Histoid hansen's with transepidermal elimination: Five cases

Supekar Bhagyashree Babanrao; Suyash Singh Tomar; Vaishali Hainath Wankhade; Lavanya Panindra; Rajesh P Singh; Dharitri Bhat

doi:10.4103/ijmy.ijmy_25_22

CASE REPORT

Year : 2022 | Volume : 11 | Issue : 2 | Page : 217-221

Histoid hansen's with transepidermal elimination: Five cases

Supekar Bhagyashree Babanrao¹, Suyash Singh Tomar², Vaishali Hainath Wankhade¹, Lavanya Panindra³, Rajesh P Singh¹, Dharitri Bhat⁴
¹ Department of Dermatology, Venereology, Leprosy, Government Medical College and Hospital, Nagpur, Maharashtra, India
² Department of Dermatology, Venereology, Leprosy, Mayo Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
³ Department of Dermatology, Venereology, Leprosy, Indira Gandhi Government Medical College, Nagpur, Maharashtra, India
⁴ Department of Pathology, Government Medical College and Hospital, Nagpur, Maharashtra, India

Date of Submission	20-Feb-2022
Date of Decision	24-Apr-2022
Date of Acceptance	17-Jun-2022
Date of Web Publication	14-Jun-2022

Correspondence Address:
Vaishali Hainath Wankhade
Department of Dermatology, Venereology, Leprosy, Government Medical College and Hospital, Nagpur, Maharashtra
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijmy.ijmy_25_22

Abstract

Histoid leprosy is an expression of multibacillary leprosy with unique histopathology and characteristic bacterial morphology. It is characterized by the occurrence of asymptomatic firm, dome-shaped succulent papulonodules on apparently normal skin, or subcutaneous tissues. There are very few reports of the detection of Mycobacterium leprae in the epidermis in leprosy, which may have a possible role in transmission. We report five de novo cases of histoid leprosy with transepidermal elimination.

Keywords: De novo, histoid leprosy, transepidermal elimination

How to cite this article:
Babanrao SB, Tomar SS, Wankhade VH, Panindra L, Singh RP, Bhat D. Histoid hansen's with transepidermal elimination: Five cases. Int J Mycobacteriol 2022;11:217-21

How to cite this URL:
Babanrao SB, Tomar SS, Wankhade VH, Panindra L, Singh RP, Bhat D. Histoid hansen's with transepidermal elimination: Five cases. Int J Mycobacteriol [serial online] 2022 [cited 2022 Jul 6];11:217-21. Available from: https://www.ijmyco.org/text.asp?2022/11/2/217/347513

Introduction

The term histoid was coined in the year 1960 by Wade in patients from the Philippines as a histological concept of bacillary-rich leproma composed of spindle-shaped cells in the absence of globi.^[1] Histoid leprosy mostly occurs after inadequate or irregular treatment or due to dapsone-resistant mutant strains but may occur de novo.^[2] Namisato et al. described “transepidermal elimination (TEE)” of Mycobacterium leprae in lepromatous leprosy, which may indicate an unusual role of the skin in the transmission of leprosy.^[3] There are scant reports of the detection of M. leprae in the leprous epidermis.^[4],[5]

Case Report

We report a series of five cases, who presented to us with similar complaints of a raised lesion over the face, trunk, and extremities. Other complaints included variable degrees of tingling and numbness of extremities in all cases. None of the cases had a history of similar lesions in any family members. All five patients denied a history of taking any treatment in the form of monthly packets for these complaints. On examination, all cases had multiple dome-shaped, succulent papules, and nodules on normal skin with predominant involvement of the face, abdomen, back, and extremities in all cases. There were few strikingly eroded lesions with central eschar over back and arms in all cases [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f, [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d, [Figure 4]e, and [Figure 5]a, [Figure 5]b, [Figure 5]c, [Figure 5]d. There was no sensory or motor deformity in any of the cases; however, all of them had thickening of bilateral ulnar, radial cutaneous, and common peroneal nerves on palpation. Slit-skin smears were positive in all the cases with Morphological Index(MI) ranging from 15% to 50% and Bacteriological Index(BI) ranging from 3 + to 5+ [Figure 1]e, [Figure 2]f, [Figure 4]f, [Figure 5]e, and [Figure 6]a. Histopathology with Fite-Faraco staining from a biopsy sample of nodular lesions was suggestive of histoid Hansen's disease. Skin biopsies taken from eroded lesions showed the presence of lepra bacilli in the epidermis confirming the diagnosis of TEE of bacilli on Fite-Faraco stain [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 6]b, [Figure 6]c, [Figure 6]d, [Figure 7]a, [Figure 7]b, [Figure 7]c, [Figure 7]d, [Figure 7], [Figure 7]f, [Figure 8]a, [Figure 8]b, [Figure 8]c, [Figure 8]d, [Figure 8]e, [Figure 9]a, [Figure 9]b, [Figure 9]c, [Figure 9]d, and [Figure 10]a, [Figure 10]b, [Figure 10]c, [Figure 10]d). The demographic, examination, and investigative profiles of all patients are shown in [Table 1]. All the cases were started on multibacillary multidrug therapy (MB-MDT) and asked to follow-up regularly.

