‘Something’s tightening under my skin’

Dr Lata Bichile

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 four months, with a shooting pain from the wrist to the fingers, making simple movements like 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 struggled 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 spared — 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 other organ systems 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 hundred cases have been reported worldwide since it was first 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 cell 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.


Three 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 two 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.


(Dr Lata Bichile is a consultant rheumatologist at Centre for Arthritic and Rheumatic Diseases Treatment, Mumbai. She spoke to Sharmila Ganesan Ram)