Pages 204 - 208 Volume 91, Issue 2
Back
Hansen’s Disease presenting as tenosynovitis

Tenosynovitis often occurs in Hansen’s Disease (HD) as part of a Type 1 leprosy reaction and rarely as the sole presenting feature of HD. Here, we report a case where the patient presented with tenosynovitis and was later diagnosed as having HD.

Cite this article
Punya Suvarna, Sudhir Nayak U. K., Shrutakirthi D. Shenoi, Chethana Babu K. Udupa, Anil K. Bhat;
Hansen’s Disease presenting as tenosynovitis; Leprosy Review; 2020; 91; 2; 204-208; DOI: 10.47276/lr.91.2.204
LEPROSY
Leprosy Review
0305-7518
British Leprosy Relief Association
Colchester, UK
Introduction
Hansen’s Disease (HD) often involves the musculoskeletal system during reactional states and rarely as the sole presenting feature. A high degree of suspicion of HD should be maintained in cases of arthritis and tenosynovitis, not responding to treatment.
In treated cases, the multiplication of ‘persisters’ is often the cause for relapse of the disease and the patient should be followed up at 6 monthly intervals to rule out early relapse.
We report a case of tenosynovitis as the sole presenting feature of HD, in an already treated case; this was most likely due to a relapse caused by persister bacilli.
Case report
A 43 year old fisherman presented in 2019 to the Orthopaedic department with insidious onset of pain and swelling of the fingers of his right hand, of 2 months duration. It was a dull aching type of pain, aggravated on movement, associated with difficulty in gripping and lifting objects and performing fine movements, such as mixing food and buttoning and unbuttoning of the shirt. Ultrasound study of the right wrist revealed diffuse synovial thickening involving the flexor pollicus longus and flexor tendons to the index and middle fingers at the level of the wrist joint, and thickening of the A2 pulley of the thumb and the A1 pulley of the index and middle fingers; all features were suggestive of right hand flexor tenosynovitis. He underwent exploration, tenosynovectomy and open carpal tunnel release with excess synovial tissue being sent for histopathological analysis. The section showed features of hyperplastic synovium overlying a fibro-collagenous stroma composed of confluent non-caseating granulomas in perivascular and perineural areas comprising of epithelioid histiocytes, numerous Langhans giant cells and lymphocytes (Figure 1). This histopathological finding provided a diagnosis of granulomatous tenosynovitis suggestive of tuberculoid leprosy.
Figure 1.
The section showed features of hyperplastic synovium overlying a fibro-collagenous stroma composed of confluent non-caseating granulomas in perivascular and perineural areas comprising of epithelioid histiocytes, numerous Langhans giant cells and lymphocytes.
He was referred to the dermatology department for further evaluation and management. On additional questioning, he provided a past history of tingling and numbness along the distribution of the left ulnar nerve along with a few hypopigmented patches over the forearm in 2015. At that time, he was diagnosed to have Borderline Tuberculoid Hansen’s Disease with left ulnar nerve neuropathy for which he had completed 12 months of MB-MDT. He reported clearance of patches and recovery of the lost sensation over the left arm. Furthermore, his slit skin smear showed no evidence of acid fast bacilli. Hence, he was considered to be treated and was asked to come for follow-up at yearly intervals.
He was re-examined on this visit, and found to have decreased range of movement at the right wrist joint. The right ulnar nerve was found to be thickened with intact sensations. Slit Skin Smear was done and found to be negative. In view of the histopathology report and thickened right ulnar nerve, he was restarted on MB-MDT and is being followed up on a monthly basis. He has also been advised physiotherapy exercises to improve mobility.
Discussion
Involvement of the musculoskeletal system is the third most common organ system involved in Hansen’s Disease with a common rheumatologic presentation being that of acute painful edema of the hands with arthritis or tenosynovitis.1 A combination of arthritis, tenosynovitis with or without paresthesia and thickened nerves is highly suggestive of leprosy.
Tenosynovitis is occasionally seen in Type 1 leprosy reactions. The pathogenesis of tenosynovitis can be explained by the hematogenous spread of leprosy bacilli and bacillary antigens getting trapped in the synovial lining of the tendon sheath, giving rise to an inflammatory cascade.2
Cases of Hansen’s Disease may present with rheumatological complaints of inflammatory arthritis with or without tenosynovitis, along with other features of leprosy reactions, such as neuritis and constitutional symptoms.1,3 Prasad et al. described patients presenting with rheumatological manifestations to a rheumatology department, who were subsequently diagnosed to have leprosy.4 Our case was unique as the patient presented with tenosynovitis alone without features of leprosy reaction or associated arthritis.
