Rare Cases
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  • Lofgren's Syndrome (Sarcoid Arthritis)
    Lofgrens - Psoriasis Lofgrens A58 year old male, a retired bank employee, presented with 3 week history of bilateral ankle pain and swelling. He also had Lofgrens B Lofgrens Hilar Nodesintermittent dry cough that would be continuous on lying down. 2 painless red nodules developed recently on his left lower extremity. He had recent onset fatigue and lost 2 Kg weight during those 3 weeks. He was a known hypertensive on drugs since 10 years and had skin and nail psoriasis since 34 years. Clinical examination revealed synovitis of both ankles and wrists. He had skin and nail psoriasis along with discrete lesions of erythema nodosum on legs and right hand. A few inspiratory crackles could be auscultated on respiratory examination. ESR was 57 mm at one hour, Rheumatoid factor 57 IU and Serum ACE normal. Other laboratory investigations were normal. His X-ray of chest showed hilar prominence confirmed by CT scan to be lymphadenopathy. He was treated with a 4 week tapering course of prednisolone and settled completely without any recurrence for next 3 years. Association with psoriasis appears to be incidental and rarely reported.
    Sarcoidosis is an inflammatory disorder affecting mainly lungs and lymph nodes. Small inflammatory nodules appear at various sites. It can involve multiple systems including joints, bones, eyes, muscles, liver, spleen and bone marrow. The disease can be mild and asymptomatic to debilitating and lead to death in less than 5% cases. Steroids and methotrexate form mainstay of therapy at present.

    Lofgrens - Psoriasis Lofgrens ALofgren's - Psoriasis
    Lofgren's A 

    Lofgrens B Lofgrens Hilar NodesLofgren's B
    Lofgren's Hilar Nodes

  • Multicentric Reticulohistiocytosis

    Courtesy: Dr. Sunil Tolat (Associate Professor), Dr Arvind Kaul, Department of Dermatology and Venerology, Sasson General Hospital, Pune)

    A 50 year old army man presented with severe debilitating pain in multiple joints for 6 months along with nodular, itchy skin rashes for 4 months. These were associated with loss of appetite, ulcers in mouth and significant weight loss. Various joints - including small hand joints, wrists, elbows, shoulders, knees and ankles - were swollen, tender, restricted and deformed. There were multiple discrete but grouped reddish brown nodules of varying sizes (2mm-2cm) over lower abdomen, face, forearms, back, buttocks, both knees and ankles. Biopsy of skin lesions confirmed clinical diagnosis of multicentric reticulohistiocytosis. Aggressive therapy with zoledronate and methotrexate was instituted. Pain reduced after a period of 3 months and skin lesions, too, started settling down. At the end of One year, deformities still persist and skin lesions, though milder, keep on cropping up intermittently. Multicentric reticulohistiocytosis is a rare disease with destructive arthritis and skin lesions. The disease can involve other organs and may be associated with underlying malignancy. Exact cause of the disease remains unknown and various drugs seem to offer very limited benefit. The disease can remain active for prolonged period. (8 or more years) often leaving the patient with crippling and deformed joints.

    MRH 1
    MRH 1

    MRH 2
    MRH 2

     

  • Osteopoikilosis (Spotted Bone Disease)

    A 23 year old male presented with swelling of both knees. He had history of similar problem 18 months ago treated with joint aspiration and non-steroidal anti-inflammatory drugs. There was no other significant history. Clinical examination revealed another swelling of enthesitis near left ankle. His X-ray revealed osteopoikilosis. Arthritis was treated with non-steroidal anti-inflammatory drugs and settled over a period of 6-8 weeks. Osteopoikilosis is an inherited condition of small, rounded or oval, multiple, symmetric areas of bone sclerosis (increased density) occurring near growing ends and adjacent areas of bones. These densities appear in childhood and persist throughout life. They do not cause any symptoms nor require any treatment.

    Osteopoikilosis 3     Osteopoikilosis 4      Osteopoikilosis Knee Effusion
           Osteopoikilosis 3                                       Osteopoikilosis 4                               Osteopoikilosis Knee Effusion

     

    Osteopoikilosis 1Osteopoikilosis 1



    Osteopoikilosis 2Osteopoikilosis 2

     

     

  • Osteitis condensans ilii
    A 52 year old female complained of intermittent low back pain of varying severity radiating to right thigh since 3 years. The pain was more frequent in cold season and increased after exertion. She underwent hysterectomy 12 years back for dysmenorrhoea. Laboratory investigations, including inflammatory markers, were normal. Her X-ray and MRI showed well defined areas of sclerosis around sacroiliac joints. There was no sacroiliitis.

    Osteitis condensans ilii is a benign condition more common in obese multiparous females. Low back pain radiating to buttocks is a common feature. Pain is exacerbated by activity and relieved by rest. Back pain may be milder in some cases. X ray shows bilateral symmetric sclerosis on iliac sides of sacroiliac joints. The sclerosis is triangular in shape with base pointing inferiorly. Sacroiliitis needs to be differentiated by clinical examination and MRI in doubtful cases. Similar changes can occur at other sites such as pubic bones and medial end of clavicle. The condition may resolve spontaneously or X-ray picture may change from time to time. Therapy consists of posture correction and active exercises for 6-12 months. A few nonresponsive cases may require sacroiliac fusion surgery.

