What are cutaneous manifestations of eosinophilic fasciitis (EF)?

Updated: Dec 24, 2020
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Cutaneous manifestations include the following [14, 15] :

  • The cutaneous manifestations of eosinophilic fasciitis evolve as the disease progresses. In the acute inflammatory stage, cutaneous changes include erythematous swelling and nonpitting edema. These findings are later replaced by skin induration, and, eventually, fibrosis predominates. The affected skin is taut and firmly adherent to underlying tissues. Dimpling,  peau d'orange, and venous furrowing, or the "groove sign," can be seen. See the images below.
  • Eosinophilic fasciitis. The arm of this patient demonstrates the puckered, so-called orange-peel or cobblestone skin that may occur in eosinophilic fasciitis.
    Eosinophilic fasciitis. The arm of this patient de Eosinophilic fasciitis. The arm of this patient demonstrates the puckered, so-called orange-peel or cobblestone skin that may occur in eosinophilic fasciitis.
  • Eosinophilic fasciitis. The skin of the patient's back appears shiny due to the stretched dermis overlying an inflamed fascia. Mild diffuse hyperpigmentation is present, along with a U-shaped area of hypopigmentation extending approximately from T10 to L4.
    Eosinophilic fasciitis. The skin of the patient's Eosinophilic fasciitis. The skin of the patient's back appears shiny due to the stretched dermis overlying an inflamed fascia. Mild diffuse hyperpigmentation is present, along with a U-shaped area of hypopigmentation extending approximately from T10 to L4.
  • Eosinophilic fasciitis. The skin of the abdomen and breasts is shiny and taut. The thigh reveals puckering or cobblestoning of the overlying dermis due to scattered retraction from scarred fascia.
    Eosinophilic fasciitis. The skin of the abdomen an Eosinophilic fasciitis. The skin of the abdomen and breasts is shiny and taut. The thigh reveals puckering or cobblestoning of the overlying dermis due to scattered retraction from scarred fascia.
  • Other cutaneous changes reported include  urticaria, bullae, alopecia,  lichen sclerosus et atrophicusvitiligo, and hyperpigmentation.
  • Cutaneous manifestations are generally bilateral and symmetric. The upper extremity, proximal and distal to the elbow, and the lower extremity, proximal and distal to the knee, are most commonly involved. The trunk and neck can also be involved. Face and hand involvement are rare.
  • A concurrent localized lesion of morphea may be seen in 25% of patients.

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