 100,000 at age 18 years
  • 220 cases per 100,000 at age 80 years

Etiology and Pathophysiology

  • Sclerosis is limited to the skin and systemic sclerosis is absent.
  • Biopsy shows lymphocytic perivascular infiltration of the dermis, decreased number of blood vessels and eccrine (sweat) glands.
  • Causation thought to be from vascular damage and enhanced collagen production, although likely with underlying autoimmune etiology
  • Drug-induced morphea primarily identified in association with TNF-α inhibitors
  • Some association with past trauma, including surgery and vaccination administration, or radiation to the area

Risk Factors

Unknown. Raynaud phenomenon is risk factor for systemic scleroderma, not localized.

Commonly Associated Conditions

  • Occurs with slightly greater frequency in those with a personal or family history of autoimmune disorders
  • Sometimes associated with other diagnosis, such as arthralgias, carpal tunnel syndrome, Raynaud phenomenon, spina bifida, lichen planus, lichen sclerosis, vitiligo, and alopecia areata

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Citation

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TY - ELEC T1 - Morphea ID - 816329 ED - Baldor,Robert A, ED - Domino,Frank J, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816329/all/Morphea PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -