Back ground: Histoid Hansen’s disease is a rare form of multibacillary leprosy whereas isolated mucosal histoid is rarer with distinct clinical and histopathological features. This type of leprosy is a variant of lepromatous leprosy with a very high bacterial reserve. Case Report: A 45 year old male was diagnosed as mucosal histoid lepromatous leprosy.It is a matter of concern as we found an isolated mucosal histoid leprotic lesion inside the nasal cavity in post-global leprosy elimination era. Our case had no history of leprosy or exposure to dapsone/ multidrug therapy, with heavy bacillary index. Discussion and Conclusion: We are reporting this case to highlight the rarity of mucosal lesion of histoid leprosy, nasal cavity involvement and to create awareness and so as to avoid misdiagnosis, which will help in prompt treatment so that we can minimize the complications and deformities of the patient and also prevent its spread in the community.
Keywords: Histoid hansen’s disease, leprosy, mucosal, nasal cavity
|How to cite this article:
Swain SK, Jena AK, Panda M, Mahapatra D. Isolated mucosal histoid hansen’s disease of nasal cavity in post elimination era. Ann Trop Med Public Health 2017;10:241-3
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Swain SK, Jena AK, Panda M, Mahapatra D. Isolated mucosal histoid hansen’s disease of nasal cavity in post elimination era. Ann Trop Med Public Health [serial online] 2017 [cited 2017 Jul 15];10:241-3. Available from: https://www.atmph.org/text.asp?2017/10/1/241/205551
Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. It is an infectious condition with high morbidity. Histoid leprosy is a rare variant of lepromatous leprosy with incidence varying from 1-2% among total leprosy patients and an average age at diagnosis is between 21 and 40 years. The adult population is most commonly affected with a male predominance. The bacillary load is high in these patients and usually occurs in the lepromatous type and in dapsone monotherapy and dapsone resistant cases. It is clinically characterized by multiple discrete, shiny, smooth, painless, globular, firm nodules and papules on the normal appearing skin. The lesions are usually located on the posterolateral aspects of arms, buttocks, thighs, dorsum of hands, and on the lower part of the back and over the bony prominences, especially over the elbows and knees., It is extremely rare in genital area, which occurs in more severe form. Aetiopathogenesis, that is, the cause and subsequent development of an abnormal condition is unclear. The characteristic histopathological findings with high bacillary index confirm the diagnosis in the clinically doubtful cases. As the bacillary load is very high in these patients, they can form a potential reservoir of the infection in the community. Mucosal type of histoid leprosy is extremely rare in community.
A 45 year old male was presented to ENT OPD with right nostril block since six months and intermittent nasal bleeding from same side. The patient had no rhinorrhea and anosmia. He did not mention any major disease suffering in the past and did not have history of drugs use as well. On inspection and anterior rhinoscopy, it was found that there was a small reddish mass in the anterior part of nasal cavity [Figure 1]. Diagnostic nasal endoscopy was done to confirm the lesion inside the nasal cavity and its site of attachment. The CT scan of nose and paranasal sinus was done to know the exact size and extent of the mass in the nose and paranasal sinuses. The scan showed a mass confined to anterior part of the nasal cavity, which was attached to septum and anterior part of inferior turbinate [Figure 2]. There was no history of sneezing and hyposmia and the ear and throat examination were found to be normal. Also, general physical examination, systemic examinations and routine blood tests were within normal limit. The mass was excised endoscopically under general anesthesia and sent for histopathological examination.
|Figure 1: Patient showing presence of mass at the anterior part of right nasal cavity
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|Figure 2: CT scan of nose and paranasal sinus (coronal cut) showing mass at the anterior part of right nasal cavity
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The biopsy was reported as a histoid type of lepromatous leprosy [Figure 3] and [Figure 4]. After unexpected histopathology report, more history was extracted. The patient did not have any familial background of the disease and did not mention any contact with any patient suffering from leprosy and also there were no cutaneous and peripheral nerve involvements. Then, the patient was referred to out patient department of dermatology for further evaluation and treatment of histoid lepromatous leprosy. After confirmation of the diagnosis the patient was started on anti-leprotic multibacillary therapy with rifampicin, clofazamine and dapsone. The patient was advised for regular follow up. In the past six months of treatment, the patient has responded well.
|Figure 3: Microphotograph shows round to spindle shaped cells arranged in sheets (H and EX400)
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|Figure 4: Microphotograph shows globi of lepra bacilli in the cytoplasm of histiocytes (Wade-Fite stainX400)
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Histoid leprosy was first reported in 1963 by Wade. It is an unusual multibacillary form of leprosy having unique clinical, histological, and bacteriological findings. Irregular and inadequate therapies as well as resistance to dapsone, and/or mutated organism (histoid bacillus) are responsible factors for causing histoid Hansen’s disease. But our case has no past history of leprosy or drug therapy for it. This form of Hansen’s disease has public health importance in this post elimination era as it belongs to the lepromatous pole with high infectivity and increased risk of bacillary transmission in the community. The histoid leprosy is considered as a variant of lepromatous leprosy and there exists an enhanced immune response against Mycobacterium leprae as compared to lepromatous leprosy with respect to both cell mediated immunity and humoral immunity. Although there are adequate number of macrophages, it has been claimed that they are unable to kill the bacilli that exists in high numbers in histoid lesion.
Histoid leprosy in nasal cavity may present with nasal obstruction, epistaxis, rhinorrhea, hyposmia etc. Evidences of nasal mucosa involvement are seen by anterior and posterior rhinoscopy. Diagnostic nasal endoscopy and radiological test like CT scan of nose and paranasal sinuses is a very useful tool to assess the lesion inside the nasal cavity.
Histopathological findings of the histoid nodules shows fusiform histocytes arranged in whorled, criss-cross or storiform pattern. Within the histiocytes, acid fast bacilli are seen in large numbers. The acid fast bacilli are longer than the normal bacilli, uniform in length, more solid and are arranged in parallel bundles along the long axis of the histiocytes. There is an epidermal atrophy and classical Grenz-zone just below the epidermis., There are three histological variants of histoid leprosy: Pure fusocellular, fusocellular with epitheloid component and fusocellular with vaculated cells. The third type of histopathological picture is most commonly seen. The mucosal histoid hansen clinically simulates different granulomatous lesions of nasal cavity like syphilis, tuberculosis, rhinosporidiosis, rhinoscleroma etc, and also inverted papilloma and malignancy in elderly patients. The management includes treating the patient with multidrug antileprosy regimen consisting of dapsone, clofazimine and rifampicin. Regular follow up is adviced in these patients for the complete treatment and preventing the spread of infection. In January 2007, India was declared to have eliminated leprosy. Mucosal histoid type serves as a reservoir of leprosy and as a source of new cases. This can create a serious threat to our elimination program.
The high bacillary load in the histoid variety is a potential reservoir of the infection. This rare mucosal lesion in the nasal cavity provides high chance of spread to others. It is essential to do surveillance for new cases and to give complete treatment so that to achieve our goal of elimination of leprosy. This case is unique in the sense that, this mucosal variety of histoid leprosy is extremely rare, and this patient has no history of leprosy or ingestion of dapsone/multidrug therapy. Histoid leprosy requires early detection and prompt treatment. These should be made a priority in the national program against leprosy.
Declaration of Patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for 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.
The authors would like to thank Prof. Manoj Ranjan Nayak, President and Er. Gapobandhu Kar, Managing Member of Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India for their constant encouragement.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]