Intermediate uveitis, splenomegaly and cutaneous ulceration : Leprosy in modern Rheumatology practice
Lucy M Carter, Hudaifa Al-Ani, Samantha Line, Sri Sharma, Alexander Brand, Lorraine O’Neill
Oxford University Hospitals NHS Foundation Trust
We present a case which illustrates the epidemiology and clinical features of leprosy, as relevant to current Rheumatology practice.
A 48 year old lady, originating from Timor Leste, presented to the Eye Hospital with subacute visual disturbance. Ophthalmology assessment revealed bilateral intermediate uveitis. The patient had no prior medical history and was on no regular medication. She had limited contact with health services since arrival in the UK six years previously. Further enquiry identified progressive skin lesions and 10 kg weight loss over the prior six months.
Clinical examination revealed extensive well demarcated and relatively hypoalgesic cutaneous ulcers, below the knees bilaterally with surrounding hyperpigmentation. There was a solitary annular macule with central atrophy on the left upper arm and a subtle saddle nose deformity. Cardiorespiratory examination and vital signs were normal. Peripheral joint examination was unremarkable. There were no long tract signs and no clinically apparent neural thickening.
Cross sectional imaging confirmed clinical findings of 16 cm splenomegaly and bilateral lymphadenopathy confined to the inguinal region. Laboratory investigations showed severe anaemia Hb 53 g/L with iron deficiency haematinics and normal white cell differential. Routine clinical chemistry was normal. Acute phase markers were mildly elevated, CRP 16.2 mg/L. Serum ACE was significantly elevated at 132 U/L. HIV, syphilis and toxoplasma serology were negative.
Microscopy of inguinal lymph node biopsy revealed non-caseating gramulomata and numerous acid fast bacilli subsequently confirmed by culture and molecular techniques as mycobacterium leprae. Skin biopsy confirmed mycobacterial infection with chronic inflammation and extensive scarring. The patient was commenced on multidrug therapy.
Despite active worldwide leprosy elimination campaigns, areas of high endemicity persist and multisystem involvement may still bring this disease to the attention of the Rheumatologist.