Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1219  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 88-90
Atypical post kala azar dermal leishmaniasis with ''Muzzle area'' swelling


1 Department of Skin, Army College of Medical Sciences and Base Hospital, Cantt, New Delhi, India
2 Department of Pathology, Army College of Medical Sciences and Base Hospital, Cantt, New Delhi, India
3 Military Hospital, Dehradun, Uttarakhand, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Sandeep Arora
Skin Centre, Base Hospital, New Delhi Cantt - 110 010, Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147809

Rights and Permissions

   Abstract 

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.


Keywords: Post kala azar dermal leishmaniasis, rK39 leishmania antigen, sodium stibogluconate


How to cite this article:
Arora S, Bal AS, Baveja S, Sood A, Rathi KR, Patil P. Atypical post kala azar dermal leishmaniasis with ''Muzzle area'' swelling. Indian J Dermatol 2015;60:88-90

How to cite this URL:
Arora S, Bal AS, Baveja S, Sood A, Rathi KR, Patil P. Atypical post kala azar dermal leishmaniasis with ''Muzzle area'' swelling. Indian J Dermatol [serial online] 2015 [cited 2020 Feb 21];60:88-90. Available from: http://www.e-ijd.org/text.asp?2015/60/1/88/147809

What was known?

  1. 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.
  2. rK39 based tests serve as a valuable tool in diagnosis of visceral leishmaniasis.



   Introduction Top


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 Top


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)

Click here to view


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

Click here to view


He was managed with injection sodium stibogluconate 850 mg intravenous (iv) [1],[2] 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 Top


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. [4],[5] 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. [7],[8],[9] 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.

 
   References Top

1.
Arora PN, Arora S. Diseases caused by parasitic worms and protozoa. In: Valia RG, Valia A, editors. Text Book of Dermatology. 3 rd ed, vol. 1. Bombay: Bhalani Publishing House; 2008. p. 432-89.  Back to cited text no. 1
    
2.
National Vector Borne disease control program, Directorate General of Health Services, Ministry of Health and Family Welfare. Guidelines-Diagnosis-Treatment-Kala Azar. Available from: http://nvbdcp.gov.in/Doc/Guidelines-Diagnosis-Treatment-KA.pdf [Last accessed on 2013 Mar 18].  Back to cited text no. 2
    
3.
WHO. Leishmaniasis. Burden of disease. Available from: http://www.who.int/leishmaniasis/burden/en [Last accessed on 2013 Mar 18].  Back to cited text no. 3
    
4.
Sultana A, Zakaria SM, Bhuiyan SI, Habib A, Dey SK, Rahman M, et al. Spectrum of skin lesions of post-kala-azar dermal leishmaniasis in kala-azar endemic areas of Bangladesh. Mymensingh Med J 2012;21:529-32.  Back to cited text no. 4
    
5.
Ul Bari A, Ejaz A. Rhinophymous leishmaniasis: A new variant. Dermatol Online J 2009;15:10.  Back to cited text no. 5
    
6.
Singh D, Pandey K, Das VN, Das S, Verma N, Ranjan A, et al. Evaluation of rK-39 strip test using urine for diagnosis of visceral leishmaniasis in an endemic region of India. Am J Trop Med Hyg 2013;88:222-6.  Back to cited text no. 6
    
7.
ter Horst R, Tefera T, Assefa G, Ebrahim AZ, Davidson RN, Ritmeijer K. Field evaluation of rK39 test and direct agglutination test for diagnosis of visceral leishmaniasis in a population with high prevalence of human immunodeficiency virus in Ethiopia. Am J Trop Med Hyg 2009;80:929-34.  Back to cited text no. 7
    
8.
Ritmeijer K, Melaku Y, Mueller M, Kipngetich S, O'keeffe C, Davidson RN. Evaluation of a new recombinant K39 rapid diagnostic test for Sudanese visceral leishmaniasis. Am J Trop Med Hyg 2006;74:76-80.  Back to cited text no. 8
    
9.
Sundar S, Maurya R, Singh RK, Bharti K, Chakravarty J, Parekh A, et al. Rapid, noninvasive diagnosis of visceral leishmaniasis in India: Comparison of two immunochromatographic strip tests for detection of anti-K39 antibody. J Clin Microbiol 2006;44:251-3.  Back to cited text no. 9
    
10.
Hasker E, Kansal S, Malaviya P, Gidwani K, Picado A, Singh RP, et al. Latent infection with leishmania donovani in highly endemic villages in Bihar, India. PLoS Negl Trop Dis 2013;7:e2053. Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3573094 [Last accessed on 2013 Mar 19].  Back to cited text no. 10
    

What is new?
Post Kala Azar Dermal Leishmaniasis presenting as muzzle area swelling.


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,243 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2043    
    Printed37    
    Emailed1    
    PDF Downloaded71    
    Comments [Add]    

Recommend this journal