Dermatology Online Journal

Rhinophymous leishmaniasis: A new variant
Arfan ul Bari MBBS MCPS FCPS¹, Amer Ejaz MBBS MCPS FCPS²
Dermatology Online Journal 15 (3): 10

1. Consultant Dermatologist, Combined Military Hospital, Peshawar, Pakistan
2. Consultant Dermatologist, Combined Military Hospital, Kharian, Pakistan. amer_ejaz@yahoo.com

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Abstract

Cutaneous leishmaniasis is known for its wide clinical spectrum. The nose is one of the usual sites where the disease can present in many forms, such as psoriasiform plaques, furunculoid nodules, lupoid plaques, and erysipeloid or mucocutaneous types. We present a new morphology, i.e. rhinophyma-like plaque in an elderly male patient who presented with a large infiltrated plaque involving his nose and the adjoining area of his upper lip. It appeared to be rhinophyma of the nose but was diagnosed as cutaneous leishmaniasis after the demonstration of leishmania parasites in a skin smear preparation; he was treated satisfactorily with antimonials.

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Introduction

Cutaneous leishmaniasis (CL) occurs both in the New World and the Old World [1]. Old World disease primarily is caused by Leishmania tropica in urban areas and Leishmania major in rural areas. The incubation period is two to eight weeks, although longer periods have been noted. The disease begins as an erythematous papule at the site of a sand fly bite on exposed parts of the body. The papule increases in size and becomes a nodule. It eventually ulcerates and crusts over. There may be multiple lesions, especially when the patient has encountered a nest of sand flies. Old World leishmaniasis lesions tend to heal spontaneously in months. After healing, a depressed scar remains that is usually round, but can be irregular. Satellite lesions with nodular lymphangitis resembling sporotrichosis have been described [1, 2, 3]. Cutaneous leishmaniasis can become disseminated (diffuse cutaneous leishmaniasis), especially in immunosuppressed persons. This illness can go on for years and does not heal spontaneously. Patients with human immunodeficiency virus (HIV) infection are particularly susceptible [4]. Other unusual types of cutaneous disease include leishmaniasis recidivans, in which small nodules develop around a healed scar, and post kala-azar dermal leishmaniasis, in which widespread cutaneous lesions arise after a visceral infection. The mucosal form usually occurs after an initial cutaneous infection and may progress to destruction of mucosa and even cartilage [2, 3, 4, 5]. Cutaneous leishmaniasis typically appears on exposed/uncovered body sites. Because the nose projects and is a non-motile part of face, it is most vulnerable for sand fly bites. Clinically, nasal lesions may be as minor as a small papule, nodule or psoriasiform plaque, which heal satisfactorily with out any complications. However, occasionally the lesions may behave aggressively and cause mutilation and destruction of nasal tissue, thus causing significant destruction [6, 7]. Herein, we present a case in which a large and horrifying example of cutaneous nasal leshmaniasis showed an excellent response to treatment with an antimonial compound (meglumine antimonate).

Case history

Figure 1
Figure 1. Erythematous infiltrated plaque of cutaneous leishmaniasis giving rise to enlarged and bulbous nose (rhinophyma-like)

A 73 year-old patient presented with an 8 month history of a progressive, painless swelling of his nose. It started as a small plaque near the tip of the nose that gradually extended to involve the entire nose along with a little adjoining area (Fig. 1). The patient's nose became heavy and bulbous but there were no associated symptoms. There was no history of nasal obstruction, rhinorrhea, or epistaxis. He had no history of facial flushing or rosacea. He was otherwise in a good state of health; laboratory examinations including complete blood count, liver function tests, renal function tests, and blood sugar levels were within normal limits. Chest X-ray was also unremarkable. Because the patient belonged to an endemic area of cutaneous leishmaniasis, he was suspected to have the disease in an unusual form and was subjected to cytodiagnostic tests for CL.

Figure 2	Figure 3
Figure 2. Skin slit smear showing intracellular and extracellular leishmania amastigotes (LT bodies) Figure 3. Fine needle aspiration cytology showing intracellular and extra cellular leishmania amastigotes (LT bodies)

Figure 4
Figure 4. Healing after 20 days treatment with meglumine antimonite

From the lesion on side of the nose, a skin slit smear was made and fine needle aspiration for cytological examination was also done. Both the tests were able to demonstrate leishmania parasites (LT bodies) (Figs. 2 & 3). Hence no further confirmation by skin biopsy or parasite culture was needed and intramuscular injections of meglumine antimonite (20 mg/kg/day) for 20 days were administered. He completed treatment without any adverse effects and his lesion healed completely after treatment (Fig. 4).

Discussion

The clinical picture in leishmaniasis depends not only on the infecting Leishmania species, but also on the host immune response, which is largely mediated through cellular immunity. Other factors that affect the clinical picture include the number of parasites inoculated, the site of inoculation and the nutritional status of the host. Patients that are non-indigenous, of older age, co-infected with human immune deficiency virus (HIV), using oral corticosteroids, or have experienced wound contamination with inorganic materials may show an altered clinical picture of CL [2, 4, 5]. Nasal cutaneous leishmaniasis has frequently been mentioned in the literature as a presentation of CL and involvement of nasal mucosa and nasopharynx has also been described as part of mucocutaneous leishmaniasis. Lesions of the mucocutaneous type range from simple edema to large disfiguring and nodulo-ulcerative plaques that can lead to nasal obstruction, epistaxis, and even septal perforation [2, 3, 6, 7]. Frequent nasal affliction in CL can possibly be explained by the fact that nose is the most exposed and unprotected part of the face.

Our patient presented with a large bulbous nose that appeared similar to rhinophyma, but considering that he did not have any previous history of rosacea, an alternative diagnosis of CL was considered; this was confirmed by slit smear. Mucocutaneous and lupoid nasal leishmaniasis can present with edematous or swollen nasal tissue, but an appearance resembling rhinophyma (enlarged bulbous nose) has not been reported to occur in CL. The precise pathogenesis of this rhinopymatous appearance is not well understood. However, the main factors could possibly be an altered cell-mediated immune host response to the leishmania parasite and the peculiar structural anatomy of nasal tissue. Moreover the laxity of the facial tissue and the great vascularization may enhance cutaneous expression of the Leishmania infestation, producing variable morphology [8]. The altered immune host response may be associated with defective macrophage function, altered production of lymphokines (especially interferon), decreased chemotaxis, increased delayed hypersensitivity, or imbalance between TH1 and TH2 responses [2, 9].

Previously the rhinophymatous presentation of leishmaniasis was only reported in association with post Kala azar dermal leishmaniasis [10]. Considering the increasing spectrum of CL in endemic areas, a high clinical suspicion for leishmaniasis should always be maintained by dermatologists and otolaryngologists when they encounter unusual lesions on the nose. A timely diagnosis can avoid complications and help institute early and effective treatment.

Conclusion

Nasal leishmaniasis is a common form of CL that can have a variety of appearances. Dermatologists and otolaryngologists should keep this disease in mind when examining lesions of the nose and nasal mucosa, especially in endemic regions. A timely diagnosis can avoid complications and help institute early and effective treatment.

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