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E-IJD-CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 3  |  Page : 323
Scleromyxedema: An atypical case


1 Department of Dermatology, Christian Medical College, Ludhiana, India
2 Department of Medicine, Christian Medical College, Ludhiana, India

Date of Web Publication6-May-2015

Correspondence Address:
Anisha George
Department of Dermatology, Christian Medical College, Ludhiana 141 008
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.156456

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   Abstract 

Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. Patients usually have associated paraproteinemia. We describe the case of a 59-year-old gentleman with features of scleromyxedema, who had severe pruritus, scalp involvement, unrestricted mobility and associated peripheral eosinophilia, but no monoclonal gammopathy.


Keywords: Eosinophilia, papular mucinosis, scleromyxedema


How to cite this article:
Thomas E, George A, Deodhar D, John M. Scleromyxedema: An atypical case . Indian J Dermatol 2015;60:323

How to cite this URL:
Thomas E, George A, Deodhar D, John M. Scleromyxedema: An atypical case . Indian J Dermatol [serial online] 2015 [cited 2020 Feb 19];60:323. Available from: http://www.e-ijd.org/text.asp?2015/60/3/323/156456

What was known?
Scleromyxedema is a rare disease with a guarded prognosis.



   Introduction Top


Scleromyxedema is a rare, chronic idiopathic disorder characterized by the appearance of numerous papules and areas of induration that are due to dermal mucin deposition in association with an increase in dermal collagen. Patients also have a monoclonal gammopathy and may have lethal systemic manifestations. This entity must be distinguished from localized variants of lichen myxedematosus where skin is the sole site of involvement. There are, however, occasional patients who have an atypical constellation of findings and fall between scleromyxedema and localized lichen gentleman who had findings of scleromyxedema and associated peripheral eosinophilia, but no monoclonal gammopathy.


   Case Report Top


A 59-year-old gentleman from Punjab, India, presented with severe generalized pruritus and closely studded papules all over the body for 7 years with an exacerbation for 15 days. The rash initially began as erythematous papules over the face, then progressed to involve the trunk and limbs. The patient had also noted generalized hyperpigmentation, which was progressive and gradual. General physical examination revealed cervical and axillary lymphadenopathy with 1 cm-sized, mobile, firm and multiple lymph nodes. Systemic examination was normal, apart from a palpable liver; 6 cm below the costal margin.

Cutaneous examination revealed numerous, 3-4 mm sized, closely spaced papules, which were firm and waxy, arranged in a widespread symmetrically distributed pattern all over the body including the scalp, and sparing only the mucosae and genitalia. The surrounding skin was shiny and indurated. The glabella was typically involved with deep longitudinal furrowing and madarosis, giving the appearance of leonine facies. Skin over the flexures was thrown into folds, however mobility was not restricted. Overlying the proximal interphalangeal joints, a doughnut sign was visible as a central depression surrounded by an elevated rim of infiltration due to skin thickening [Figure 1]. Mild diffuse palmo-plantar keratoderma was also present.

Abnormal investigations included a high total leukocyte count of 36,800/mm 3 , with an absolute eosinophil count of 31,000/mm 3 . Serum creatinine was elevated at 1.5 mg/dl. Ultrasound abdomen revealed hepatomegaly, with a liver span of 17.7 cm. High-resolution computed tomography (HRCT) of chest showed mediastinal and axillary lymphadenopathy. Though serum protein electrophoresis was normal, urine showed a faint albumin band. Thyroid function tests were normal.
Figure 1: Infiltration of the face resulting in leonine facies, coalescing sheets of papules all over the body with thedoughnut sign visible on the fingers and the skin of the flexures thrown into folds

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A skin biopsy revealed a moderately hyperkeratotic and acanthotic epidermis. The upper and mid dermis showed a moderately dense chronic inflammatory cell infiltrate of lymphocytes, plasma cells and histiocytes. In the mid dermis, collagen bundles showed fragmentation and splitting surrounded by loose myxoid stroma and plump fibroblastic cells, consistent with the diagnosis of scleromyxedema [Figure 2]. Mucin stain was positive, confirming the diagnosis.
Figure 2: A moderately dense chronic inflammatory cell infiltrate in the upper dermis. Collagen bundles with fragmentation and splitting surrounded by loose myxoid stroma and plump fibroblastic cells all over the mid-dermis, hematoxylin and eosin x10

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   Discussion Top


Scleromyxedema, also known as Arndt Gottron syndrome or generalized lichen myxedematosus, is a rare disease, etiology of which is not yet known. It usually affects middle-aged individuals. [1] It is a chronic and progressive disease, in which response to therapy has been inconsistent. [2]

Diagnostic criteria of scleromyxedema include a generalized papular eruption and sclerodermoid features, histological features of mucin deposition, fibroblast proliferation and fibrosis, monoclonal gammopathy on serum protein electrophoresis and the absence of thyroid disease. [3]

Diffuse thickening of the skin underlies the papules in this syndrome. Accentuation of facial folds and creases may distort the features giving the appearance of leonine facies. The mucous membranes and scalp are usually spared. Rash is usually asymptomatic, but severe pruritus has been described. As the condition progresses, skin stiffening, sclerodactyly and decreased mobility of joints is seen. [2] Nail fold and mat-like telangiectasias, and calcinosis, as seen in systemic sclerosis are absent. The skin in scleromyxedema moves over the subcutaneous tissue, unlike in scleroderma.

