Lepr Rev (2018) 89, 301 305 CASE REPORT Identicl twins with orderline lepromtous leprosy mimicking extensive lopeci ret: A rre presenttion RUBINA JASSI*, KRISHNA DEB BURMAN**, BHAVYA SWARNKAR** & RADHIKA AGARWAL** *All Indi Institute of Medicl Sciences, New Delhi, Indi **Muln Azd Medicl College, Bhdur Shh Zfr Rod, New Delhi, Indi Accepted for puliction 5 August 2018 Summry Nineteen-yer old identicl femle twins presented with complints of progressive hir loss ll over the ody, spring the sclp, xille nd puic region long with few trophic scrs on the knees, thighs nd elows. A possile dignosis of typicl presenttion of lopeci ret ws mde. However, in view of history of orderline tuerculoid leprosy in their younger siling, complete exmintion for leprosy ws done. There ws symmetricl enlrgement of multiple peripherl nerves without ny sensory or motor loss. Histopthology confirms the dignosis of BL Hnsens. Such presenttion of leprosy in identicl twins is not reported in literture. Keywords: identicl twins, leprosy, lopeci, contcts of leprosy Introduction Leprosy is disling infection cused y Mycocterium lepre, which my e cquired vi close contct with leprosy ptient. However, mnifesttions of the infection depend on multiple fctors like genetic susceptiility, stndrd of living, nutrition etc. A closed overcrowded environment long with similrity in genetic mke-up fvours disese spred mongst memers of the sme fmily. We report this cse of identicl twins with orderline lepromtous leprosy presenting s extensive generlised lopeci. These cses were erlier misdignosed s lopeci-ret. Cse Report Two identicl 19 yer-old twin sisters presented to our outptient deprtment with complints of progressive hir loss over the fce (including eyerows nd eyelshes), rms, trunk nd legs Correspondence to: Ruin Jssi, All Indi Institute of Medicl Sciences, New Delhi, Indi (e-mil: jssi. ruin@gmil.com) 0305-7518/18/064053+05 $1.00 q Lepr 301
302 R. Jssi et l. spring the sclp, xillry nd puic hir since the pst 1 1 = 2 yers simultneously. They lso hd 2 3 episodes of sudden onset of spontneous erosions on elows, knees nd thighs in the pst 1 1 = 2 yers which heled with trophy nd scrring. A possiility of typicl presenttion of lopeci ret ws kept ut in view of the history of orderline tuerculoid leprosy in the younger rother, thorough exmintion ws done to rule out Hnsen s disese. On exmintion, oth the ptients hd mdrosis (Figures 1, 1) with loss of hir ll over the ody except sclp, xill nd groin. The skin ppered shiny, however there ws no evidence of ny specific cutneous lesions suggestive of Hnsen s disese. Multiple trophic scrs of size 0 5 to 1 cm dimeter were present over the elows, knees nd thighs in oth the ptients (Figures 2, 2). Also, multiple peripherl nerves were enlrged symmetriclly in oth the silings. There ws no evidence of ny sensory loss or motor wekness. A slit skin exmintion ws done in oth the ptients, which showed Bcillry Index of 3þ from the eyerows. Skin iopsy done from the dorsum of hnd nd trophic scrs showed epiderml trophy with perivsculr, perippendegel nd perineurl collection of histiocytes, lymphocytes nd few epitheloid cells (Figures 3, 3). c d Figure 1. Diffuse infiltertion over the fce with mdrosis in oth the silings.
Twins with leprosy mimicking lopeci ret 303 Figure 2. Multiple heled trophic scrs of size 0.5 1 cm over the elows nd knees in oth the ptients. Figure 3. 10X H&E section of skin iopsy from dorm of hnd showing epiderml trophy with perivsculr, perippendgel nd perineuril lympho-histicytic infilterte with few epitheli cells.
