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Wade Histoid Leprosy masquerading as eruptive xanthomas

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Wade Histoid Leprosy masquerading as eruptive xanthomas
Puja Monga DDVL1, Vandana Mehta MD, DNB1, C Balachandran MD1, Mary Mathew MD2
Dermatology Online Journal 14 (8): 21

1. Department of Skin & STD, Kasturba Medical College, Manipal, Karnataka, India. vandanamht@yahoo.com
2. Dept of Pathology, Kasturba Medical College, Manipal, Karnataka, India



Introduction

Histoid Hansens is an uncommon variant of lepromatous leprosy with characteristic clinical, immunologic, and bacteriologic findings. Wade described this pattern in 1960 and 1963 in patients from the Phillipines who had been treated with sulfones for a short period [1]. Today we know that Wade leproma is a Hansen node presenting in bacilliferous patients. We report one such case of Histoid leprosy that was mistakenly treated as eruptive xanthomas until he presented to us.


Case History


Figure 1Figure 2
Figure 1. Scattered skin colored nodules on the trunk
Figure 2. Nodules on the eyebrow

Figure 3Figure 4
Figure 3. Multiple skin colored nodules on the leg
Figure 4. Nodules on the elbow with grouping

A 30-year-old male presented with a six-month history of asymptomatic skin colored nodules on the left eyebrow associated with madarosis. On further examination, there were similar lesions found on the upper and lower extremities and trunk with grouping of lesions on the knees and elbows (Figs. 1, 2, 3, 4). The patient's medical and family history was non-contributory and there was no history of any previous treatment taken. Before approaching us he was diagnosed as a case of multiple eruptive xanthomas and treated accordingly without any change in the lesional morphology. All his routine hematological and biochemical parameters were within normal limits; however, the slit skin smear for acid fast bacilli showed BI of 6+ and MI 3 percent. Biopsy from the nodule showed epidermal atrophy with a prominent grenz zone and sheets of spindle shaped histiocytes with pyknotic nuclei and foamy cytoplasm in the dermis (Fig. 5). Wade fite stain for lepra bacilli showed plenty of AFB (Fig. 6). Thus, based on the histopathological and clinical findings he was diagnosed as a case of Histoid variant of lepromatous leprosy.


Figure 5Figure 6
Figure 5. Photomicrograph showing presence of a macrophage granuloma in the dermis (x10)
Figure 6. Fite stain showing presence of numerous bacilli

Discussion

Histoid leprosy is a rare and uncommon variant of lepromatous leprosy with its incidence among Hansen patients being 2.79 percent to 3.6 percent [2]. It has a striking male preponderance. Initial reports of it were associated with dapsone resistance and with relapse after dapsone monotherapy, however this manifestation of leprosy has also been reported in patients in whom relapse occurred in the presence of supervised monthly dose of multidrug therapy and in patients without any treatment [3]. Ebenzer et al. described a patient who developed Histoid Hansens after seven years of supervised MDT [4].

Clinically it is characterized by multiple, smooth, painless, firm skin colored to yellow brown nodules on normal appearing skin. The nodules may involve face, back, limbs and bony prominences, as well as mucous membranes in severely affected patients. Classical histopathological findings include epidermal atrophy as a result of dermal expansion by the underlying leproma and an acellular band (Unna band) located immediately below the epidermis. The leproma consists of fusiform histiocytes arranged in a tangled or storiform pattern containing acid fast bacilli [5]. There are three histologic variants of Histoid Hansens: pure fusocellular, fusocellular with epitheloid component, and fusocellular with vacuolated cells. The third pattern is most commonly observed [6]. Bacilloscopy shows abundant bacilli with histoid habit or less frequently forming globis. The Fernandez-Mitsuda reaction is negative. Histoid Hansens clinically simulates dermatofibromas, xanthomas, neurofibromas, reticulohistiocytosis and cutaneous metastasis. Each of them can be differentiated from Hansens on the basis of the characteristic histopathology, absence of mycobacteria on slit skin smear and nerve thickening.

Our patient, had lesions typical of Histoid Hansens with SSS for AFB showing abundant bacilli. Nerve thickening was absent in our case which probably was the reason his diagnosis was missed and he was treated as xanthomas. He was started on WHO MB-MDT and is currently under follow up.

References

1. Wade HW. Histoid variety of lepromatous leprosy. Int J Leprosy 1963;31:129-42. PubMed

2. Seghal VN, Shrivastav G. Histoid leprosy: a prospective diagnostic study in 38 patients. Dermatologica 1988;177:212-17. PubMed

3. Shaw IN, Ebeneger G, Rao GS, Natarajan NM, Balasundram B. Relapse as Histoid leprosy after multidrug receiving therapy(MDT); report of three cases. Int J Lepr Other Mycobact Dis 2000;68:272-6. PubMed

4. Ebenezer GJ, Barkataki A, Job CK. Leprosy relapse presenting in histoid form after multidrug therapy. Br J Dermatol 1999;140:159-60. PubMed

5. Seghal VN, Shrivastav G. Histoid leprosy. Int J Dermatol 1985;24:286-92. PubMed

6. Weidmann MV, Argento CM, Garramuno R. Lepra histoire de Wade. Arch Argent Dermatol 2001;51:235-237.

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