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Cutaneous leishmaniasis affecting the face: Report of a case
1066
CUTANEOUS LEISHMANIASIS AFFECTING THE FACE
J Oral Maxillofac Surg 58:1066-1069, 2000
Cutaneous Leishmaniasis Affecting the Face: Report of a Case Michael Amin, FDS, FRCS,* and Mehmet Manisali, MSc, FFD, FRCS† Leishmaniasis is an intracellular infection caused by the protozoa Leishmania, which is transmitted by the sandfly. It occurs widely throughout Africa, Asia, South America, the Middle East, and the Meditteranean region. In humans, the disease can affect the skin, viscera, or mucocutaneous areas, varying according to the geographic region in which different Leishmania species are found and the host response. The life cycle of the parasite involves 2 stages: ●
●
The amastigote (Leishman-Donovan body) is present in hosts such as humans, dogs, and rodents. The parasite invades and multiplies in macrophages and other reticuloendothelial cells, which then rupture and release the organisms into the bloodstream. When the female sandfly bites an infected host, the amastigotes enter the sandfly and develop into the infective promastigotes. The cycle is completed when the sandfly bites another host.
In patients with a weak immune response, the reticuloendothelial system is laden with infected macrophages, whereas in subjects with an increased immune response, granulomas form in the lymph nodes, liver, and spleen, with few clinical symptoms. A localized lesion may form at the site of the insect bite. In endemic areas, permanent immunity develops to a particular species of leishmania, with inapparent infection being common. Because of the increase in both international travel and immigrant populations living in the United States and Western Europe, the numbers of reported cases
Received from the Norman Row Maxillofacial Unit, Queen Mary’s Hospital, Roehampton, London, England. *Specialist Registrar. †Senior Registrar. Address correspondence and reprint requests to Dr Amin: The Norman Rowe Maxillofacial Unit, Queen Mary’s Hospital, Roehampton Lane, Roehampton, London SW15 5PN, England; e-mail: [email protected] © 2000 American Association of Oral and Maxillofacial Surgeons
0278-2391/00/5809-0022$3.00/0 doi:10.1053/joms.2000.8755
of leishmaniasis is likely to increase. It is therefore important that oral and maxillofacial surgeons are able to recognize the cutaneous lesions caused by leishmaniasis and be aware of the appropriate management.1
Report of Case An 11-year-old girl from Afghanistan presented to our Oral and Maxillofacial Department in December 1998 with an 8-month history of a slowly enlarging, asymptomatic nodule on her upper lip and a similar lesion on the left wrist. The lesions did not bleed or cause any irritation. The patient, who spoke very little English, denied any history of insect bites or trauma and her medical history was unremarkable. Examination showed a healthy-looking girl with a 2 ⫻ 1.5-cm diameter, well demarcated, nodular lesion on her upper lip and a similar lesion on the lateral aspect of the left wrist (Figs 1, 2). The lesions were painless and had an erythematous, crusted, raised border and central ulceration. There was no cervical lymphadenopathy, and results of the systemic examination was normal, with no evidence of organomegaly or generalized lymphadenopathy. The hematologic and biochemcial studies were all within normal limits, with no evidence of immunosuppression. The differential diagnosis of the skin lesions, in view of the patient’s geographic origin, included leishmaniasis, sarcoidosis, tuberculosis, and leprosy. A punch biopsy of the lesions was performed under local anesthesia, and the samples were sent for microscopic examination and microbiologic culture. Microscopically, the lesion showed skin with hyperkeratosis, parakeratosis, and acanthosis. The dermis was filled with aggregates of large, pink, histiocytes, and mixed chronic inflammatory cells. The histiocytes contained dot-like organisms typical of cutaneous leishmaniasis (Figs 3, 4). Slit skin smears showed leishmania and the culture yielded promastigotes that were typed as Leishmania donovani. The patient was admitted to hospital for a 3-week course of daily intravenous infusions of sodium stibogluconate (Pentosam; Glaxo Wellcome UK, London, England). Five months after completion of treatment, the lesions showed some evidence of improvement (Fig 5). The patient remains under long-term follow-up, with further treatment planned with sodium stibogluconate.