Scleromyxedema is almost always associated with paraproteinemia. The monoclonal gammopathy is usually IgG with lambda light chains [4] seen in 80% patients. [1] The paraproteinemia is said to either stimulate the production of hyaluronic acid and prostaglandin E by fibroblasts or represents an immunologic response to dermal mucin. The patients who do not have paraproteinemia should be followed up for its presence for years. Patients of scleromyxedema can have a number of internal manifestations, in particular- muscular, neurologic, rheumatologic, pulmonary, renal and cardiovascular involvement. Gastrointestinal involvement is quite common and they present with distal dysphagia.

Staging of the disease has been proposed, with stage I being limited cutaneous papular mucinosis, stage II is generalized cutaneous mucinosis and/or extracutaneous manifestation(s) and stage III generalized cutaneous mucinosis and/or extracutaneous manifestation(s) and disease related Karnofsky PS <50%. [4] Eosinophilia has been described in patients with papular mucinosis. [5]

The characteristic histopathologic triad includes diffuse dermal mucin deposition, increased collagen and numerous irregularly shaped fibroblasts. [6]

Treatment is difficult; melphalan is the most commonly used therapeutic agent; [7] high doses have been recommended for stage II and III patients. [4] Other therapies include steroids, methotrexate, cyclophosphamide, thalidomide, cyclosporine, oral retinoids, extracorporeal photopheresis and plasmapheresis. [8] IV immunoglobulins can be considered as a well tolerated and efficacious second line therapy and also as first line therapy in cases of acutely worsening neurologic function. [9] High-dose melphalan along with autologous stem cell therapy was found to be effective. [4] BEAM regimen (BCNU, etoposide, cytarabine and melphalan) has been recommended as the conditioning prior to autologous stem cell transplantation (ASCT) for prolonged remission. [10] The level of evidence ascribed to all these therapies is level III, as this is a rare disease.

 
   References Top

1.
Pomann JJ, Rudner EJ. Scleromyxedema revisited. Int J Dermatol 2003;42:31-5.  Back to cited text no. 1
    
2.
Harris AO, Altman AR, Tschen J, Wolf JE. Scleromyxedema. Int J Dermatol 1989;28:661-7.  Back to cited text no. 2
    
3.
Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 2001;44:273-81.  Back to cited text no. 3
    
4.
Donato ML, Feasel AM, Weber DM, Prieto VG, Giralt SA, Champlin RE, et al. Scleromyxedema: Role of high-dose melphalan with autologous stem cell transplantation. Blood. 2006;107:463-6.  Back to cited text no. 4
    
5.
McAdam LP, Pearson CM, Pitts WH, Sadoff L, Verity MA. Papular mucinosis with myopathy, arthritis, and eosinophilia. A histopathologic study. Arthritis Rheum 1977;20:989-96.  Back to cited text no. 5
[PUBMED]    
6.
Mehta V, Balachandran C, Rao R. Arndt Gottron scleromyxedema: Successful response to treatment with steroid minipulse and methotrexate. Indian J Dermatol 2009;54:193-5.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol 1995;33:37-43.  Back to cited text no. 7
    
8.
Allam M, Ghozzi M. Scleromyxedema: A case report and review of the literature. Case Rep Dermatol 2013;5:168-75.  Back to cited text no. 8
    
9.
Manousaridis I, Loeser C, Goerdt S, Hassel JC. Managing scleromyxedema with intravenous immunoglobulin: Acute worsening of scleromyxedema with biclonal gammopathy. Acta Dermatovenerol Alp Panonica Adriat 2010;19:15-9.  Back to cited text no. 9
    
10.
Illa I, de la Torre C, Rojas-Garcia R, Altes A, Blesa R, Sierra J, et al. Steady remission of scleromyxedema 3 years after autologous stem cell transplantation: An in vivo and in vitro study. Blood 2006;108:773-4.  Back to cited text no. 10
[PUBMED]    

What is new?
Scleromyxedema can also present with severe pruritus, scalp involvement, unrestricted mobility and associated eosinophilia without paraproteinemia.


    Figures

  [Figure 1], [Figure 2]



 

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