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: 1510  
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-IJD CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 2  |  Page : 215
Scleromyxedema, a therapeutic dilemma


1 Basic Science, School of Medicine, Universidad de Monterrey, Nuevo León, Mexico, USA
2 Department of Dermatology, University Hospital "Dr. Jose E. González", Universidad Autonoma de Nuevo Leon, Nuevo León, Mexico, USA

Date of Web Publication3-Mar-2015

Correspondence Address:
Julio Cesar Salas-Alanis
Department of Dermatology, Yale University School of Medicine, Otomie 206, Colonia Azteca Guadalupe, Nuevo León, Mexico CP 67150,
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.152600

Rights and Permissions

   Abstract 

Scleromyxedema is characterized by indurated erythematous papules disseminated on the face, chest and limbs. About twenty cases treated with thalidomide, stem cells, melphalan and immunoglobulin with varying results have been described. We present the case of a 28-year-old male patient diagnosed with scleromyxedema not associated with monoclonal gammopathy, multi-treated with anti-leprosy drugs, UVA1, and thalidomide for 4 years with no improvement.


Keywords: Papular mucinosis, scleromyxedema, thalidomide, treatment failure, UVA1


How to cite this article:
Salas-Alanis JC, Martinez-Jaramillo B, Gomez-Flores M, Ocampo-Candiani J. Scleromyxedema, a therapeutic dilemma. Indian J Dermatol 2015;60:215

How to cite this URL:
Salas-Alanis JC, Martinez-Jaramillo B, Gomez-Flores M, Ocampo-Candiani J. Scleromyxedema, a therapeutic dilemma. Indian J Dermatol [serial online] 2015 [cited 2019 Oct 22];60:215. Available from: http://www.e-ijd.org/text.asp?2015/60/2/215/152600

What was known?
Scleromyxedema is a rare disease with no standard treatment. Patients die due to systemic symptoms or even from chemotherapy.



   Introduction Top


Scleromyxedema is a generalized rare papular mucinosis (also known as lichen myxedematosus) associated to a monoclonal gammopathy and systemic symptoms. [1] Our case is an atypical scleromyxedema because the patient does not have the last two characteristics. There are about 250 cases reported in all of its modalities and no reports about this disease have emerged from our dermatology department in Mexico. Due to the extreme rarity of the disease, treatment is very difficult and most patients end up disabled or dead.


   Case Report Top


The patient is a 28-year-old Mexican male with no previous medical history who presents a disseminated pruritic dermatosis of the face and extremities characterized by confluent erythematous papules and nodules on the face and ears causing a leonine facies [Figure 1]a. He has had an 18-month evolution during which the lesions have increased in number and size. He was treated for one year for multibacillary leprosy with WHO multidrug therapy (WHO-MDT), which includes 12 months of treatment with rifampicin 600 mg monthly + clofazimine 300 mg once a month and 50 mg daily + dapsone 100 mg daily. [2] The patient had no sign of improvement.
Figure 1: a) Symmetric generalized dermatosis with uncountable millimetric brown papules; b) Central papule associated with epidermal hyperplasia; note the abundance of mucin stained with alcian Blue (4x) and fibroblastic proliferation.

Click here to view


Laboratory studies, such as complete blood count, blood chemistry, liver function and thyroid function tests, HIV testing, and serum protein electrophoresis, were normal or negative.

Biopsy revealed irregular proliferation of fibroblasts associated with abundant mucin deposition in the papillary dermis establishing the diagnosis of atypical scleromyxedema not associated with monoclonal gammopathy [Figure 1]b. Due to an error by pathology, the patient was diagnosed and treated as leprosy, with the absence of systemic disease and completely normal laboratory tests, including monoclonal gammopathy being notable.

The patient was treated unsuccessfully with hydroxychloroquine 200 mg/day for four months, plus 21 sessions of UVA1 at doses of 10-40 J/cm 2 followed by thalidomide 50-100 mg/day for 3 months.