    Osteitis Condensans Ilii XRay
    Osteitis Condensans Ilii XRay

    Osteitis Condensans Ilii MRI
    Osteitis Condensans Ilii MRI

  • Eosinophilia - Myalgia Syndrome

    65 year old Nigerian Indian presented with fever, bone and joint pains as well as severe pain in hands and calf muscles since 3 months. Itchy, extensive skin rash appeared along with. The rash did respond to therapy with residual pigmentation on his forehead. Fever did not respond despite various antibiotics including those used for tuberculosis. He then came to India for further management. His examination revealed pigmentation over forehead, muscle tenderness over extremities, swollen and tender wrist joints and pain and crepitus in both knees (suggestive of osteoarthritis). Blood examination revealed high eosinophil (a type of white blood cell) count and raised ESR. Other laboratory investigations were normal. Fever and myalgia responded to steroids and painkillers. The patient went back to Nigeria after 3 weeks or so.

    Eosinophilia Myalgia syndrome is closely linked to ingestion of tryptophan in diet although evidence of its ingestion is not essential for diagnosis of this disease. Tryptophan was, therefore, withdrawn from US market in 1991 but sales were again allowed from 2001. Tryptophan is available in food stores as diet supplement. People use it for insomnia, depression and menstrual problems. Average daily dose of 1250 mg for 6 months to 9 years is supposed to lead to this disease.
    Tryptophan is a routine constituent of most protein-based foods or dietary proteins. Chocolates, oats, bananas, mangoes, dried dates, milk and milk products, spirulina, peanuts, red meat, eggs, fish, poultry and turkey are all rich sources of tryptophan.

    Symptoms of Eosinophilia Myalgia syndrome can be difficult to respond to therapy. Muscle pain and weakness, fatigue, joint pains, breathlessness, memory loss and numbness can persist even at the end of 1-4 years.

  • Relapsing Polychondritis


    (Courtesy: Dr. Ajay Khurana, Physician, Pune, India)

     

    A 38 year old male had complaints of intermittent pain and mild swelling in ankle and foot joints since 2 years. He was under treatment of a homeopathic doctor without any significant relief. His joint pain was associated with pain in rib cage and sometimes with a change of voice and redness of eyes. He used to feel feverish during these arthritic episodes and was generally unwell, developed loss of appetite and nutritional anaemia.

    In view of no response to homeopathic drugs, he switched to Ayurveda therapy. The Ayurveda physician prescribed him purging which was followed by severe abdominal pain, diarrhea and dehydration. He was hospitalized for the same where his condition stabilized. He developed pain and mild swelling in some of his joints along with pain in left lower ribs. This was followed by painful, pink, tender and warm swelling of pinna of his left ear – a classic finding in Relapsing Polychondritis.

    Relapsing Polychondritis is a multisystem autoimmune disease due to swelling of cartilage tissue. Cartilage is a firm (softer than bone) and flexible structure that is found in ear (external as well as internal), nose, glottis (phonating organ in throat), wind-pipe (trachea), rib cage, joints and intervertebral discs. Relapsing Polychondritis can be primary or be associated (30% cases) with various autoimmune and other diseases. Recurrent swelling of ear, nose and other organs leads to deformities (cauliflower ear, saddle nose). Obstruction of wind-pipe, deafness and redness of eye with diminution of vision are dreaded complications occurring in about 50% patients over course of this disease. Defects of heart valves (aortic), swelling of blood vessels, involvement of nervous system and kidneys can also occur in Relapsing Polychondritis. Prognosis of patients with Relapsing Polychondritis has significantly improved in recent years due to better management with drugs and surgery in selected patients.

    Relapsing Polychondritis
    Relapsing Polychondritis

    Relapsing Polychondritis 2
    Relapsing Polychondritis

     

     

  • Bisphosphonate induced Osteonecrosis of Jaw
    Bisphosphonates are notorious to cause necrosis of jaw bones in rare cases. A 48 year old male was on Ibandronate for over 18 months. His right molar tooth cracked and was removed subsequently. The operation wound did not heal and developed infection. This required another extensive debridement surgery after 2 months. The wound is still unhealed at the end of 4 months.
    Bisphosphonates are drugs used in treatment of osteoporosis, cancers and a few other rheumatology conditions. There is increased incidence of jaw necrosis in patients on low dose bisphosphonates although no definitive causal link is established as yet. The overall incidence appears to be 1 in over 10000 patient years. Dental trauma and surgery, poor oral hygiene and long term high dose intravenous (as in cancer therapy) use of bisphosphonates are some of the risk factors for development of osteonecrosis. Patients on these drugs must maintain a good oral hygiene and inform their dentists about use of these drugs before use of any dental procedure.

    ONJ 1
    ONJ 1

    ONJ 2
    ONJ 2

  • Risedronate induced Uveitis in patient with Pagets Disease

    (Courtesy: Dr. Aditya Kelkar, National Insitute of Ophthalmology, Pune)

    A 66 year old male was suffering from Paget's disease since 1999. He developed stress fracture of left thigh bone on January 2006. The patient was referred in May 2006 as fracture healing was not satisfactory. He was administered 2 intravenous injections of pamidronate and was prescribed risedronate 35 mg weekly later on. Both of these belong to aminobisphosphonate group of drugs used in treatment of active Paget's disease. The fractures healed. Patient did not visit rheumatologist and continued to take risedronate till November 2008.
    He developed dimness of vision due to uveitis in July 2008 and received various treatments including intraocular injections from different ophthalmologists for the same. His vision finally returned to normal 5 months after withdrawal of risedronate. World Health Organisation issued a poster reporting over 500 adverse ocular side effects until May 2004. These can cause redness and pain in eye as well as loss of vision. Withdrawal of the offending drug leads to restoration of visual problem.

    Pagets Vitreous haze B scanus     Pagets A      Pagets 4
    Pagets Vitreous haze B scanus                        Pagets A                                                 Pagets 2

     

    Pagets 1
    Pagets1


    Pagets 2Pagets 2

    Pagets 3Pagets 3

     

     

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