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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 1  |  Page : 103-106

Are we witnessing a paradigm shift in leprosy: Two atypical presentations of histoid hansen's disease


1 Department of Dermatology, Command Hospital, Pune, Maharashtra, India
2 Department of Dermatology, Kirkee, Military Hospital, Ambala, Haryana, India
3 Department of Dermatology, Military Hospital, Pune, Maharashtra, India
4 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
5 Department of Community Medicine, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission12-Feb-2019
Date of Decision07-Dec-2019
Date of Acceptance16-Dec-2019
Date of Web Publication6-Mar-2020

Correspondence Address:
Vikas Pathania
Department of Dermatology, Command Hospital (SC), Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmy.ijmy_187_19

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  Abstract 


Hansen's disease is a chronic infectious granulomatous disease with varied clinical presentation. In the postelimination era, histoid Hansen's disease is an important emerging lepromatous subset known to mimic varied dermatoses, thereby making clinical diagnosis difficult and often delayed. We report two cases of histoid Hansen's disease bereft of clinical cardinal signs of leprosy.

Keywords: Atypical, Hansen's disease, histoid


How to cite this article:
Pathania V, Singh J, Sinha A, Kothari R, Baveja S, Shankar P. Are we witnessing a paradigm shift in leprosy: Two atypical presentations of histoid hansen's disease. Int J Mycobacteriol 2020;9:103-6

How to cite this URL:
Pathania V, Singh J, Sinha A, Kothari R, Baveja S, Shankar P. Are we witnessing a paradigm shift in leprosy: Two atypical presentations of histoid hansen's disease. Int J Mycobacteriol [serial online] 2020 [cited 2020 Apr 10];9:103-6. Available from: http://www.ijmyco.org/text.asp?2020/9/1/103/280144




  Introduction Top


Leprosy is a chronic granulomatous disease caused by Mycobacterium Leprae bacillus.[1] Every year, >200,000 leprosy cases are registered globally.[2] Histoid Hansen's disease is an important emerging subset of leprosy which with its varied morphological presentations poses a diagnostic challenge for the clinician.[3] It assumes even greater importance, especially in the postelimination era in being a reservoir of infection as a result of high lesional mycobacterial load.[4] As per the eighth meeting of the WHO expert committee on leprosy, a case of leprosy is defined as an individual who has not completed the course of treatment and has one or more of the three of the cardinal signs. These include hypopigmented or erythematous skin lesions with the loss of sensation, the involvement of peripheral nerves in the form of definite thickening with sensory impairment, and skin smear positive acid-fast bacilli (AFB).[5] We report two atypical cases of histoid Hansen's disease bereft of clinical cardinal signs of leprosy.


  Case Reports Top


Case 1

A 32-year-old otherwise healthy male, resident of Haryana, India, presented with a history of progressively increasing multiple raised asymptomatic reddish-to-skin-colored lesions on his abdomen over 9 months. He gave no history of light-colored numb patch or weakness of limb. On examination, he was found to have multiple, discrete, and grouped erythematous to dusky-red papulonodular lesions on the right side of the abdomen [Figure 1]. There were no other cutaneous lesions, hypoesthesia, or nerve thickening. Skin biopsy from one of the representative lesions revealed focal thinning of the lining stratified squamous epithelium with the underlying dermis showing splaying of the collagen and replacement by sheets of foamy histiocytes with periadnexal and perineural accentuation. Numerous foamy histiocytes were noted, with some of the histiocytes showing spindly appearance. Modified Ziehl–Neelsen (ZN) staining showed numerous lepra bacilli present singly and in clumps [Figure 2]. Slit-skin smear also showed multiple (AFB) with a bacillary index (BI) of 6+. In view of these findings, a diagnosis of histoid Hansen's disease was made, and the patient was started on WHO multibacillary (MB) multidrug therapy (MDT).
Figure 1: Multiple, discrete, and grouped lesions on the right side of the abdomen (a) which on closer examination revealed erythematous to dusky red papulonodules (b)