304 R. Jssi et l. Figure 4. 40X Fite Frco stined section of the skin iopsy showing mycocterium lepre. Wde-fite stin ws strongly positive (Figures 4, 4). Thus, dignosis of orderline lepromtous leprosy ws mde nd oth the ptients were strted on WHO multicillry multi-drug therpy (MB-MDT). Discussion The presence of M. lepre cilli cuses infection ut not definitive disese. Its mnifesttion depends on the susceptiility of the individul nd the opportunity of contct with the microorgnism. 1 It hs een proposed tht pproximtely 90% of the generl humn popultion is inherently immune to Mycocterium lepre infections. 2 A very complex interply exists etween environmentl (microil nd non-microil) nd host (genetic nd non-genetic) fctors determine n individuls immunity to infection or its clinicl outcome. 3 Also, the spred of disese is either through droplet infection or y direct contct with skin. 4 So the risk of infection s well s disese increses with the presence of ny open cse of leprosy in the fmily or neighourhood. Attention hs een given to fmilil contcts prticulrly ecuse the fmily memers shre common genetic similrity, s well s the opportunity for prolonged close contct. 1 Leprosy my occur in memers of sme fmily. Since identicl twins shre similr genetics, the spectrum of leprosy is more likely to e sme. First cse report of leprosy in twins ws reported in 1939 y Ryrie. One of the children hd tuerculoid leprosy while the other ws lepromtous leprosy. But it did not mention if the twins were identicl or not. 5 In 1955, Brown et l. reported identicl twins dignosed with orderline tuerculoid leprosy. Bcteriologicl exmintion ws negtive ut skin iopsy reveled the dignosis. There ws strong positive history of leprosy in oth the pternl nd mternl side. 6
Twins with leprosy mimicking lopeci ret 305 Also leprosy is strong mimcker. The spectrum of the lesion morphology is vst. The lesions resemling nodulr suepiderml firosis, 7 denom seceum, 8 sweets syndrome 9 nd even urticril whels. 10 In our cse report, three out of five children of the sme fmily hd leprosy. The younger rother hd orderline tuerculoid leprosy while the two identicl twin sisters hd orderline lepromtous leprosy. Also, the clinicl presenttion in the two twins mimicked more like extensive lopeci ret (involving the eyerows, eyelshes nd ody hir). Suspicion of leprosy ws sed on strong fmily history nd histopthology confirmed the dignosis. Lstly, leprosy is gret mimicker. We should lwys keep our eyes nd mind open to dignose ny typicl presenttion of leprosy. References 1 Meht V, Vsnth V, Blchndrn C. Conjugl leprosy: A rrity. Dermtol Online J, 2010; 16: 15. 2 Trutmn JR. Epidemiologic spects of Hnsen s disese. Bull NY Acd Med, 1984; 60: 722 731. 3 Csnov JL, Ael L. Inorn errors of immunity to infection: the rule rther thn the exception. J Exp Med, 2005; 202: 197 201. 4 Silv VP, Fonsec HH, Sens MM, Bender AT. Indeterminte leprosy nd lepromtous index cse: four cses in the sme fmily. An Brs Dermtol, 2014; 88: 105 108. 5 Ryrie SA. Populr misconceptions of leprosy. Lepr Rev, 1939; 2: 123. 6 Brown JA, Stone MM. Tuerculoid leprosy in identicl twins. Lepr Rev, 1958; 29: 53 55. 7 Snchez J. Lepromtous leprosy with lesions resemling nodulr suepiderml firosis. Int J Lepr, 1965; 33: 179 185. 8 Brmn KD, Gupt U, Sify K et l. Lepromtous leprosy with unusul fetures resemling denom seceum. Indin J Lepr, 2004; 76: 359 360. 9 Rmesh V, Phw M. Some unusul type 2 rections in leprosy. Int J Dermtol, 2010; 49: 172 175. 10 Brmn KD, Gupt U, Sify K et l. Su- polr lepromtous leprosy presenting s urticril whels: cse report. Indin J Lepr, 2004; 76: 223 228.