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CASE REPORT
Year : 1990  |  Volume : 56  |  Issue : 5  |  Page : 389-390

Scleromyxoedema




Correspondence Address:
T Susan John


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  Abstract 

A case of a 35-year-old lady with scleromyxoedema is reported. The lesions were initially urticarial plaques and nodules. Which evolved into a generalized lichenoid papular eruption. Bone marrow was normal.


Keywords: Scleromyxoedema, Urticarial plaques, Nodules


How to cite this article:
John T S, Nair V L, Sugathan P. Scleromyxoedema. Indian J Dermatol Venereol Leprol 1990;56:389-90

How to cite this URL:
John T S, Nair V L, Sugathan P. Scleromyxoedema. Indian J Dermatol Venereol Leprol [serial online] 1990 [cited 2020 Sep 19];56:389-90. Available from: http://www.ijdvl.com/text.asp?1990/56/5/389/3582


Scleromyxoedema is a variant of lichen myxoedematosus, a generalised form of primary cutaneous mucinosis. The first case of lichen myxoedematosus was described by Dubreuilh in 1906.[1] Perry et a1[2] classified them into four clinical types, a generalized lichenoid type, a discrete papular type involving the trunk and extremities, localized or generalized lichenoid plaques and urticarial plaques and nodules which tend to be transient and change into one of the other three types.

To the best of our knowledge only two cases of scleromyxoedema have been reported from India[3],[4] but the disease presenting as urticarial plaques and nodules has not been reported so far.


  Case report Top


A thirty-five year old lady carne with urticarial wheals and oedema of the face, forearms and hands associated with mild fever and arthralgia of four days duration. It rapidly progressed to involve the sides of the neck, upper trunk and knees. Discrete, firm, non­tender nodular lesions of about 1.5cm in size were present on the forehead, ears-and gluteal region. A provisional diagnosis of urticarial vasculitis was made and she was treated symptomatically. The oedema of the face and arms regressed partially in a few days. Soon she developed discrete and grouped, dime shaped, skin colored, firm, papular lesions 2­3mm in size over the face, sides of the neck, arms, back of the trunk, abdomen and extensor aspect of the knees. Linear arrangement of lesions were also noted [Figure - 1]. The skin was thickened at the site of involvement but was not bound down. Total and differential counts, urinalysis, serum cholesterol, liver and renal function tests, X-ray chest, ECG, serum electrophoresis and bone marrow studies were normal. Rose Waaler test was negative. Biopsy from a papular lesion on the thigh showed a normal epidermis with fibroblastic proliferation and splitting of collagen bundles by mucin deposition mainly in the upper dermis. Mucin was stained with alcian blue at ph 2.5.

With toluidine blue it stained metachromatically purple. Our patient was treated with topical steroids and she responded favourably with gradual resolution of the thickened skin within four months.


  Comments Top


In scleromyxoedema diffuse thickening of the skin underlies the papules. Sclerodactyly and raynauds phenomenon may occur[5]. Associated myopathy[6], seronegative polyarthritis, bizarre neurological findings including psychosis[7] and accelerated coronary disease[8] have been reported in a few patients. Our patient did not have any systemic involvement and had no paraproteins on electrophoresis though IgG paraproteinemia, mainly of the lambda variety is reported[9]. The bone marrow was not involved in our patient though the occurrence of plasmacytosis when revealed by marrow studies may be a forerunner of multiple myeloma[10]. Cytotoxic agents and steroids are used with good results. Dermabrasion, electron beam treatment[11] and puva treatment[12] are also beneficial. As the bone marrow was not involved cytotoxic drugs were not considered for this patient.

 
  References Top

1.Debreuilh W : Fibromes miliares follicularies. Sclerodemie consecutive. Arch Dermatol Syphiligr, 1908; 92: 569, quoted by From L and Assaad D: Neoplasms, Pseudo neoplasms, Hyperplasias and mucinoses of supporting tissue origin in : Dermatology in General Medicine, third ed, Editors, Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen KF, McGraw-Hill Book Co., New York, 1987; pp 1046.  Back to cited text no. 1    
2.Perry HO, Montgomery H and Stickney JM: Further observations on lichen myxoedematosus, Ann Int Med, 1960; 53: 955-969.  Back to cited text no. 2    
3.Singh M, Kaur S, Kumar B et al: Scleromyxoedema, Ind J Dermatol Venereol Leprol, 1985; 51:111-113.  Back to cited text no. 3    
4.Bajaj AK, Gupta SC, Tripathi A et al, Scleromyxoedema, Ind J Dermatol Venereol Leprol, 1986; 52: 340-342.  Back to cited text no. 4    
5.Fine JD and Moschella SL: Diseases of nutrition and metabolism in : Dermatology seconded, Editors Moschella SL and Hurley HJ: WB Saunders Co., Philadelphia, 1988,1475-1477.  Back to cited text no. 5    
6.Verity MA, Toop J, Mc Adam et al Scleromyxoedema myopathy histochemical and electron microscopic observations, Am J Clin Patho, 1978; 69: 446-451.  Back to cited text no. 6    
7.Kanter GR, Bergfield WF and Kaizen WE: Scleromyxoedema associated with renal disease and acute psychosis. J Amer Acad Dermatol, 1968; 14: 879-888.  Back to cited text no. 7    
8.Rudner EJ, Mehregan A and Pinkus H: Scleromyxoedema, A variant of lichen myxoedematosus, Arch Dermatol, 1966; 93: 3-12.  Back to cited text no. 8    
9.Lai A Fat RFM, Suurmond D, Radl J et al : Scleromyxoedema (lichen myxoedematosus) associated with a paraprotein IgG, of type Kappa, Brit J Dermatol, 1973; 88: 107-116.  Back to cited text no. 9    
10.Feldman P: Lichen Myxoedematosus, with plasma cell dyscrasia, Arch Dermatol, 1968; 97:606-608.  Back to cited text no. 10    
11.Lowe NJ, Dufton PA, Hunter RD et al: Electron beam treatment of scleromyxoedema, Brit J Dermatol, 1982; 106: 449-453.  Back to cited text no. 11    
12.Farr PM and Ive FA: PUVA treatment of scleromyxoedema, Brit J Dermatol, 1984; 110 347-350.  Back to cited text no. 12    


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