Something’s tightening under my skin’ by Dr.Lata Bichile, a rheumatologist at Centre for Arthritic and Rheumatic Diseases Treatment in Mumbai.
MK, a 42-year-old engineer, walked into my OPD along with his wife in August 2023. He had been experiencing swelling and stiffness in his right forearm for the past 4 months with a shooting pain from the wrist to the fingers, making simple movements like bending his fingers, bending his elbow or turning his wrist difficult. Similar pain and stiffness had begun appearing in both thighs and his left upper arm as well. So much so that he struggles to sit down and stand up. Even lifting his left arm hurt. “ it feels as if something inside is tightening:, he said.
MK
had no fever, no weight loss, no breathlessness, no cough, no colour
changes in his fingers and no known allergies or asthma. His hands and
feet were normal. That detail stood out. In some autoimmune diseases,
the skin on the fingers thickens or changes colour. But here, the
extremities were entirely spread- a clue that steered my mind towards a
much rarer possibility.
On
examination, his right elbow movement was restricted. The skin over his
forearm and thighs appeared subtly puckered- almost tethered down. His
median nerve seemed affected, causing numbness in his fingers. Yet, his
muscle strength was normal, reflexes intact, and the other organ system
perfectly fine.
Could this be Eosinophilic Fasciitis? I wondered.
Eosinophilic
Fasciitis, or Shulman syndrome, is a rare inflammatory condition
affecting the fascia- the thin layer of connective tissue that surrounds
our muscles. It causes painful swelling, tightness, and skin
thickening. Only a few 100 cases have been reported worldwide since it
was 1st described in 1974. Most patients are middle aged, though it can
occur at almost any age. Many doctors may never encounter it in their
career.
His
blood tests revealed high inflammation markers (ESR, CRP) and very high
eosinophils- a type of white blood cells that can indicate
inflammation. His muscle enzyme levels were normal, helping rule out
primary muscle disease. Tests for autoimmune diseases- ANA, Anti-Scl70,
Anti-centromere, ANCA- were negative, which helped exclude systemic
sclerosis and other mimicking disorders.
He
was not willing to undergo a biopsy as it was an invasive procedure.
So, I ordered an MRI of his thighs. Imaging today often helps confirm
what we suspect clinically. The scan showed exactly what I had thought=
inflammation and fluid along the fascial layers, with thickening but no
muscle damage- a textbook picture of Eosinophilic Fasciitis.
I
explained to him and his wife that this was a rare but treatable
condition, and that we had caught it early. Relief flooded their faces.
People with rare illnesses often fear they are being dismissed or
misunderstood. We started treatment with high-dose steroids, carefully
adjusted to his body weight and monitored.
Alongside,
we gave him a steroid-sparing medication to protect him long-term.
Physiotherapy became an important part of recovery. I scheduled close
follow-ups as rare diseases deserve regular attention.
3 months
later, he returned smiling. His elbow moved normally, his thighs were
supple, his shoulder pain had disappeared, and the skin tightening had
eased dramatically. He continued working without interruption. His
consistency, trust, and calm courage played a big role in his
improvement.
I
am 75 and, in a career spanning around 35 years, I’ve seen only 2 cases
of Eosinophilic Fasciitis. But every rare patient reinforces key
lessons: awareness, early diagnosis, and trust in treatment matters. This
peculiar case shows how rare diseases may masquerade as routine
problems. The case also taught me that not every success in medicine is
dramatic. Sometimes, victory looks like a restored elbow bend, a
pain-free walk or a man returning to work feeling whole again.