Eosinophillic Fascitis

Question 1

What is Eosinophilic Fasciitis?

Answer 1

A fibrosing disorder with rapid onset of woody induration of the extremities and peripheral eosinophilia.

Question 2

Cutanoues features of Eosinophilic Fasciitis?

Answer 2

Edema and pain of the invovlmed extremity

Progresses rapidly to sclerosis and dimpled skin

Groove sign - linear depression where the viens appear sunken

Tend to be symmetrical, spares hands and feet and face

Question 3

What Ix would you do for someone with eosinophilic fasciitis? and what are the expected results?

Answer 3

FBC
- peripheral eosinophilia
- anaemia / thrombocytopaenia / leukocytosis / paenia - (ass with myelodysplastic syndromes)

UEC
LFTs

ESR - elevated

SPEP - hyergammaglobulinaemia

Fascia Biopsy - thickened deep fascia (10 - 50 times) - Patchy infiltrate of lymphocytes, plasma cells, and variable eosinophils in fascia and subfascial muscle.

+/- MRI

Consider BM biopsy if concerned for haem malginancy

Question 4

How is eosinophilic fasciitis treated?

Answer 4

high dose systemic steroids:
- 1 - 2mg / kg per day, tapered over 6 - 24 months

Steroid sparing agents:
- MTx
- MMF

If an inadequate response
- pulse IV steroids
- IVIG
- HCQ
- JAK inhibiotrs
- UVA based therapy
- Cyclosporin

Question 5

Triggers for developing eosinophilic fasciitis include:

Answer 5

Unusually vigorous activity — most common aetiological factor reported in 50%

Insect bites

Radiation therapy

Infections — Mycoplasma arginini, Borrelia burgdorferi

Medications — atorvastatin, carbidopa, immune checkpoint inhibitors (eg, pembrolizumab, nivolumab)

Paraneoplastic (5–10%) — usually haematolymphoid (eg, leukaemias, myeloproliferative disorders) rather than solid tumours

Chronic graft-versus-host disease.

Question 6

What are the complications of eosinophilic fasciitis?

Answer 6

Joint contractures restrict the range of movement

Symmetrical non-erosive arthritis

Carpal tunnel syndrome due to pressure on the median nerve

Other compartment syndromes

Question 7

Diagnostic criteria for eosinophillic fascitis?

Answer 7

Diagnosis of eosinophilic fasciitis requires the major criterion and one or both of the two minor criteria:

Major criterion
1/ symmetrical plate-like sclerotic lesions on all four limbs

Minor criteria
1/ Skin biopsy
- shows fibrosis of the subcutaneous connective tissue, thickening of the fascia, and infiltrate of eosinophils and monocytes

2/ Thickening of the fascia seen on imaging such as MRI or PET