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 .
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 .
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 .
skin biopsy revealed a granulomatous infiltration negative for acid - fast bacilli as well as leishmania donovan bodies [ figure 2 ] .
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 .
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 ) 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 ] .
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 .
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 .
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 , can not be accurately ascribed to in our case .
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 .
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 .
the reported specificity and sensitivity of rk 39 in visceral leishmaniasis is 97 - 100% and 81 - 100% in various studies .
he was , hence , diagnosed as a case of pkdl based on the above features and a positive pcr for leishmania .
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 , 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 .
 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 . 
Introduction
Case Report
Discussion