Borderline tuberculoid hansens with type 1 reaction in a HIV patient: an IRIS phenomenon over an unusual site

INTRODUCTION

Leprosy is an ancient and most feared disease which has a strong social stigma and continues to be a public health problem in many resource poor countries. There is a geographical overlapping in the incidence of leprosy and HIV which will lead to an increase in dually infected individuals, particularly in resource poor countries.

Immune reconstitution inflammatory syndrome (IRIS) is a paradoxical deterioration in the clinical status of a HIV infected patient on highly active antiretroviral therapy (HAART) due to recuperating immune system. IRIS can occur due to both infectious as well as non-infectious causes and mostly occurs with low CD4 count. Leprosy presenting as IRIS in HIV patients after initiation of antiretroviral therapy has been reported previously.¹ In contrast to previous reports, we report a case of HIV on HAART who presented with borderline tuberculoid leprosy with type 1 lepra reaction presenting as IRIS over unusual site.

CASE REPORT

A 35 year old male patient came with complaints of multiple red, swollen, painful patches over right elbow, back of hand, abdomen and penis of 1 month duration. He was diagnosed to be HIV positive 4 years back and was started on HAART one year back as his CD4 count was 182 cells/cu. mm.

On cutaneous examination, multiple, erythematous, edematous, tender well defined plaques of 1-7 cm size, few plaques showing scaling over the extensor aspect of right elbow, dorsum of right hand, right flank and penis with multiple satellite lesions were observed [Figures 1-4].

Loss of fine touch sensation and temperature differentiation over the plaques and patches were present. Right and left ulnar and left popliteal nerves were thickened. Right ulnar nerve was tender to palpate.

Scrapings for KOH from glans penis were negative. Venereal disease research laboratory was negative. CD4 count was 218 cells/cu.mm, slit skin smear was negative.

On histopathological examination, multiple Langhans giant cells in the mid dermis along with lymphocytic inflitrate in upper and mid dermis was seen which was suggestive of tuberculoid granuloma [Figures 5 and 6].

The hypoasthetic, erythematous and odematous plaque occurring over penis was rather unusual in our case. With the clinical and histopathological findings, a diagnosis of borderline tuberculoid leprosy with type 1 reaction presenting as IRIS in HIV patient was made. Patient was started on anti-inflammatory drugs, oral corticosteroids and Mycobacterium- multidrug therapy.

DISCUSSION

IRIS is due to the impaired immune response after the start of ART. The characteristic feature of the IRIS is paradoxical worsening of an existing infection or disease process or appearance of a new infection/disease process soon after initiation of therapy. The immunopathogenesis of IRIS is not clearly identified but few studies show that it is due to the overt inflammatory response in patients receiveing ART due to imbalance of effector and regulator T cells. Biomarkers like body mass index, hemoglobin level, total leukocyte count, albumin level, high-density lipoprotein level, erythrocyte sedimentation rate value, C-reactive protein level and absolute eosinophil level less than 351 cells/mm³ showed significant (p<0.05) difference among HAART naïve and on HAART patients with IRIS.²

Few other biomarkers, which have been found to have a role in IRIS are interferon-γ, tumour necrosis factor-α and interleukin- 2, 6 and 7. Also mycobacterial infections, fungi and herpes viruses can be associated with IRIS.³ To be diagnosed as IRIS, few minimum criteria need to be fulfilled. Temporal association between initiation of antiretroviral therapy and subsequent development of symptoms has to be established. The clinical course should neither be consistent with the usual course of a previously diagnosed opportunistic infection or a new infectious process; nor should the symptoms and signs be explained by drug toxicity.³

In our case, there is only a minimal increase in CD4+ count after starting HAART and viral load was not done due to unaffordability. It has been found that even a minimal decrease in viral load in the absence of a significant rise in CD4+ cell count itself can precipitate IRIS.⁴ The first case of Hansen's disease presenting as IRIS was reported in 2003 by Lawn et al.⁵ In the study by Pereira et al, a significant percentage of co-infected patients were classified as the borderline tuberculoid leprosy.⁶

A review of the Indian scenario revealed only six cases of Hansen's disease presenting as IRIS. All were of the borderline tuberculoid type and were associated with type 1 lepra reaction.^7-11 Thus our case can be added to the Indian scenario.

CONCLUSION

Although leprosy is fading out in other countries, it is still a major problem in countries like India and thus coexistence of HIV and leprosy should be always kept in mind and diagnosis is not to be missed.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

REFERENCES

1. 1. Cusini A, Günthard HF, Weber R, Huber M, Kamarashev J, Bertisch B, et al. Lepromatous leprosy with erythema nodosum leprosum as immune reconstitution inflammatory syndrome in an HIV-1 infected patient after initiation of antiretroviral therapy. BMJ Case Rep. 2009;2009:bcr0520091904.
2. 2. Mishra SK, Khadka S, Dhital S, Mahto RK, Manandhar KD. Biomarkers in immune reconstitution inflammatory syndrome (IRIS) among people living with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS). J AIDS Clin Res. 2017;8:728.
3. 3. Sharma SK, Soneja M. HIV and immune reconstitution inflammatory syndrome (IRIS). Indian J Med Res. 2011;134:866–77.
4. 4. Hirsch HH, Kaufmann G, Sendi P, Battegay M. Immune reconstitution in HIV infected patients. Clin Infect Dis. 2004;38:1159-66.
5. 5. Lawn SD, Wood C, Lockwood DN. Borderline tuberculoid leprosy: An immune reconstitution phenomenon in a human immunodeficiency virus-infected person. Clin Infect Dis. 2003;36:e5-6.
6. 6. Pereira GA, Stefani MM, Filho JA, Souza LC, Stefani GP, Martelli CM. Human immunodeficiency virus type 1 (HIV-1) and Mycobacterium leprae co-infection: HIV-1 subtypes and clinical, immunologic and histopathologic profiles in a Brazilian cohort. Am J Trop Med Hyg. 2004;71:679-84.
7. 7. Kharkar V, Bhor UH, Mahajan S, Khopkar U. Type 1 lepra reaction presenting as immune reconstitution inflammatory syndrome. Indian J Dermatol Venereol Leprol. 2007;73:253-6.
8. 8. Rao GR, Amareswar A, Sandhya S. Can highly active antiretroviral therapy unmask leprosy?. A case of type 1 lepra reaction in a HIV-seropositive patient. Indian J Dermatol Venereol Leprol. 2012;78:101-3.
9. 9. Mehta S, Padhiar B, Shah B. Leprosy presenting as immune reconstitution inflammatory syndrome. Indian J Sex Transm Dis. 2008;29:96-7.
10. 10. Mukhopadhyay P, Pal S, Mallik S, Biswas S, Saha B. Borderline tuberculoid leprosy: A manifestation of immune reconstitution inflammatory syndrome in a human immunodeficiency virus infected person. Indian J Dermatol. 2006;51:278-80.
11. 11. George A, Vidyadharan S. Hansen's disease in association with immune reconstitution inflammatory syndrome. Indian Dermatol Online J. 2016;7:29-3.