A high index of suspicion, careful history, and physical examination, especially of the peripheral nerves, are helpful in arriving at a diagnosis. Leprosy arthritis responds well to anti-leprosy medications and is an easily treatable cause of arthritis. This also avoids long and unnecessary administration of anti-rheumatic drugs.5 The past history of BT spectrum of Hansen’s Disease, the thickened right ulnar nerve, and confirmatory synovial tissue histopathological findings helped us come to a diagnosis of relapse of Hansen’s Disease in this patient.
The patient was considered to be cured of Hansen’s Disease as he had completed the recommended 12 month duration of MB-MDT and his skin and neural complaints resolved. However, he presented after an interval of three years with involvement of the opposite hand and nerve with rheumatological complaints. Relapses occurring within three years of completing MDT suggest the existence of ‘persisters’ as a possible explanation. Persisters consist of permanently or partially dormant organisms that have the capacity to survive in the host, despite adequate chemotherapy. They have been identified in immunologically favorable sites such as dermal nerves, smooth muscle, lymph nodes, iris, bone marrow and liver. These organisms, which are responsible for relapse, are present in about 10% of the MB patients. At the same time, we should also consider a possible ‘reinfection’ with leprosy bacilli. Reinfection is very difficult to exclude, especially in an endemic area. When cured, leprosy patients continue to live in and around the original site of leprosy acquisition. In hyperendemic areas, they may develop the disease again due to exogenous infection.6 It may be possible to differentiate reinfection from relapse by molecular typing of M. leprae, based on nucleic acid amplification techniques such as PCR.7
In a country endemic for Hansen’s Disease, it is important to keep it as a differential diagnosis for tenosynovitis or arthritis, which presents suddenly and fails to respond to conventional lines of therapy, irrespective of the skin lesions or nerve involvement. In treated or ‘cured’ cases of Hansen’s Disease, a yearly examination is necessary to identify relapse or reinfection of the disease as soon as possible. Regular Slit Skin Smears, biopsy of representative or old lesions or immunological tests like the dipstick test to detect anti-PGL-1 antibodies, can do this.6 Early detection and initiation of treatment can prevent the progress of the disease and irreversible deformities from occurring.
On literature review, we could only find two other cases of Hansen’s Disease presenting with isolated tenosynovitis, with only one being confirmed by PCR.8 Thus, we would like to report this case keeping in mind its distinctiveness as well as its uncommon manifestation as a potential relapsing feature of Hansen’s Disease.
Declaration
No review board approval was necessary.
Conflicts of interest
The authors report no conflicts of interest.
Funding
No funding was given for reporting this case.
Patient consent statement
Patient consent has been obtained.
Contributorship statement
0
Dr. Punya Suvarna, Dr. Sudhir Nayak and Dr. Shrutakirthi D Shenoi saw the patient in the Dermatology OPD, worked him up for Hansens Disease; wrote and edited the manuscript.
0
Dr. Chethana Babu was the pathologist who analysed the synovial tissue section and reported the findings.
0
Dr. Anil K Bhat was the Orthopaedician who diagnosed him to have tenosynovitis and operated on him for the same.
Guarantor
Dr. Sudhir Nayak UK
References
[1]HaroonN, AgarwalV, AggarwalA, KumariN, KrishnaniN, MisraR. Arthritis as presenting manifestation of pure neuritic leprosy: a rheumatologist’s dilemma. Rheumatology, 2007; 46: 653656.
[2]AgarwalV, WakhluA, AggarwalA, MisraR. Tenosynovitis as presenting manifestation of leprosy. J Indian Rheumatol Assoc, 2002; 10: 6970.
[3]BavejaS, BishtPB, SoodA. Tenosynovitis: an unusual presentation of leprosy. Int J Mycobacteriol, 2017; 6: 196198.
[4]PrasadS, MisraR, AggarwalA, LawrenceA, HaroonN, WakhluA Leprosy revealed in a rheumatology clinic: a case series. Int J Rheum Dis, 2013; 16: 129133.
[5]WakhluA, SawlaniKK, HimanshuD. Rheumatological manifestations of hansen’s disease. Indian J Rheumatol, 2018; 13: 1419.
[6]KaimalS, ThappaDM. Relapse in leprosy. Indian J Dermatol Venereol Leprol, 2009; 75: 126135.
[7]SenguptaU. Immunological aspects of relapse in leprosy. Indian J Lepr, 1995; 67: 8183.
[8]DeA, SuranaTV, BiswasS, RejaAH, ChatterjeeG. Isolated tenosynovitis as a sole manifestation: the great mimicker still continues to surprise us. Indian J Dermatol, 2015; 60: 213.