I had signed out the report as consistent with either Morphea or Eosinophilic fasciitis
I received many comments from people who either thought this was morphea (limited scleroderma) or suggested the DDx of Morphea vs Eosinophilic Fasciitis….as that is apparently the main differential.
I’ve included highlights from an online reference about eosinophilic fasciitis.
Summary
Eosinophilic fasciitis is a rare disorder characterized by inflammation of the tough band of fibrous tissue beneath the skin (fascia). The arms and legs are most often affected. Inflammation is caused by the abnormal accumulation of certain white blood cells including eosinophils in the fascia. Eosinophilic fasciitis eventually causes the skin to swell and slowly thicken and harden (induration). The disorder most commonly affects middle-aged adults. The specific symptoms and severity of eosinophilic fasciitis can vary from one individual to another. The exact cause of eosinophilic fasciitis is unknown.
Introduction
Eosinophilic fasciitis, also known as Shulman syndrome, is named after the physician who, in 1974, was the first to report on the disorder in the medical literature. Some researchers believe that eosinophilic fasciitis is a variant of scleroderma (systemic sclerosis), an autoimmune connective tissue disorder characterized by hardening of the skin. However, these entities can usually be distinguished by their clinical characteristics.
Treatment
The treatment of eosinophilic fasciitis is directed toward preventing and alleviating tissue inflammation. In some cases, affected individuals improve without treatment (spontaneous remission). Many individuals respond favorably to corticosteroid therapy, and the drug prednisone is often prescribed. Prednisone therapy may be required for two months or longer. In many cases, high doses of corticosteroids are used at first and slowly tapered off. Cases have been reported where eosinophilic fasciitis eventually recurred following corticosteroid therapy.
Additional treatment is symptomatic and supportive. For example, surgical decompression of affected nerves may be used to treat carpal tunnel syndrome. Surgery may also be recommended to treat contractures. Non-steroidal anti-inflammatories (NSAIDs) may be used to provide relief as well. Physical therapy may be beneficial in some cases.
Sigrud Skaar Holme sent a very informative comment with references:
We had quite often patients with eosinophilic fasciitis, though, and I think your images could look like eosinophilic fasciitis. It looks like the oedema follows the fascias of the muscles, including the deep fascia. Your images look like the morphea images in the article mentioned, too, I think. Perhaps the findings are not specific, but they are consistent with the diagnosis of morphea (or a fasciits…)?
I found and article: https://link.springer.com/article/10.1007/s40257-017-0269-x describing eosinophilic fasciitis as an extreme form of morphea… Found the link here: https://bindevevssykdommer.no/eosinofil-fasciitt/ (Norwegian website, but in English 😊, run by my former college Øyvind Palm, a rheumatologist at the hospital in Oslo. Annoying advertising on the website…). Morphea diagnosis is mentioned as a differential together with some other, one which was a bit scary:
Fasciitis-panniculitis syndrome . Erythema, oedema, induration in extremities, arthritis, myalgia, fever. Previously thought to be a subgroup of eosinophilic fasciitis, but eosinophilia is often absent, and the condition is more frequently associated with malignancy (reference: Nishikubo M, 2021 ). Tissue sample (histological) shows infiltration of inflammatory cells in fatty tissue ( panniculitis ) and muscle fascia compatible with septal and lobular panniculitis. The edema can be misinterpreted as deep vein thrombosis (reference: Morales M, 2006 ).
On Mon, Jul 29, 2024 at 3:22 PM hilary umans <hilary.umans> wrote:
Referring rheumatologist gives history of subcutaneous fibrosis and requested "rule out morphea or other deg fibrosis"I have a clinical note from her internist in March that reports hand hand joint and bone pain and no skin changes or rash
I have no experience with Morphea…but having looked up the term, I see it refers to localized scleroderma and a number of autoimmune conditions involving skin thickening and increased collagen deposition
I gather there is a Mayo Clinic classification that was discussed in an AJR article from 2008….though I’m not really sure how this would be classified.
It does resemble images from the literature
Is this specific for Morphea?
Or is that a silly question because Morphea is not actually a specific diagnosis?
Is there a differential diagnosis?
