|Year : 2019 | Volume
| Issue : 2 | Page : 208-210
A dissimulate presentation of histoid Hansen's disease in the form of erythema nodosum leprosum
Vikas Pathania1, Bhavni Oberoi2, Sukriti Baveja1, Divya Shelly3, Ruby Venugopal1, Prerna Shankar4
1 Department of Dermatology, Command Hospital(SC), Pune, Maharashtra, India
2 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
3 Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India
4 Department of Community Medicine, Armed Forces Medical College, Pune, Maharashtra, India
|Date of Web Publication||14-Jun-2019|
Department of Dermatology, Command Hospital (SC), Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
Hansen's disease is a chronic infectious granulomatous disease with varied clinical presentation. Histoid Hansen's disease is an important emerging lepromatous subset of Hansen's disease known to mimic varied dermatoses. Occurrence of reactions, especially erythema nodosum leprosum (ENL), is rare in this form of leprosy. We report a case of Histoid Hansen's disease with initial presentation of ENL while undergoing management for infertility.
Keywords: Erythema nodosum leprosum, Histoid Hansen's disease, Type II Lepra reaction
|How to cite this article:|
Pathania V, Oberoi B, Baveja S, Shelly D, Venugopal R, Shankar P. A dissimulate presentation of histoid Hansen's disease in the form of erythema nodosum leprosum. Int J Mycobacteriol 2019;8:208-10
|How to cite this URL:|
Pathania V, Oberoi B, Baveja S, Shelly D, Venugopal R, Shankar P. A dissimulate presentation of histoid Hansen's disease in the form of erythema nodosum leprosum. Int J Mycobacteriol [serial online] 2019 [cited 2020 Feb 19];8:208-10. Available from: http://www.ijmyco.org/text.asp?2019/8/2/208/260378
| Introduction|| |
Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae bacillus. It can often have myriad and confusing presentation, especially to an unsuspecting and naïve physician, thereby delaying diagnosis and treatment.,, Histoid Hansen's disease is an important emerging subset of leprosy which with its dissimulate presentation poses a special diagnostic challenge for the clinician. It assumes even greater importance, especially in the national leprosy eradication era in being a reservoir of infection as a result of high lesional mycobacterial load. The diagnostic dilemma maybe further compounded when preceded by the rare occurrence of erythema nodosum leprosum (ENL) in this subset. We report a case of histoid Hansen's disease who was diagnosed incidentally when he reported with recurrent erythema nodosum-like lesions while undergoing evaluation for infertility.
| Case Report|| |
A 32-year-old male, resident of Bihar, undergoing evaluation for infertility at a tertiary care center was referred for a dermatological consult with complaints of transient red raised and tender lesions present over the right leg and abdomen which appeared spontaneously over a period of 5 days. The episode was associated with mild fever and malaise which was managed symptomatically with tablet paracetamol 650 mg. At presentation, the lesions had remitted leaving dark spots. Incidentally, during his evaluation for infertility, he was detected to be hepatitis B virus surface antigen positive. With the history of evanescent tender lesions, absence of any active lesion, and evidence of HBV infection, he was referred to a gastroenterologist for further management, who after evaluation concurred with the presumptive diagnosis of hepatitis B infection with erythema nodosum and prescribed him tablet tenofovir 300 mg once daily. Over the next 1 year, he continued to develop recurrent crops of similar lesions with spontaneous resolution and pigmentary sequelae with each episode. A year later, the patient was again given a dermatologic referral for chance detection of acid-fast bacilli (AFBs) in his semen as part of his infertility workup. On examination this time, there were multiple asymptomatic skin-colored nontender succulent papules and nodules over both the ears, dorsa of both the hands, and scrotum [Figure 1]. There were multiple faintly hypopigmented, norm esthetic patches over the back and proximal extremities tending to symmetry. There was also patchy hypesthesia in a glove and stocking distribution. Both ulnar, common peroneal and posterior tibial nerves were firm and thickened but nontender. On revisiting history, there was stuffiness of the nose along with three episodes of epistaxis over the past 1 month. On investigation, his baseline hematological and biochemical parameters were within normal limits. The lesional slit-skin smear (SSS) for AFBs (lepromatous) revealed a bacillary index of 6+. Histopathology of an excisional skin biopsy specimen from a representative papule over the dorsa of the left hand revealed a thin grenz zone with nodular aggregates of epithelioid cells and foamy histiocytes which were laden with stacks of long and slender AFBs on Ziehl–Neelsen stain [Figure 2]. Based on the history, clinical findings, AFBs on SSS, and characteristic histopathology, a diagnosis of Histoid Hansen's disease with recurrent ENL was made. In view of his chronic HBV coinfection, he was started on modified multidrug therapy (MDT) in the form of daily minocycline, ofloxacin, and clofazimine on an institutional basis. During his hospital stay, the patient developed fresh crops of red raised evanescent tender nodules over the trunk and all four extremities suggestive of Type II lepra reaction (ENL). Histopathological examination of an incisional skin biopsy from one of these lesions over the right thigh was consistent with features of ENL [Figure 3]. He was managed with tapering doses of capsule thalidomide 100 mg over 3 weeks. The patient continues to be on modified MDT and on regular uneventful follow-up with good compliance.
