Recurrent uveitis and nodular skin lesions revealing lepromatous leprosy

Case report

A 51-year old woman from North India with type 2 diabetes mellitus presented with a 5-year history of recurrent episodic redness of both eyes with photophobia and blurring of vision. These episodes occurred every 3–4 months, lasted for 7–10 days and responded to topical steroids and cycloplegics. During the active episodes, visual acuity was preserved. Slit-lamp examination revealed cells and flare in the anterior chamber, consistent with anterior uveitis, without pupillary abnormalities, impaired light reflexes, posterior synechiae nor lens changes. The posterior segment was unremarkable, with no evidence of diabetic retinopathy nor posterior segment inflammation. A diagnosis of recurrent acute anterior uveitis was suggested. She also had recurrent erythematous tender nodules and plaques distributed over the body, predominantly involving the extremities (Fig. 1A and B), abdomen and back. These lesions were well-defined, indurated and variably sized, with some showing superficial scaling and resolved within a week without ulceration, discharge, scarring or post-inflammatory hyperpigmentation.

OPEN IN VIEWER

Figure 1.

Clinical and histopathological images of lepromatous leprosy (A) Erythematous nodular lesion with superficial scaling over the thigh. (B) Multiple well-defined erythematous nodules over the arm. (C) Histopathology of the skin lesion showing perivascular and periadnexal inflammatory infiltrate composed of foamy histiocytes (H&E stain, ×400). (D) Wade–Fite stain demonstrating numerous acid–fast bacilli within foamy histiocytes (highlighted by circles) (×1000).

There was no history of cough, dyspnoea, oral or genital ulcers, altered bowel habit, haematochezia, joint pain or joint swelling. There were no hypo-aesthetic skin lesions, madarosis, lagophthalmos or leonine facies. Detailed peripheral nerve examination did not reveal sensory or motor deficits, nerve tenderness or nerve thickening. Differential diagnoses included sarcoidosis, tuberculosis, Behçet disease and inflammatory bowel disease–associated uveitis. Investigations revealed no anaemia (Hb 131 g/L), normal angiotensin-converting enzyme level (36 IU/L), a normal chest radiograph, a negative tuberculin skin test and a negative HLA-B27 test by polymerase chain reaction.

Histopathological examination of one of the skin lesions revealed superficial and deep dermal inflammatory infiltrates in a predominantly perivascular and periadnexal distribution. The infiltrate was composed mainly of foamy histiocytes (Fig. 1C) mixed with mature lymphocytes. Focal extension of inflammation into the subcutaneous tissue with mild lobular panniculitis was noted. Wade–Fite staining demonstrated numerous acid–fast bacilli clustered within the cytoplasm of the foamy histiocytes (Fig. 1D). Histopathological features were consistent with a lepra reaction–related inflammatory process rather than erythema nodosum leprosum, in view of the absence of prominent neutrophilic infiltration. The slit-skin smear also revealed acid–fast bacilli with a bacteriological index of 4+. Based on these findings, a diagnosis of lepromatous leprosy was established.

Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae and remains an important clinical mimic of several rheumatic and inflammatory disorders, particularly in endemic regions. ¹ Reported frequency of rheumatic manifestations shows marked variation across studies, ranging from 1% to 2% in large dermatology-based series to as high as 60% to 80% in rheumatology clinic–based cohorts. ² Musculoskeletal manifestations such as arthritis, tenosynovitis, vasculitic lesions, erythema nodosum and peripheral neuropathy may closely resemble autoimmune diseases, often leading to diagnostic delay. ³ Ocular involvement is more common in lepromatous leprosy and has been reported in up to 85% of patients. ⁴ It may result from direct bacillary infiltration, involvement of the facial and trigeminal nerves, immune-mediated inflammatory reactions or secondary changes affecting the eyelids, lacrimal drainage apparatus and supporting periocular tissues. Reported ophthalmic manifestations include madarosis, lagophthalmos, exposure keratitis, episcleritis, scleritis and uveitis. ⁵

Our patient was started on rifampicin, dapsone, clofazimine and prednisolone, which was initiated at a dose of 0.5 mg/kg/day and continued for one month, followed by gradual tapering by 5 mg every 2 weeks to 15 mg/day, and thereafter by 2.5 mg every 2 weeks until complete discontinuation over one year, according to World Health Organisation recommendations.

At 1-year follow-up, there was complete resolution of skin lesions and no recurrence of uveitis. Repeat ophthalmologic evaluation showed quiescent anterior and posterior segments with no pupillary abnormalities, posterior synechiae, lens changes or retinal involvement. No new peripheral neuropathy nor steroid-related complications, including worsening glycaemic control, were identified. Public health authority notification was made according to standard regulations.

Our case highlights the protean manifestations of leprosy and emphasizes that recurrent uveitis may rarely be the presenting feature of lepromatous leprosy.