Eosinophilic fasciitis

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - OVERVIEW

Eosinophilic fasciitis (Shulman’s syndrome) is a rare fibrosing disorder of the fascia. It is characterized by symmetrical swelling and skin induration of the extremities, generally without the involvement of the hands and feet, accompanied by peripheral eosinophilia.

3 - SYMPTOMS

Patients with eosinophilic fasciitis typically present:

— Abrupt-onset edema followed by induration of the extremities, with all four extremities commonly affected, symmetrically; Face and digits are typically spared.

— “Groove sign”, which is a depression along the course of the superficial veins in the affected limbs;

— Peau d’orange appearance of the involved skin;

— Joint contractures.

4 - CAUSES

The etiology of eosinophilic fasciitis is unknown.

5 - TREATMENT

Systemic corticosteroids are the first-line treatment for eosinophilic fasciitis.

The efficacy of methotrexate for treating eosinophilic fasciitis has also been reported. Therefore, its use has been proposed as a treatment option, especially in combination with systemic corticosteroids.

In addition, phototherapy (psoralen plus ultraviolet A therapy) may also be considered as an alternative option for the treatment of eosinophilic fasciitis.

6 - TIPS FOR MANAGING

Early recognition and prompt treatment are essential.

Physical therapy should be routinely recommended in combination with systemic treatment to help prevent or improve joint contractures and related functional limitation.