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Figure 2: Hematoxylin and eosin staining shows focal thinning of the lining stratified squamous epithelium with the underlying dermis showing splaying of the collagen and replacement by sheets of foamy histiocytes (×40) (a), with some of these histiocytes showing spindly appearance (×40). (b) Modified Ziehl–Neelsen staining showed numerous lepra bacilli present singly and in clumps (c)

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Case 2

A 30-year-old male, resident of Maharashtra, India, presented with multiple asymptomatic red raised lesions over the body of 8-month duration. The patient initially noticed an asymptomatic coin-sized red raised lesion over his right lower back 8 months back which progressively increased in size over the next 3 months. He also developed multiple red round swellings over this patch during the same period. Similar multiple pea-sized red raised lesions appeared over his hands and elsewhere on the body. He gave no history of numbness over these lesions or any accompanying constitutional symptoms. The patient consulted multiple practitioners during this time and was evaluated for sarcoidosis, leishmaniasis, deep fungal infections, and cutaneous tuberculosis. However, no definitive treatment was started, and he was managed symptomatically without any relief. At presentation, his general physical and systemic examination was essentially normal. Dermatological examination revealed the involvement of lower back in the form of two well-defined erythematous norm-esthetic plaques, the larger plaque measuring 20 cm × 15 cm over the right flank and was studded with multiple erythematous papulonodular lesion. Similar such lesions were present over the pinna of both the ears, extensor aspect of both the forearms, and dorsum of both the hands [Figure 3]. There were no thickened peripheral nerves or hypoesthetic patches. Lesional slit-skin smear revealed numerous AFB with a BI of 4+by a modified ZN stain. Histopathological examination of skin biopsy taken from a representative lesion over the right side back showed flattened epidermis with loss of rete pegs, with papillary and reticular dermis replaced by sheets of scattered histiocytes without the formation of granulomas. Numerous solid-staining bacilli were present in clusters and globi [Figure 4]. He was diagnosed as case of histoid Hansen's disease and was started WHO MB-MDT with regular counseling and health education of the patient and his contacts to ensure adherence to the treatment. He responded well with near-complete resolution of all plaques and nodules within 3 months of MDT [Figure 5].
Figure 3: Extensive involvement of lower back in the form of two well-defined erythematous plaques studded with multiple erythematous papulonodular lesions (a). Similar such lesions over the dorsum of both the hands (b)

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Figure 4: H and E staining shows flattened epidermis with loss of rete pegs (×20) (a), with papillary and reticular dermis replaced by sheets of scattered periadnexal foamy histiocytes (×40) (b). Numerous solid-staining bacilli were present in clusters and globi (c)

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Figure 5: Near-complete resolution of lesions over the lower back (a) and over the dorsum of both the hands (b)