Discussion Three clinical entities caused by leishmaniasis have been described: cutaneous, visceral, and mucocutaneous.2 Cutaneous leishmaniasis (oriental sore) is char-
AMIN AND MANISALI
FIGURE 1. The initial clinical presentation of the lesion on the lip.
acterized by single or multiple painless nodules that occur on exposed areas of the skin after the sandfly bite. These enlarge and ulcerate, with a characteristic erythematous raised border. An overlying crust may develop. The lesions heal slowly over months or years, sometimes leaving a disfiguring scar.3,4 Diagnostic investigations include: the Giemsa stain on a split skin smear, which demonstrates leishmania-infected macrophages; a positive Leishmanin skin test; a culture of the parasite; and isoenzyme or DNA studies.
FIGURE 2. Appearance of the lesion on the wrist.
1067 Treatment of small lesions may not be necessary. Larger lesions, or those in prominent areas of the body, can be treated locally with surgical excision, currettage, or cryotherapy. Medical management involves the parenteral use of pentavalent antimony compounds (eg, sodium stibogluconate) given for up to 21 days. Antibiotics are given for intercurrent infection. Patients with a poor response to the initial therapy are treated with intravenous amphotericin or pentamadine.2 Visceral leishmaniasis (Kala-azar) is caused by Leishmania donovani, and usually affects young people, often with an incubation period of months or even years. Clinical symptoms include a characteristic biphasic fever, cough, and diarrhea. Splenic enlargement occurs, which may be massive, and hypersplenism is chiefly responsible for the pancytopenia seen. If left untreated, death usually occurs within 2 to 3 years because of pulmonary or gastrointestinal superinfection. The organism can be cultured in the Nicolle-NovyMcNeal culture medium, and a full blood count shows anemia of chronic disease as well as a neutropenia and thrombocytopenia. Characteristic Leishman-Donovan bodies may be demonstrated in smears of the blood or bone marrow, as well as in aspirates of lymph node, liver, or spleen. Serologic tests also are available. The Leishmanin skin test is negative early in the course of the disease and is therefore not reliable in the initial diagnosis. Mucocutaneous leishmaniasis occurs in South America and is characterized by itchy, painful nodules on the lower limbs, which become ulcerated. Lymphangitis occurs, but healing usually occurs sponta-
FIGURE 5. The cutaneous lip lesion 5 months after treatment with sodium stibogluconate.
1068
CUTANEOUS LEISHMANIASIS AFFECTING THE FACE
FIGURE 3. Photomicrograph of a granuloma. (H&E stain, original magnification ⫻200.)
neously in a few months. In up to 40% of patients, secondary lesions develop several years later in areas such as the nasopharynx, with evidence of nasal obstruction, ulceration, septal perforation, and destruction of the nasal cartilages (Espundia). The diagnosis is confirmed with a punch biopsy of the lesion, but often only few leishmania are present. The leishmanin skin test is positive, and serum antibodies are present. Treatment is with systemic antimony compounds, but
FIGURE 4. Photomicrograph showing intracellular amastigotes. (Giemsa stain, original magnification ⫻400.)
relapse is common and secondary infection is the usual cause of death.2 The case presented showed the typical clinical and histologic features of cutaneous leishmaniasis. It is unusual in that the culture yielded Leishmania donovani rather than Leishmania tropica, which would have been a more likely cause of the cutaneous lesions in Afghanistan. There was no evidence to suggest that the patient was suffering from post kala-azar
SMITH, ARMSTRONG, AND DAVENPORT
dermal leishmaniasis, a condition that occurs in a small proportion of patients successfully treated for visceral leishmaniasis.5
References 1. Kubeyinje EP, Belagavi CS, Jamil YA: Cutaneous leishmaniasis in expatriates in northern Saudi Arabia. East Afr Med J 74:249, 1997
1069 2. Kumar P, Clark M: Clinical Medicine (ed 4). Philadelphia, PA, Saunders, 1998, pp 71-73 3. Funt T, McCormack P: Dermal plaques on the face of a child: Cutaneous Leishmaniasis (Leishmania tropical complex). Arch Dermatol 128:681, 1992 4. Hill PA: A case of granulomatous dermatitis: cutaneous leishmaniasis. Pathology 29:434, 1997 5. Ono H, Ghoreishi M, Yokozeki H, et al: A case of post-kala-azar dermal leishmaniasis. J Dermatol 25:118, 1998
CUTANEOUS LEISHMANIASIS AFFECTING THE FACE
J Oral Maxillofac Surg 58:1066-1069, 2000
Cutaneous Leishmaniasis Affecting the Face: Report of a Case Michael Amin, FDS, FRCS,* and Mehmet Manisali, MSc, FFD, FRCS† Leishmaniasis is an intracellular infection caused by the protozoa Leishmania, which is transmitted by the sandfly. It occurs widely throughout Africa, Asia, South America, the Middle East, and the Meditteranean region. In humans, the disease can affect the skin, viscera, or mucocutaneous areas, varying according to the geographic region in which different Leishmania species are found and the host response. The life cycle of the parasite involves 2 stages: ●
●
The amastigote (Leishman-Donovan body) is present in hosts such as humans, dogs, and rodents. The parasite invades and multiplies in macrophages and other reticuloendothelial cells, which then rupture and release the organisms into the bloodstream. When the female sandfly bites an infected host, the amastigotes enter the sandfly and develop into the infective promastigotes. The cycle is completed when the sandfly bites another host.
