IJD
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: 29  
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


 
CASE REPORT
Year : 2005  |  Volume : 50  |  Issue : 4  |  Page : 221-223
Large plaque parapsoriasis in a child


Department of Dermatology, Ramakrishna Mission Seva Pratisthan Hospital, 99, Sarat Bose Road, Kolkata 700 026, W.B, India

Correspondence Address:
Asok Kr Gangopadhyay
Department of Dermatology, Ramakrishna Mission Seva Pratisthan Hospital, 99, Sarat Bose Road, Kolkata 700 026, W.B
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions

   Abstract 

A case of large plaque parapsoriasis with extensive skin lesions is presented for its unusual clinical features. The controversial issue of its nosological position is discussed as it has a considerable impact on the management of such cases.


Keywords: Parapsoriasis en plaques, Mycosis fungoides


How to cite this article:
Das JK, Gangopadhyay AK. Large plaque parapsoriasis in a child. Indian J Dermatol 2005;50:221-3

How to cite this URL:
Das JK, Gangopadhyay AK. Large plaque parapsoriasis in a child. Indian J Dermatol [serial online] 2005 [cited 2020 Oct 27];50:221-3. Available from: https://www.e-ijd.org/text.asp?2005/50/4/221/19749



   Introduction Top


Parapsoriasis is a group of uncommon but not rare disorders, characterized by persistent, scaly, inflammatory eruptions. The current, generally accepted classification includes three entities: large plaque parapsoriasis (LPP), small plaque parapsoriasis (SPP), and pityriasis lichenoides.[1]

Parapsoriasis is a term coined by Brocq in 1902, as a central link of his grand design encompassing all inflammatory dermatoses, to a group of diseases characterized by the common features such as unknown etiology, chronicity, failure to respond to therapy, and lack of symptoms, particularly of pruritus.[1] Large plaque parapsoriasis is a disease of the middle-aged and older people, with a peak incidence in the fifth decade, with slight male preponderance and no racial and geographical predilection.[1]

Clinically, LPP lesions are oval or irregular shaped asymptomatic or mildly pruritic patches or very thin plaques, with most lesions bigger than 5 cm in diameter, and with fine wrinkles and scanty scales; telangiectasia and mottled pigmentation may also be observed. They are found mostly on the 'bathing trunk' and flexural areas, and also on extremities and upper trunk, especially breasts in women.[1] On histological examination, one observes an interface infiltrate, and in the more advanced lesions, definite epidermotropism.[1]


   Case report Top


A five year old female child presented with numerous asymptomatic hypopigmented macules developing for the past 4 years. To start with, erythematous slightly itchy scaly small macules developed on abdomen. Individual macules gradually became hypopigmented and then tended to persist and spread. Similar lesions gradually appeared on back, face, and extremities. Topical steroids, urea and emollients gave temporary relief. On examination, widespread numerous hypopigmented and a few depigmented asymptomatic macules were found all over the body. The older lesions were larger with more than 5 cm diameter and the new smaller lesions were apparently still increasing in size. No lymphadenopathy was noted. General examination was unremarkable.

Histological examination of a representative hypopigmented macule on the abdomen showed an unremarkable epidermis and a mild to moderate upper dermal infiltrate of mononuclear cells, with a few cells invading the epidermis. No atypical cells were seen. Hemogram, routine blood biochemistry, chest X-ray and urine analysis were normal. There was no atypical cell in the peripheral blood.


   Discussion Top


The classification and nomenclature of parapsoriasis is plagued with controversy. A historical reason of such controversies is the fact that the word 'plaque' in French means 'patch' without thickening or induration, and Brocq had that in mind while coining such term as 'parapsoriasis en plaques'. But in English 'plaque' refers to a circumscribed thickened or indurated area, causing confusion.[1] The nosological position of SPP and LPP is a highly debated issue, and so is their relationship with MF. [3],[4],[5],[6] Immunohistological studies have shown similar features in LPP and early mycosis fungoides lesions, including widespread epidermal expression of human leukocyte antigen (HLA-DR), a predominance of CD4+ T cell subsets, and frequent CD[7] antigen deficiency.[1]