Figure 1: (a-e) Multiple discrete dome-shaped firm succulent papulonodules (classical histoid nodules) over back (a and b). With eroded surface of nodules (black arrow) at places over the arm (c) and thigh (d). Slit-skin smear of the same case (BI 5 and MI 30% live bacilli) (e)

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Figure 2: (a-f) Classical histoid nodules with eroded nodules (black arrow) at places over the face (a), upper limbs (b and c), buttocks (d), lower limbs (e), and Slit skin smear (SSS) revealed BI 4 and MI 30%. (f)

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Figure 3: (a-c): Classical histoid nodules with eroded nodules (black arrow) at places over the face (a and b) and trunk (c and d)

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Figure 4: (a-f) Classical histoid nodules with additional umbilicated nodules (white arrow) at places over the cheek (a), forehead (b), ears (c), right arm (d), and left elbow (e). SSS revealed BI 3 and MI 20%.(f)

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Figure 5: (a-e) Clinical histoid papulonodules with eroded nodules (white arrow) at places over the face (a), ears (b), and bilateral upper limbs (c and d). SSS revealed BI 3 and MI 20%

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Figure 6: (a-d) SSS of case 3 revealed MI 15%, BI 3 (a), fusocellular variant of histoid Hansen's disease (H and E, ×10: b, ×40: c), with AFB in the dermis, grenz zone, and epidermis (FF stain × 40: d). AFB: Acid-fast bacilli

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Figure 7: (a-f) Fusocellular with vacuolated variant of Hansen's disease (H and E stain, ×10, ×40) (b) with AFB in the dermis (c and d) along with prickle cell layer and stratum corneum (e, f). (FF × 10 and × 40, black arrow). AFB: Acid-fast bacilli

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Figure 8: Fusocellular variant of histoid Hansen's disease (H and E, ×10: a, ×40: b) along with AFB in the dermis, dermoepidermal junction, and grenz zone (black arrow) (FF stain × 40: c-e)

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Figure 9: (a-d) Fusocellular with vacuolated variant of histoid Hansen's disease (H and E, ×10: a, ×40: b) with AFB arranged at grenz zone and epidermal layer (black arrow) (FF stain × 40: c and d). AFB: Acid-fast bacilli

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Figure 10: (a-d) Fusocellular variant of histoid Hansen's disease (H and E × 10: a, ×40: b) with AFB arranged in the upper and lower dermis and grenz zone (black arrow) (FF stain × 10: c, ×40: d). AFB: Acid-fast bacilli

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Table 1: Clinical characteristics, examination findings, and relevant investigations

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Discussion

Histoid leprosy is an expression of multibacillary (MB) leprosy with unique histopathological findings, characteristic bacterial morphology, and very high bacillary load. It is considered a clinical variant of lepromatous leprosy by some authors, whereas others considered it a distinct clinical entity. Histoid lesions may be occasionally seen with borderline lepromatous and indeterminate leprosy. In the Indian literature, histoid leprosy was found to constitute 1.8% of all leprosy cases and occurred de novo in 12.5% of cases.^[6] All five of our cases had de novo presentation of histoid leprosy. It is typically characterized by a firm, erythematous or copper-colored, hemispherical, dome-shaped, glistening shiny, and succulent papulonodules over apparently normal-looking skin. The sites of predilection are back, buttocks, face, extremities, and over bony prominences, especially around the elbows and knees, as seen in our all five cases. While papules are the most common presentation, cutaneous/subcutaneous nodules, plaques, and less frequently ulcerated, umbilicated, and xanthomatous lesions may occur.^[7] Okada et al. first observed lepra bacilli in epidermis and electron microscopy and proposed that dermal bacilli could be gradually transferred to the epidermal layers through the phagocytic activity of young basal cells, and finally eliminated, possibly from the intact skin.^[8] Langerhans cells in the epidermis are antigen-presenting cells (dendritic cells) that phagocytose microorganisms and particles thus may be responsible for TEE of lepra bacilli in lepromatous and histoid leprosy; thus it have a role in the course and clinical expression of the disease.^[9] Job and Chacko (1986) have demonstrated that 60% of untreated MB patients had bacilli in the keratin layer and 80% had M. leprae DNA in skin washings in his polymerase chain reaction studies.^[10] Namisato et al. were the first to report TEE in an untreated 35-year-old male lepromatous patient. He proposed that dense rapidly growing, granulomatous, and upper dermal infiltrate may result in partial excretion of the intradermal granulomas and bacilli and follicular epithelium, as a mechanism for TEE of bacilli.^[3] Ghorpade A (2008) have observed lepra bacilli in epidermal erosion on histopathology.^[4] Later in 2011, he first described TEE in histoid leprosy.^[11] He described the presence of numerous solid-staining acid-fast bacilli (AFB) arranged discretely and in clumps in the dermis, including the grenz zone, in vacuoles in the prickle cell layer and in the stratum granulosum as TEE, as observed in our all cases on histopathology. The presence of bacilli at various levels in the epidermis suggested their movement upward along with the epidermal cell maturation and their ultimate elimination from the stratum corneum into the environment, thus, it may have a possible role in the transmission of leprosy. The umbilicated/molluscoid lesion is a rare morphological variant of histoid leprosy. The Type 2 (pseudoisomorphic) Köebner's phenomenon has been reported in this form of histoid leprosy further substantiates the fact that viable bacilli must be eliminated transepidermally for this to occur clinically.^[11],[12] There are reports of initial single leprosy lesions developing at the site of tattooing, vaccination scar, or trauma suggesting that the bacilli could gain entry through traumatized skin. There is a paucity of literature describing TEE of lepra bacilli in histoid leprosy as they could be missed/underreported unless specifically looked for [Table 2]. Thus, we report this case series of histoid leprosy with TEE. The transepidermal exit of the M. leprae indicates that possibly skin could be a portal of both exit and entry for the bacillus and larger studies should be carried to establish its role in leprosy transmission.

Table 2: Review of the literature of TEE in leprosy

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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