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: 3629  
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 
E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 1  |  Page : 85
Morphea simulating paucibacillary leprosy clinically and histopathologically


1 Department of Dermatology, Mário Kröeff Hospital, Associação Pele Saudável, Rio De Janeiro, Brazil
2 Section of Dermatopathology At Anatomical Lab, Rio De Janeiro, Brazil

Date of Web Publication31-Dec-2012

Correspondence Address:
José Saulo Torres Delgado
Serviço de Dermatologia, Hospital Mário Kröeff, Associação Pele Saudável, Rua Magé 326, Penha Circular, Rio de Janeiro - RJ - CEP 21020-130
Brazil
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.105325

Rights and Permissions

   Abstract 

Clinically and histopathologically paucibacillary leprosy shows similar features with initial morphea. In this case we report a 24 yr-old male patient who presented to our dermatology department with diagnosed paucibacillary leprosy by his local dermatologist, and confirmed by perineurovascular lymphocytic infiltrate in the histopathological exam. On physical examination we found new plaque lesions that were suggestive of morphea with alteration of sensitivity. A new biopsy was performed showing sclerotic superficial dermis with thickening of the collagen bundles in deep dermis and linear arrays lymphocytic infiltrate between the collagen bundles that confirm the diagnosis of morphea.


Keywords: Morphea, paucibacillary leprosy, perineurovascular lymphocytic infiltrate


How to cite this article:
Delgado JT, Cavalcanti ML, Kac BK, Pires CL. Morphea simulating paucibacillary leprosy clinically and histopathologically. Indian J Dermatol 2013;58:85

How to cite this URL:
Delgado JT, Cavalcanti ML, Kac BK, Pires CL. Morphea simulating paucibacillary leprosy clinically and histopathologically. Indian J Dermatol [serial online] 2013 [cited 2019 Nov 13];58:85. Available from: http://www.e-ijd.org/text.asp?2013/58/1/85/105325

What was known?
Leprosy is a disease that shares some characteristics with multiple skin disorders; the early stage of morphea is one of those disorders with not only clinical but also histopathological similarity.



   Introduction Top


Through the history, the true diagnosis of leprosy has always been a challenge, not only for the multiple diseases that shares some clinical features but also because the similarity of a few in the histopatological aspect. We report in here a case of morphea showing very similar characteristics in the clinical and histopatological findings with paucibacillary leprosy, we also discuss the different aspects between the two entities and finally we focus in an atypical pattern of infiltration in morphea.


   Case Report Top


A 24 yr-old male presented to our dermatology department complaining of hypochromic plaques in his abdomen and neck.

In November of 2009, he consulted for a solitary oval hypochromic plaque with reddish-edge and smooth surface in the abdomen with absent of hair inside the plaque [Figure 1].
Figure 1: Oval hypocromic plaque with lilac - edge and smooth surface in the abdomen with absent of hair inside the plaque. Notice the scar of the first biopsy done from the edge of the lesion

Click here to view


A biopsy was performed in the edge of the lesion under suspicion of paucibacillary leprosy. The result of the biopsy showed perineurovascular lymphocytic infiltration [Figure 2]. A bacilloscopy was performed with negative result.
Figure 2: Perineurovascular and intersticial infiltrate predominantly lymphocytic, with linear arrays between the collagen bundles (H and E, ×100)

Click here to view


The patient was diagnosed with paucibacillary leprosy and treatment was established.

The patient received treatment for five months with rifampicin 600 mg/monthly and dapsone 100 mg/daily without improvement. In the fifth month of treatment the patient developed a satellite lesion with similar characteristics and another plaque with the same morphology in the neck, reason that made him consult again.

On physical examination the main lesion was an oval indurated hypochromic plaque with reddish-edge and smooth surface in the abdomen with some hairless areas inside the plaque and no alteration of sensibility. In the upper part of the main lesion there was a smaller hypocromic plaque with lilac-colored edge and no hair inside, did not have alteration of sensibility either; in the neck there was another hypochromic plaque with light erythematous edge and scaly surface with alteration of sensibility.

A new biopsy from the center of the initial plaque was performed suspecting localized morphea. The result showed subepidermic hyalinized collagen with loss of epidermal rete ridge pattern and some dilated superficial venules in the superficial dermis; there was atrophy of the adnexal structures. In the middle dermis there was a lymphocytic infiltrate with linear arrays between the thickened collagen bundles that enclose some ducts and eccrine glands [Figure 3].
Figure 3: There is a hyalinized superficial dermis with thickened collangen bundles in the mid and deep reticular dermis; notice the absence of adnexal structures (H and E, ×100)

Click here to view


Those findings in the histopatological exam confirmed the diagnosis of morphea.


