Can you identify the disease?
MD, DM
Consultant Rheumatologist, Sterling Ramakrishna Specialty Hospital, Gandhidham, Kutch, Gujarat
Question:
59-year-old lady presented with two months history of diffuse right arm pain along with bilateral small and large joint polyarthritis with early morning stiffness of 30 minutes, dorsal hand and feet pitting edema. On investigating, hemogram, RFT and LFT was normal, rheumatoid factor and anti-cyclic citrullinated peptide was negative, ESR and CRP was 34 mm and 7 mg/L respectively. She was treated as remitting seronegative symmetric synovitis with pitting edema (RS3PE).
On one monthly follow up, she responded poorly to low dose oral steroids and Methotrexate. She became wheelchair bound and presented with new onset rapidly progressing palmar and finger contractures along with difficulty in pronating right hand (due to mechanical restriction) (Picture). X ray hands was normal.
View Answer
Answer:
The clinical picture of rapidly progressing palmar contractures, polyarthritis and poor response to steroids led to the suspicion of Palmar fasciitis and polyarthritis (PFPO) syndrome. A CECT chest and abdomen was done which was suggestive right ovarian carcinoma. After relevant further workup she underwent chemotherapy and responded well.
Suggested Reading:
1. Manger B, Schett G. Palmar fasciitis and polyarthritis syndrome-systematic literature review of 100 cases. Semin Arthritis Rheum.2014;44(1):105-111.
The Indian Rheumatology Association
The Professional Organization of Clinical Immunologists and Rheumatologists In India
Dr. Vinod Ravindran
Consultant Rheumatologist, Centre for Rheumatology, Calicut, Kerala.