Monday, August 11

Scleromyxedema - Papular mucinosis - Lichen myxedematosus

Papular mucinosis - Lichen myxedematosus - Scleromyxedema.
Lichen myxedematosus (aka papular mucinosis or scleromyxedema) are heterogenous spectrum of rare idiopathic cutaneous disorders characterized by excess deposits of mucin (mucinosis).
Scleromyxedema, a generalized lichen myxedematosus, presents generalized mucinosis with papules, sclerosis and paraproteinemia. Scleromyxedema is associated with several systemic involvements that can result in impaired functions.

Papular mucinosis, a localized form of lichen myxedematosus, is characterized by papules, nodules and plaques. Unlike scleromyxedema, papular mucinosis does not present monoclonal gammopathy, sclerosis or systemic manifestations. Scleromyxedema form of lichen myxedematosus is not reported in children. Scleromyxedema usually affects adults above thirty years of age. There is no gender predilection in scleromyxedema affliction.

Causes of scleromyxedema and papular mucinosis

The cause of primary lichen myxedematosus disorder is idiopathic and unknown.
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Lichen myxedematosus is believed to be a fibroblast disorder inducing excess mucinosis. The cytokines interleukin (IL)–1, tumor necrosis factor (TNF)–alpha, and TNF-beta are also believed to cause mucinosis. Some of these papular mucinosis disorders are found to be associated with bone marrow cancers, HIV infection and hepatitis C.

Signs and symptoms of papular mucinosis

The localized form of lichen myxedematosus disorder involves mucinosis of limited areas on hands, face and extremities and appears as waxy solid bumps, nodules, papules and plaques. The papules in papular mucinosis contain mucin and on injury the papular wound may exude mucin.

Signs and symptoms of scleromyxedema

The generalized lichen myxedematosus disorder covers larger parts of the body with scleroderma-like induration and skin stiffening.
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The mobility of the mouth, lips, hands, arms, joints and legs may be hampered by scleromyxedema. The hair on the eyebrows, armpits and pubic area may be sparse in the scleromyxedema affected persons. The generalized mucinosis involved areas may be erythematous and edematous with pinkish-brownish discoloration.

Systemic manifestations of scleromyxedema

Scleromyxedema presents systemic manifestations. Systemic involvements of generalized scleromyxedema include neurologic complications, rheumatologic manifestations, respiratory problems, gastrointestinal abnormalities, renal problems and cardiac abnormalities. Some of the systemic manifestations of scleromyxedema are listed below.
  • Difficulty in swallowing
  • Proximal muscle weakness
  • Reduced oxygen intake
  • Dyspnea
  • Arthritis of the joints
  • Renal failure
  • Inflammatory myopathy
  • Raynaud syndrome
  • Ectropion eyelid
  • corneal opacities
  • Acute neurologic complications
  • Encephalopathy
  • Peripheral neuropathy
  • Cardiovascular abnormalities
  • Myocardial ischemia
  • Valvular mucin deposition
  • Nasal regurgitation
  • Decreased epiglottis mobility
  • Decreased vocal cord mobility
  • Paraproteinemia - typical symptom of Scleromyxedema

Diagnosis of scleromyxedema and papular mucinosis

In scleromyxedema histologic examination may reveal mucinosis, fibroblast proliferation, and fibrosis.
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There is monoclonal gammopathy. However thyroid disease is absent. In papular mucinosis, though certain amount of fibroblast proliferation is seen, both paraproteinemia and thyroid disease are absent.

Atypical forms of lichen myxedematosus disorder do not meet the criteria for either papular mucinosis or scleromyxedema. Peng Wang et al have described case of an atypical localized lichenoid plaque form in a patient appearing like papular mucinosis. The patient had lichenoid plaque eruptions restricted to neck region. There were no paraproteinemia or thyroid dysfunction in the patient. The patient also suffered from the systemic involvement of kidney with immunoglobulin A nephropathy (IgA nephropathy). There was proliferation of fibroblasts and mucin deposition in the renal interstitium and glomeruli.

Myxedema of thyroid disease, scleroderma (systemic sclerosis), scleredema, and nephrogenic systemic fibrosis may have to be ruled out by differential diagnosis lichen myxedematosus.

Treatment options for lichen myxedematosus

Treatment of both mucinosis and scleromyxedema include use of topical tacrolimus, retinoids, isotretinoin, thalidomide, corticosteroids, high-dose dexamethasone, methotrexate, psoralen UV-A (PUVA), plasmapheresis, UV phototherapy, intravenous immunoglobulin, dermabrasion, carbon dioxide laser excision, electrocoagulation, autologous stem cell transplantation and lenalidomide. These treatments shows various degrees of success.

M. Dolenc-Voljc et al reported successfully treating scleromyxedema having subcutaneous nodules with thalidomide and intravenous immunoglobulin. Biopsies of the scleromyxedema lesions showed fibroblasts proliferation and mucinosis between collagen bundles. Treatment with thalidomide and intravenous immunoglobulin lead to complete scleromyxedema remission. Dilip K Sa et al reported a case of localized papular mucinosis. Treatment with oral cyclophosphamide showed excellent response and papular mucinosis lesions had disappeared.
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References:
1.M. Dolenc-Voljc, V. Jurcic, A. Hocevar, M. Tomsic. Scleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin. Case Rep Dermatol. 2013;Sep-Dec; 5(3): 309–315.
2.Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. 2001; Feb;44(2):273-81.
3.Dilip K, Aparajita Ghosh, Piyush Kumar, Ramesh C Gharami. A Case of Localized Papular Mucinosis Showing Excellent Response to Cyclophosphamide. Indian J Dermatol. 2014 Mar-Apr; 59(2): 202–204. doi: 10.4103/0019-5154.127697.

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