   Discussion Top


Scleromyxedema is a chronic rare skin disease of unknown etiology that affects middle-aged adults. It manifests as a generalized symmetric eruption of 2-3 mm firm waxy papules in a linear pattern that converge in indurated plates on the extremities, upper trunk, glabella, neck and ears, sometimes mimicking the leonine facies of leprosy, a finding similar to that of our patient. It never affects the mucous membranes or the scalp. [1],[3],[4],[5]

There may be erythema, edema, hyperpigmentation and itching, and in advanced stages, thickening of the skin with sclerodactyly, and decreased mobility of the mouth and joints, which causes severe disability. Skin biopsy shows fibroblast proliferation, and increased collagen and mucine. [1],[3] It is associated with a monoclonal gammopathy (IgG λ). Extracutaneous manifestations include cardiovascular, gastrointestinal, pulmonary, rheumatologic, and neurologic signs and symptoms. [1],[5],[6]

Multiple drugs have been used without success; however, there are recent reports of cases successfully treated with intravenous immunoglobulin, thalidomide, melphalan, bortezomib, and autologous stem cell transplantation [Table 1].
Table 1: A few case reports with successful treatment


Click here to view


The use of thalidomide (100 mg/day) for one year in scleromyxedema has been reported to improve mobility and the skin. [7] Bortezomib and melphalan associated with autologous stem cell transplantation, thalidomide or dexamethasone have also been reported with good results. [8] The use of immunoglobulin has been satisfactory, more so in cases of papular mucinosis and recently there is a case in combination with lenalidomide. [9],[10] On occasion, the increase in monoclonal antibodies disappears with treatment, but this is not related to its symptomatology. [1]


   Conclusion Top


Scleromyxedema is a chronic and systemic disease without no specific treatment. Our review describes improvement of this condition with different treatment regimens. Thalidomide, melphalan and IVIG remain the best choices to treat this unpredictable disease.

Because of the rarity of this disease, and treatment failure in most cases, long-term surveillance is needed to verify the effectiveness of the treatment provided

 
   References Top

1.
Rongioletti F, Rebora A. Updated classification of popular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273-81.  Back to cited text no. 1
    
2.
De la Fuente-Garcia A, Jaramillo-Moreno G, Ocampo-Candiani J. What Caused These Changes on the Face and the Trunk? The Dermatologist 2010;18:47-50.  Back to cited text no. 2
    
3.
Rongioletti F. Lichen myxedematosus (papular mucinosis): New concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100-4.  Back to cited text no. 3
    
4.
Heymann WR. Scleromyxedema. J Am Acad Dermatol 2007;57:890-1.  Back to cited text no. 4
    
5.
Cokonis Georgakis CD, Falasca G, Georgakis A, Heymann WR. Scleromyxedema. Clin Dermatol 2006;24:493-7.  Back to cited text no. 5
    
6.
Pomann JJ, Rudner EJ. Scleromyxedema revisited. Int J Dermatol 2003;42:31-5.  Back to cited text no. 6
    
7.
Guarenti I, Sebastiani V, Pinto G, de Souza PR, de Almeida H Jr. Successful treatment of scleromyxedema with oral thalidomide. Int J Dermatol 2012;52:631-2.  Back to cited text no. 7
    
8.
Yeung CK, Loong F, Kwong YL. Scleromyxoedema due to a plasma cell neoplasm: Rapid remission with bortezomib, thalidomide and dexamethasone. Br J Haematol 2012;157:411.  Back to cited text no. 8
    
9.
Bielsa I, Benvenutti F, Guinovart RM, Ferrándiz C. Good response of scleromyxedema and dermato-neuro syndrome to treatment with intravenous immunoglobulins. Actas Dermosifiliogr 2012;103:317-20.  Back to cited text no. 9
    
10.
Brunet-Possenti F, Hermine O, Marinho E, Crickx B, Descamps V. Combination of intravenous immunoglobulins and lenalidomide in the treatment of scleromyxedema. J Am Acad Dermatol 2013;69:319-20.  Back to cited text no. 10
    


    Figures

  [Figure 1]
 
 
    Tables

  [Table 1]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (487 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed2396    
    Printed43    
    Emailed1    
    PDF Downloaded38    
    Comments [Add]    

Recommend this journal