Atypical post kala azar dermal leishmaniasis with "muzzle area" swelling.

A 50-year-old male presented with recurrent swelling of the muzzle area of the face with history of low-grade intermittent fever of 3 year duration managed variously with antibiotics, systemic steroids, and antituberculous therapy. Skin biopsy revealed a granulomatous infiltration negative for acid-fast bacilli and leishmania donovan bodies. Immunochromatography test for rK 39 antigen and polymerase chain reaction for leishmania was positive. He was diagnosed as a case of post kala azar dermal leishmaniasis, managed with injection sodium stibogluconate and followed-up thereafter.

What was known?

Post Kala Azar Dermal Leishmaniasis is known to occur following Kala Azar in a subset of affected patients typically as early hypopigmented macules and later nodular lesions.

rK39 based tests serve as a valuable tool in diagnosis of visceral leishmaniasis.

Introduction

Post kala azar dermal leishmaniasis (PKDL), a fall out of visceral leishmaniasis, characterized by macular, maculopapular, and nodular eruption usually occurs in those residing in the endemic zone. Kala azar classically presents with chronic fever, splenomegaly, and hepatomegaly along with anemia, hyperpigmentation, cachexia, and even death in the chronically untreated. Rarely, the patient such as ours may be well-preserved during the primary illness and present only as PKDL to the treating physician. Isolated muzzle area involvement in such cases has not been described in literature.

Case Report

A 50-year-old male presented with history of recurrent perioral swelling for the past 1 and half years. He was asymptomatic for 1 and a half years back when he initially noticed a small swelling on the right side of upper lip which progressively involved the entire upper lip and later the peri-oral area, over the next 6 months [Figure 1], to remit partially on treatment with antibiotics only to relapse again. He was managed at various centers with doxycycline for rosacea, antituberculous therapy for 6 months for lupus vulgaris, as well as prednisolone for Melkerson Rosenthal syndrome. The differentials entertained as per his records were rosacea, lupus vulgaris, cheilitis granulomatosis, and Melkerson Rosenthal syndrome.

Figure 1

Well-defined swelling of the muzzle area with swelling of the lips (pretreatment) and posttreatment (inset)

There was no history of any skin rash elsewhere, obvious trauma at the site of initial swelling, photosensitivity, weakness of a side of the face, swelling tongue, or oral mucosa, cough, loss of weight, anorexia, or alteration of bowel habits. He, however, did give history of intermittent low-grade febrile illness, which would remit partially on antipyretics to relapse later, for the past 3 years. Examination at presentation to this center revealed swelling of lips with diffuse swelling involving the “muzzle area” of the face (lips and perioral area extending onto the cheeks on both sides − the equivalent region of the primate muzzle area of the face) [Figure 1]. The affected area was indurated and nontender. A few skin colored papules were present over the anterior aspect of the neck and right ear lobule. The oral mucosa, tongue, and the rest of the skin, hair, and nails were not involved. There was no regional lymphadenopathy.

Investigations revealed a normal hemogram and biochemical profile. X-ray chest and ultrasound scan abdomen did not reveal any abnormality. Enzyme-linked immunosorbent assay for human immunodeficiency virus infection was negative. Skin biopsy revealed a granulomatous infiltration negative for acid-fast bacilli as well as leishmania donovan bodies [Figure 2]. Immunochromatography test for rK39 was persistently positive. Polymerase chain reaction (PCR) for leishmania from the tissue sample was also positive. He was diagnosed as a case of PKDL, based on his clinical profile, skin histopathology of a granulomatous infiltration, positive rK39, and a positive PCR for leishmania.

Figure 2

Photomicrograph revealing granulomatous infiltration (H and E, ×10) (left) and (H and E, ×40) right

He was managed with injection sodium stibogluconate 850 mg intravenous (iv)[12] for 20 days a month for 4 months and followed-up thereafter. The swelling subsided over 3 months, he was there after followed-up for 6 months and has remained symptom-free [Figure 1-inset].

Discussion

Cutaneous leishmaniasis is endemic in 88 countries majority of which are developing countries. A total of 90% of mucocutaneous leishmaniasis occurs in Bolivia, Brazil, and Peru; 90% of cutaneous leishmaniasis cases occur in Afghanistan, Brazil, Iran, Peru, Saudi Arabia, and Syria and 90% of all cases of visceral leishmaniasis occur in Bangladesh, Brazil, Ethiopia, India, Nepal, and Sudan.[3]

PKDL is a late cutaneous manifestation. Most patients give a history of having suffered from or having been treated for kala azar, although this is not an absolute must for the diagnosis as was the case in our patient. Indian PKDL usually appears 2 years or more following recovery from visceral leishmaniasis. Our patient did not have documented visceral leishmaniasis though he did have intermittent febrile illness of 2 years duration which served as a clue to his illness. The duration or period of visceral leishmaniasis, hence, cannot be accurately ascribed to in our case.

Clinical manifestations in PKDL reveal variable morphological lesions. The early hypopigmented macules and the later nodular lesions are observed as well as verrucous, papillomatous, hypertrophic, and xanthomatous lesions are seen less frequently. Face involvement in PKDL may occur as hypopigmented lesions, indurated facial erythema, or in rhinophymatous variants.[45] Isolated muzzle area involvement has not been described as was seen in our case.

Diagnosis of PKDL is based on the appearance of lesions described above with a documented history of kala azar. Kala azar diagnosis needs demonstration of parasite in tissue smears or cultures. A positive rK39 test serves as a useful tool for diagnosis when other modalities of diagnosis are not possible.[6] The reported specificity and sensitivity of rK 39 in visceral leishmaniasis is 97-100% and 81-100% in various studies.[789] Our patient was unwilling for splenic or bone marrow aspiration. He tested positive for rK 39. Skin biopsy revealed a granulomatous infiltration. He was, hence, diagnosed as a case of PKDL based on the above features and a positive PCR for leishmania. He was managed with inj. sodium stibogluconate 850 mg iv for 20 days a month for 4 months. The diffuse swelling subsided over 3 months. He has been kept on follow-up.

An otherwise healthy male presenting with isolated partially remitting swelling over the perioral region extending onto cheeks giving an appearance of the primate muzzle, diagnosed as a case of PKDL on the basis of a positive PCR for leishmania and rK39 positivity are the highlights of the case. Although asymptomatic carriers exist in endemic areas,[10] presentation such as this has not been described. It also emphasizes the need for screening patients with obscure granulomatous infiltration with PCR as well rK39 to rule out leishmaniasis and PKDL.

What is new?

Post Kala Azar Dermal Leishmaniasis presenting as muzzle area swelling.