In patients with a weak immune response, the reticuloendothelial system is laden with infected macrophages, whereas in subjects with an increased immune response, granulomas form in the lymph nodes, liver, and spleen, with few clinical symptoms. A localized lesion may form at the site of the insect bite. In endemic areas, permanent immunity develops to a particular species of leishmania, with inapparent infection being common. Because of the increase in both international travel and immigrant populations living in the United States and Western Europe, the numbers of reported cases
Received from the Norman Row Maxillofacial Unit, Queen Mary’s Hospital, Roehampton, London, England. *Specialist Registrar. †Senior Registrar. Address correspondence and reprint requests to Dr Amin: The Norman Rowe Maxillofacial Unit, Queen Mary’s Hospital, Roehampton Lane, Roehampton, London SW15 5PN, England; e-mail: [email protected] © 2000 American Association of Oral and Maxillofacial Surgeons
0278-2391/00/5809-0022$3.00/0 doi:10.1053/joms.2000.8755
of leishmaniasis is likely to increase. It is therefore important that oral and maxillofacial surgeons are able to recognize the cutaneous lesions caused by leishmaniasis and be aware of the appropriate management.1
Report of Case An 11-year-old girl from Afghanistan presented to our Oral and Maxillofacial Department in December 1998 with an 8-month history of a slowly enlarging, asymptomatic nodule on her upper lip and a similar lesion on the left wrist. The lesions did not bleed or cause any irritation. The patient, who spoke very little English, denied any history of insect bites or trauma and her medical history was unremarkable. Examination showed a healthy-looking girl with a 2 ⫻ 1.5-cm diameter, well demarcated, nodular lesion on her upper lip and a similar lesion on the lateral aspect of the left wrist (Figs 1, 2). The lesions were painless and had an erythematous, crusted, raised border and central ulceration. There was no cervical lymphadenopathy, and results of the systemic examination was normal, with no evidence of organomegaly or generalized lymphadenopathy. The hematologic and biochemcial studies were all within normal limits, with no evidence of immunosuppression. The differential diagnosis of the skin lesions, in view of the patient’s geographic origin, included leishmaniasis, sarcoidosis, tuberculosis, and leprosy. A punch biopsy of the lesions was performed under local anesthesia, and the samples were sent for microscopic examination and microbiologic culture. Microscopically, the lesion showed skin with hyperkeratosis, parakeratosis, and acanthosis. The dermis was filled with aggregates of large, pink, histiocytes, and mixed chronic inflammatory cells. The histiocytes contained dot-like organisms typical of cutaneous leishmaniasis (Figs 3, 4). Slit skin smears showed leishmania and the culture yielded promastigotes that were typed as Leishmania donovani. The patient was admitted to hospital for a 3-week course of daily intravenous infusions of sodium stibogluconate (Pentosam; Glaxo Wellcome UK, London, England). Five months after completion of treatment, the lesions showed some evidence of improvement (Fig 5). The patient remains under long-term follow-up, with further treatment planned with sodium stibogluconate.
