University Journal of Medicine and Medical Specialities

Transcription

1 University Journal of Medicine and Medical Specialities Volume 1 Issue ATYPICAL PRESENTATION OF LEPROMATOUS LEPROSY LOCALISED TO BILATERAL FLANKS ANANDAN V, RAJESH HN Stanley Medical College Abstract: Leprosy is a chronic progressive infectious disease caused by Mycobacterium leprae involving primarily peripheral nerves and skin. Lepromatous leprosy is a severe form of leprosy characterized by generalized polymorphic lesions constituting macules, patches, papules, nodules and infiltrated lesions distributed symmetrically over face, trunk and extremities1. It is characterized by defective cell mediated immunity against lepra bacilli and hence involvement tends to be generalized. However it can have various variants like, lucio leprosy3 and histoid leprosy 4 or other atypical forms like urticaria wheal like 6, localized lesions 2 or autoaggressive hanseniasis5. We report a case of a lepromatous leprosy with an atypical presentation with lesions localized to flanks. Case report: A 50 year old male patient presented to skin OPD with multiple asymptomatic non progressive raised reddish skin lesions over the abdomen, localized to the flanks for the past 8 months.there was no history of hypopigmented hypoaesthetic patch, epistaxis, pedal edema or sensory disturbances either over the lesions or over the extremities. There was no history of systemic disturbances. General examination of the patient was normal. Dermatological examination revealed multiple erythematous, well to ill defined, infiltrated, shiny papules and plaques present over lateral aspect of the lower abdomen, bilaterally ( Fig 1& 2).

2 . No other skin lesions or trophic lesions were seen. Sensation over the lesions and over the extremities were unimpaired. Peripheral nerve examination was normal. Testicular and corneal sensation was normal. Motor system examination was normal. Investigations: Routine blood investigations like hemogram, liver and renal function tests were within normal limit. Slit skin smear was done from the papular lesions over both flanks and from the normal skin. Lesional skin revealed positive solid staining acid fast bacilli with the bacteriological index of 6+ (fig 3) and normal skin was negative for acid fast bacilli. Histopathology of the lesion showed thinned out epidermis, flattened rete ridges, subepidermal free zone (Grenz zone) and diffuse infiltration of the whole of dermis with foamy macrophages (fig 4& 5). Special stain with Fite Faraco showed multiple solid staining acid fast rods along with globi formation at few locations (fig 6). Based on clinical presentation and investigational reports diagnosis of lepromatous leprosy was made and patient was started on MB-MDT. Discussion: Lepromatous leprosy is a systemic disorder presenting with generalized skin lesions with multiple symmetrical, polymorphic cutaneous lesions along with ear lobe involvement, madarosis, leonine facies, gynaecomastia, epistaxis and bilateral pedal edema. Rarely it can present in various atypical forms like urticarial wheals like 6, adenoma sebaceoum like 7, mimicking sweet s syndrome 8, localized type 9, with bullous lesions during ENL reaction 10 or just with apparently normal appearing skin 11. These atypical forms result in diagnostic difficulty and hence delay in treatment. In our case though leprosy was our first diagnosis we also considered histoid variant, cutaneous B cell lymphoma and sarcoidosis for our differential diagnosis. These conditions though mimic leprosy clinically can be easily distinguished by slit skin smear and histopathological examination. Our case presented with erythematous infiltrated papules and nodules localized to flanks in contrast to previous unusual case report by Barman et al which had generalized asymmetrical lesions with type II lepra reaction 12. However there was no madarosis, ear lobe involvement or signs of sensory disturbances in our case. But based on investigations like slit skin smear which showed 6+ BI and histopathology and special stains a diagnosis of lepromatous leprosy was made. But the reason for this localization is not known.

3 Even though rare these atypical presentations have got a great epidemiological significance both in diagnosis and treatment selection. It also indicates the importance of performing smear examination in every suspected case of leprosy and histopathological examination where ever possible even in this post elimination era. Figure 3 Skin smear stained with modified acid fast stain showing multiple acid fast solid staining bacilli (magnification 100x) Figure 1 multiple, erythematous, infiltrated papules and plaques over right lower abdomen Figure 4 Histopathology of papular lesion showing thin epidermis, flat rete ridges, grenz zone and foamy macrophages (magnification 10x) Figure 2 multiple, erythematous, infiltrated papules and plaques over left lower abdomen

4 References: 1. Ridley DS, Jopling. W.H Classification of leprosy according to immunity. A five group system. Int. J. Lepr. 1966;34: Figure 5 - Histopathology of papular lesion showing thin epidermis, flat rete ridges, grenz zone and foamy macrophages (magnification 40x) 2. Yoder LJ, Jacobson RR, Job CK. A single skin lesion-an unusual presentation of lepromatous leprosy. Internationa Journal of Leprosy and Other Mycobacterial Diseases 1986;53: Pfaltzgraff RE, Bryceson A. Clinical leprosy. In: Hasting RC, Convit J, editors. Leprosy. Edinburgh: Churchill Livingstone; 1985.p. 153; Sehgal VN, Srivastava G. Histoid leprosy. Int J Dermatol 1985;24: Azulay RD. Hanseniasis; da immunobiologia a immunopathologia. Thesis. Rio de Janeiro, 1978 Figure 6 Special stain with Fite Faraco showing acid fast solid staining bacilli and globi formation (magnification 100x) 6. Barman KD, Gupta U, Saify K et al. Subpolar lepromatous leprosy presenting as urticarial wheals: a case report. Indian J Lepr 2004;76: Barman KD, Gupta U, Saify K et al. Lepromatous leprosy with unusual features resembling adenoma sebaceum. Indian J Lepr 2004;76:

5 8. Heng YK, Giam YC et al. Lepromatous leprosy in erythema nodosum leprosum reaction mimicking Sweet's syndrome. Int J Dermatol 2011; 50: Job CK, Kahkonen ME, Jacobson RR et al (1989). Single lesion subpolar lepromatous leprosy and its possible mode of origin. Int J Lepr Other Mycobact Dis 1989; 57: Ramesh V and Pahwa M (2010). Some unusual type 2 reactions in leprosy. Int J Dermatol 2010;49: Rea TH, Levan NE. Erythema nodosum leprosum in a general hospital. Arch Dermatol 1975;111: Barman KD, Goel K, Agarwal P et al. Lepromatous leprosy with an uncommon presentation: a case report. Indian J Lepr 2013;85:27-31.