|Figure 1: Multiple asymptomatic skin-colored nontender succulent papules and nodules over both the ears (a). Scrotum (b) and dorsa of both the hands (c)|
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|Figure 2: Hematoxylin and eosin staining showing a narrow grenz zone with nodular aggregates of foamy histiocytes (×20) (a). Collection of epithelioid cells and foamy histiocytes (×40) (b). Ziehl–Neelsen stain showing stacks of slender acid-fast bacilli within these epithelioid cells (oil immersion) (c)|
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|Figure 3: Hematoxylin and eosin staining showing perivascular and periadnexal lymphocytic infiltrate (×20) (a) with septal panniculitis in the subcutis (×40) (b)|
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| Discussion|| |
Leprosy has been traditionally described as a chronic infectious disease of the peripheral nerves, skin, and reticuloendothelial system. Its cardinal features include erythematous or hypopigmented anesthetic patch and a thickened and/or tender cutaneous nerve. Histoid Hansen's disease is a subset of leprosy considered under the lepromatous spectrum and first described by Wade in 1960. Histoid leprosy comprises only 1.8%–2.9% of all leprosy cases and is more common in males with a male-to-female ratio ranging from 1.75:1 to 5.7:1., However, the initial belief of its occurrence following relapsed cases of dapsone monotherapy has since been debated with the rise in cases of “de novo” Histoid Hansen's disease in recent literature. It is speculated that focal loss of immunity is responsible in the unique morphological presentation of this entity.,, Our patient perhaps initially presented with Type II lepra reaction (ENL) while undergoing evaluation for infertility. The diagnosis of hepatitis B coinfection misled us to interpret the same as erythema nodosum. However, the chance finding of AFBs prompted a referral, by which time characteristic lesions, histopathology, and numerous AFBs on ZN stain confirmed the diagnosis. Subsequent biopsy of the ENL lesions was also consistent with Histoid Hansen's disease presenting with ENL. Although there are many isolated case reports, ENL occurs rarely in Histoid Hansen's disease.,, In a retrospective study of forty cases, Kaur et al. found that in patients of histoid leprosy who had ENL (40%), it invariably preceded the diagnosis by 1 month–2 years as was in our case. Our patient presented predominantly with papules over the ears, dorsa of the hands, and scrotum. While papules are the most common presentation, cutaneous/subcutaneous nodules and plaques and less frequently ulcerated, umbilicated, and xanthomatous lesions may occur which can mimic conditions such as neurofibromatosis, xanthomas, cutaneous sarcoidosis, post-kala-azar dermal leishmaniasis, secondary syphilis (papulonecrotic), and molluscum contagiosum. The most common sites to be affected are upper limbs, buttocks, trunk, and bony prominences with number of lesions varying from 3 to 50.,, Histopathology is characteristic with three variants, namely pure fusocellular, fusocellular with epithelioid component, and fusocellular with vacuolated cells which is the most common and was present in our case. Mean lesional bacillary index is consistently high and has been shown to range from 4.6 to 5.9 in different studies., AFBs are not arranged in globi here but rather stacked parallel to the long axis of histiocytes. Histopathologically, dermatofibromas, neurofibromas, and fibrohistiocytomas are common differentials to be excluded. Although there is no consensus in treatment schedule, multibacillary MDT (MB-MDT) is usually administered considering the lepromatous pole of the entity. Some workers have also advocated the use of rifampicin, ofloxacin, and minocycline prior to initiating MB-MDT., Our patient was treated with modified MDT (daily minocycline, ofloxacin, and clofazimine) in view of avoiding hepatotoxic drugs in a setting of hepatitis B coinfection. The case highlights the novel presentation of an atypical form of leprosy in the postelimination era and the danger it poses as a potential reservoir of the disease. Hence, in the National Leprosy Eradication Programme, voluntary reporting needs to be replaced with proactive surveillance for such cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]