Discussion Three clinical entities caused by leishmaniasis have been described: cutaneous, visceral, and mucocutaneous.2 Cutaneous leishmaniasis (oriental sore) is char-
AMIN AND MANISALI
FIGURE 1. The initial clinical presentation of the lesion on the lip.
acterized by single or multiple painless nodules that occur on exposed areas of the skin after the sandfly bite. These enlarge and ulcerate, with a characteristic erythematous raised border. An overlying crust may develop. The lesions heal slowly over months or years, sometimes leaving a disfiguring scar.3,4 Diagnostic investigations include: the Giemsa stain on a split skin smear, which demonstrates leishmania-infected macrophages; a positive Leishmanin skin test; a culture of the parasite; and isoenzyme or DNA studies.
FIGURE 2. Appearance of the lesion on the wrist.
1067 Treatment of small lesions may not be necessary. Larger lesions, or those in prominent areas of the body, can be treated locally with surgical excision, currettage, or cryotherapy. Medical management involves the parenteral use of pentavalent antimony compounds (eg, sodium stibogluconate) given for up to 21 days. Antibiotics are given for intercurrent infection. Patients with a poor response to the initial therapy are treated with intravenous amphotericin or pentamadine.2 Visceral leishmaniasis (Kala-azar) is caused by Leishmania donovani, and usually affects young people, often with an incubation period of months or even years. Clinical symptoms include a characteristic biphasic fever, cough, and diarrhea. Splenic enlargement occurs, which may be massive, and hypersplenism is chiefly responsible for the pancytopenia seen. If left untreated, death usually occurs within 2 to 3 years because of pulmonary or gastrointestinal superinfection. The organism can be cultured in the Nicolle-NovyMcNeal culture medium, and a full blood count shows anemia of chronic disease as well as a neutropenia and thrombocytopenia. Characteristic Leishman-Donovan bodies may be demonstrated in smears of the blood or bone marrow, as well as in aspirates of lymph node, liver, or spleen. Serologic tests also are available. The Leishmanin skin test is negative early in the course of the disease and is therefore not reliable in the initial diagnosis. Mucocutaneous leishmaniasis occurs in South America and is characterized by itchy, painful nodules on the lower limbs, which become ulcerated. Lymphangitis occurs, but healing usually occurs sponta-
FIGURE 5. The cutaneous lip lesion 5 months after treatment with sodium stibogluconate.
1068
CUTANEOUS LEISHMANIASIS AFFECTING THE FACE
FIGURE 3. Photomicrograph of a granuloma. (H&E stain, original magnification ⫻200.)
neously in a few months. In up to 40% of patients, secondary lesions develop several years later in areas such as the nasopharynx, with evidence of nasal obstruction, ulceration, septal perforation, and destruction of the nasal cartilages (Espundia). The diagnosis is confirmed with a punch biopsy of the lesion, but often only few leishmania are present. The leishmanin skin test is positive, and serum antibodies are present. Treatment is with systemic antimony compounds, but
FIGURE 4. Photomicrograph showing intracellular amastigotes. (Giemsa stain, original magnification ⫻400.)
relapse is common and secondary infection is the usual cause of death.2 The case presented showed the typical clinical and histologic features of cutaneous leishmaniasis. It is unusual in that the culture yielded Leishmania donovani rather than Leishmania tropica, which would have been a more likely cause of the cutaneous lesions in Afghanistan. There was no evidence to suggest that the patient was suffering from post kala-azar
SMITH, ARMSTRONG, AND DAVENPORT
dermal leishmaniasis, a condition that occurs in a small proportion of patients successfully treated for visceral leishmaniasis.5
References 1. Kubeyinje EP, Belagavi CS, Jamil YA: Cutaneous leishmaniasis in expatriates in northern Saudi Arabia. East Afr Med J 74:249, 1997
1069 2. Kumar P, Clark M: Clinical Medicine (ed 4). Philadelphia, PA, Saunders, 1998, pp 71-73 3. Funt T, McCormack P: Dermal plaques on the face of a child: Cutaneous Leishmaniasis (Leishmania tropical complex). Arch Dermatol 128:681, 1992 4. Hill PA: A case of granulomatous dermatitis: cutaneous leishmaniasis. Pathology 29:434, 1997 5. Ono H, Ghoreishi M, Yokozeki H, et al: A case of post-kala-azar dermal leishmaniasis. J Dermatol 25:118, 1998