   Discussion Top


Morpheae is a disorder of unknown cause in which there is localized sclerosis of the skin. [1] The etiology appears to be involved with the fibroblastic cells in which alterations in the grow factors (platelet-derived growth factor) [2] and receptor expression (TGF-β) [3] have been reported in in-vitro studies. Those alterations appear to lead to increased connective tissue growth factor (CTGF) gene expression and finally fibrosis. [4],[5] Immunological cytokines, auto-immune, trauma, immobility, radiotherapy, hormonal and infection etiology mainly with Borrelia burgdorferi also have been reported. [6]

Clinically the plaque lesions are indurated purplish or mauve in color. After some months they become thickened and and a characteristic lilac-colored edge develops. Frequently they present diminished sensibility and they can be bilateral multiple and asymmetric. [1],[6]

Histopathological features depend the stage of the lesion and the site of the biopsy. Biopsies done in the periphery of the lesion will show markedly lymphocytic and histiocytic inflammatory infiltrate scattered in the middle dermis. In the lower part of the dermis and subcutaneous tissue, it begins to appear as broadening of the collagen bundles with diminished interbundle spaces. [6],[7]

When the disease progresses, the inflammatory infiltrate slowly starts to disappear and is replaced by hyalinized connective tissue. The atrophic adnexal structures diminish in number until they disappear. [6],[7],[8],[9] The sweat glands generally are located in the middle of the sclerotic collagen bundles in the middle and deep dermis. [6],[8]

In Brazil, leprosy is a frequent infectious disease and is first impression differential diagnosis in several diseases.

Clinically patients with leprosy can be in two opposite sides, the paucibacillary (1-5 lesions) or the multibacillary (more the 5 lesions) subtype, reflective of the host immune response; the paucibacillary subtype is characterized by a predominantly Th1 cell-mediated immune response and the multibacillary subtype is characterized by a predominantly Th2 humoral response. [10]

The paucibacillary subtype is composed by the initial indeterminate leprosy and the tuberculoid leprosy. [11] The initial indeterminate lesions consist of hypopigmented macules with not well defined borders, generally without involving hair growth and nerve function. [12] In the histopathological findings there is a perineurovascular and adnexal non-specific infiltrate composed predominantly of lymphocytes. [13]

Tuberculoid lesions present as a plaque that is frequently solitary, with central hypopigmentation and raised erythematosus edge, the surface is sometimes scaly hairless and with alteration of sensibility. [11],[12] In the histopathological exam it presents with non-caseating granulomas composed of epitheliod cells, lymphocytes and Langhans cells. [13]

In our case the initial histopathological findings showing non- specific perineurovascular infiltrate directed to the diagnosis of paucibacillary leprosy. In the literature review there was no evidence of such pattern of infiltration in morphea but with the lack of response to the treatment and the appearance of new lesions the diagnosis of morphea became more likely.

It was interesting to note the localization of the lymphocytic infiltrate around the nerve and the linear disposition of the lymphocytes that haven't been reported until now. We suggest this could be another pattern of infiltration in morphea and there should be new studies to confirm this theory.

 
   References Top

1.Goodfield MJ, Jones SK, Veale DJ. The 'Connective Tissue Diseases', In: Rook/Wilkison/Ebling textbook of dermatology. 8 th ed. New York: Blackwell Science; 2010. p. 51.64-76.  Back to cited text no. 1
    
2.Zheng XY, Zhang JZ, Tu P, Ma SQ. Expression of platelet-derived growth factor Bchain and platelet-derived growth factor β-receptor in fibroblasts of scleroderma. J Dermatol Sci 1998;18:90-7.  Back to cited text no. 2
[PUBMED]    
3.Kubo M, Ihn H, Yamane K, Tamaki K. Up-regulated expression of transforming growth factor β receptors in dermal fibroblasts in skin sections from patients with localized scleroderma. Arthritis Rheum 2001;44:731-4.  Back to cited text no. 3
    
4.Igarashi A, Nashiro K, Kikuchi K, Sato S, Ihn H, Fujimoto M, et al. Connective tissue growth factor gene expression in tissue sections from localized scleroderma, keloid and other fibrotic skin disorders. J Invest Dermatol 1996;106:729-33.  Back to cited text no. 4
    
5.Stratton R, Shiwen X, Martini G, Holmes A, Leask A, Haberberger T, et al. Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients. J Clin Invest 2001;108:241-50.  Back to cited text no. 5
    
6.Sehgal VN, Srivastava G, Aggarwal AK, Behl PN, Choudhary M, Bajaj P, et al. Localized scleroderma/morphea. Int J Dermatol 2002;41:467-75.  Back to cited text no. 6
    
7.Fleischmajer R, Perlish JS, Reeves JR. Cellular infiltrates in scleroderma skin. Arthritis Rheum 1977;20:975-84.  Back to cited text no. 7
    
8.McNiff JM, Glusac EJ, Lazova RZ, Carroll CB. Morphea limited to the superficial reticular dermis: An under recognized histologic phenomenon. Am J Dermatopathol 1999;21:315-9.  Back to cited text no. 8
    
9.Weedon D. Skin Pathology, 2 nd ed., Philadelphia: Churchill Livingston; 2002. p. 346-51.  Back to cited text no. 9
    
10.Alter A, Grant A, Abel L, Alcaïs A, Schurr E. Leprosy as a genetic disease. Mamm Genome 2011;22:19-31.  Back to cited text no. 10
    
11.Souza CS. Leprosy: clinical forms and differential diagnosis. Med Ribeirao Preto 1997;30:325-34.  Back to cited text no. 11
    
12.Lockwood DN. Leprosy. In: Rook/Wilkison/Ebling textbook of dermatology. 8 th ed. New York: Blackwell Science; 2010. p. 32.1-32.20  Back to cited text no. 12
    
13.David W. Skin Pathology, 2 nd ed., Philadelphia: Churchill Livingston; 2002. p. 629-33.  Back to cited text no. 13
    

What is new?
The Perineural infiltration with linear array of lymphocytes in the histopathological examination could be an initial pattern of morphea.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (248 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
    Viewed2434    
    Printed52    
    Emailed2    
    PDF Downloaded56    
    Comments [Add]    

Recommend this journal