The T cells mediating most inflammatory skin diseases belong to the skin ­associated lymphoid tissue (SALT). These T cells traffic between the skin and the T cell domains of peripheral lymph nodes via lymphatics and bloodstream and express the cutaneous lymphocyte-associated antigen (CLA).[1],[7] Mycosis fungoides have been proved to be a SALT T cell neoplasm, i.e. a malignancy of a T cell circuit rather than one particular tissue. Sensitive PCR based tumor clonality assays have proved the SALT nature of MF tumor clones by showing that they can continue to traffic after neoplastic transformation. Even in patients with very early stage disease with lesions clinicopathologically consistent with LPP, trafficking of mycosis fungoides tumor cells has been detected. So, at least in some cases LPP is a monoclonal proliferation of SALT T cells having the capacity to traffic between skin and extracutaneous sites.[1],[7]

Thus LPP can be regarded as the clinically benign end of the mycosis fungoides disease spectrum, which may culminate in transformed large cell lymphoma at its malignant extreme. The possibility of cases presenting clinically as LPP to progress to malignant end, i.e. to MF, is about 10%, as shown in a meta-analysis of four large and carefully studied series.[1],[2] This progress often takes place over many years; hence the need of prolonged and careful follow-up in all cases of LPP.

A unifying feature of parapsoriasis group of diseases is that all or them appear to be cutaneous T cell lymphoproliferative disorders; i.e. LPP, SPP, pityriasis lichenoides, and lymphomatoid papulosis, all have shown to be monoclonal disorders in many cases.[1],[7] Studies on LPP/ early MF have suggested a dominant clonal density in 1 to 10 percent, and in advanced MF a density of 50 percent or more is found.[1] This suggests that progression from LPP through various stages of MF disease spectrum is accompanied by an increase in dominant T cell clonal density resulting from mutations that confer increasing growth autonomy to the neoplastic T cell clone. Analysis of peripheral blood has demonstrated that clonal T cells are often detectable in patients with LPP/early MF or even SPP, supporting the basic unity and systemic nature of these disorder.[1] These studies, however, could not be done here due to lack of infrastructure facilities.

To conclude, in the light of the findings of the latest studies, large plaque parapsoriasis may be considered as an early 'plaque' phase of mycosis fungoides, and this has considerable prognostic and therapeutic significance. The present case is reported for its rarity, and for the unusual chinical features, e.g an early age of onset and hypopigmented lesions.

 
   References Top

1.Wood GS, Hu CH. Parapsoriasis. In : Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz Sl, eds. Dermatology in General Medicine. 6th ed. New York: McGraw-Hill, 2003:450-5.   Back to cited text no. 1    
2.Lambert WC, Everett MA. The nosology of parapsoriasis. J Am Acad Dermatol 1981;5: 373-95.   Back to cited text no. 2    
3.Ackerman AS. If small plaque (digitate) parapsoriasis is a cutaneous T-cell lymphoma, even an 'abortive' one, it must be mycosis fungoides! Arch Dermatol 1996;132: 562-6.   Back to cited text no. 3    
4.Burg G, Dummer R. Small plaque (digitate) parapsoriasis is an 'abortive cutaneous T cell lymphoma' and is not mycosis fungoides. Arch Dermatol 1995; 131: 336-8.   Back to cited text no. 4    
5.Burg G, Dummer R, Andeas H, Kemp FW, Kadin M. From inflammation to neoplasia Mycosis fungoides evolves from reactive inflammatory conditions (lymphoid infiltrates) transforming into neoplastic plaques and tumors. Arch Dermatol 2001; 137: 949-52.   Back to cited text no. 5    
6.Rubegni P, Aloe GO, Renzo MD, Pompella G, Pasqui AL, Auteri A, et al. Cytokine production profile of peripheral blood mononuclear cells in patients with large-plaque parapsoriasis. Arch Dermatol 2001 ;137: 966-7.   Back to cited text no. 6    
7.Haeffner AC, Smoller SR, Zepter K, Wood GS. Differentiation and clonality of lesional lymphocytes in small plaque parapsoriasis. Arch Dermatol 1995; 131; 321-4.  Back to cited text no. 7    


Figures

[Figure - 1], [Figure - 2]



 

Top
Print this article  Email this article
 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (164 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
    Viewed6613    
    Printed128    
    Emailed0    
    PDF Downloaded150    
    Comments [Add]    

Recommend this journal