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  Discussion Top


Leprosy has been traditionally described as a chronic infectious disease of peripheral nerves, skin, and reticuloendothelial system, clinically presenting with hypopigmented or erythematous skin lesions with the loss of sensation with or without peripheral nerve thickening.[3] Postelimination era has witnessed many atypical presentations of leprosy including lepra reactions and histoid Hansen's disease.[6],[7] Histoid Hansen's disease is a subset of leprosy considered under the lepromatous spectrum and first described by Wade in 1960.[8] 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.[9],[10] 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 cases in the recent literature. It is speculated that the focal loss of immunity is responsible in the unique morphological presentation of this.[11],[12],[13] Both our patients presented with polymorphic lesions in the form of plaques and papulonodular lesions over the trunk and also as discreet papules elsewhere over the body. There were, however, no hypoesthetic or erythematous lepromatous patches or any peripheral nerve thickening in either of the cases. While papules are the most common presentation, cutaneous/subcutaneous nodules, and plaques and less frequently ulcerated lesions can be seen; further illustrating attempts by the cell-mediated immunity in perhaps isolating lepra bacilli from spreading elsewhere. Umbilicated and xanthomatous lesions may occur, all of which may 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 the upper limbs, buttocks, trunk, and bony prominences, with the number of lesions varying from 3 to 50. While concomitant existence of classical hyposthetic lepromatous lesions may be present, the co-existence of thickened peripheral nerves has almost always been documented in the literature, with the ulnar nerve being most commonly involved.[3],[9],[10] Histopathology is characteristic with three variants namely, pure fusocellular, fusocellular with an epithelioid component, and fusocellular with vacuolated cells which is the most common and was present in both our cases. The mean lesional BI is consistently high and has been shown to range from 4.6 to 5.9 in different studies. AFB are not arranged in globi here but rather stacked parallel to the long axis of histiocytes.[9],[10] Histopathologically, dermatofibromas, neurofibromas, and fibrohistiocytomas are common differentials to be excluded.[3],[12] Although there is no consensus in the treatment schedule, 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.[3],[10] Both our patients were treated with WHO MB-MDT. There is also a need for repeated counseling and health education of patients to ensure adherence to MDT and avert psychological morbidity, which was done for both the cases.[14] The case highlights the atypical presentation of a rare but emerging form of leprosy in the postelimination era and the imminent danger that it poses as a potential reservoir of the disease.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Joel Chandranesan AS, Mada PK, Ramos-Herberth F, Walsworth D, Penn R, Washburn R. Leprosy in Northwest Louisiana: A case series. Int J Mycobacteriol 2018;7:173-7.  Back to cited text no. 1
    
2.
Naaz F, Mohanty PS, Bansal AK, Kumar D, Gupta UD. Challenges beyond elimination in leprosy. Int J Mycobacteriol 2017;6:222-8.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Gupta SK. Histoid leprosy: Review of the literature. Int J Dermatol 2015;54:1283-8.  Back to cited text no. 3
    
4.
Palit A, Inamadar AC. Histoid leprosy as reservoir of the disease; a challenge to leprosy elimination. Lepr Rev 2007;78:47-9.  Back to cited text no. 4
    
5.
WHO Expert Committee on Leprosy : 8th Report. Geneva: World Health Organization; 2012. p. 61.  Back to cited text no. 5
    
6.
Pathania V, Shelly D, Shankar P, Matharu YS, Baveja V. Two atypical presentations of lepra reactions. Int J Mycobacteriol 2018;7:390-3.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
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.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Wade HW. The histoid leproma. Int J Lepr Other Mycobact Dis 1960;28:3.  Back to cited text no. 8
    
9.
Kaur S, Dhar S, Sharma VK, Ray R. Erythema nodosum leprosum in a case of histoid leprosy. Int J Lepr Other Mycobact Dis 1993;61:292-3.  Back to cited text no. 9
    
10.
Mathur M, Jha A, Joshi R, Wagle R. Histoid leprosy: A retrospective clinicopathological study from central Nepal. Int J Dermatol 2017;56:664-8.  Back to cited text no. 10
    
11.
Pandey P, Suresh MS, Dey VK. De novo Histoid Leprosy. Indian J Dermatol 2015;60:525.  Back to cited text no. 11
[PUBMED]  [Full text]  
12.
Pathania V, Kumar A, Hashmi SA, Shergill SP. A case of 'de novo' Histoid Hansen's disease. Med J Armed Forces India 2015;71:S52-4.  Back to cited text no. 12
    
13.
Bhat YJ, Hassan I, Yaseen A, Wani R. De novo histoid leprosy: A case report from a post-elimination area. Indian J Dermatol 2015;60:214.  Back to cited text no. 13
[PUBMED]  [Full text]  
14.
Govindharaj P, Srinivasan S, Darlong J. Perception toward the disease of the people affected by leprosy. Int J Mycobacteriol 2018;7:247-50.  Back to cited text no. 14
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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