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it is characterized by the formation of numerous lichenoid papules which coalesce to form generalized plaques that cause extensive thickening and hardening of the skin .
there are two main divisions of lichen myxedematosus : ( a ) generalized papular and sclerodermoid form ( scleromyxedema ) with systemic , even lethal , manifestation , and ( b ) a localized form without a demonstrable paraprotein , which runs a more benign course .
diagnostic criteria include generalized papules and sclerodermoid eruption , mucin deposition and fibroblast proliferation , monoclonal gammopathy , and absence of thyroid disease .
occasional patients with lichen myxedematosus have atypical features or features intermediate between scleromyxedema and localized lichen myxedematosus .
this group includes patients with scleromyxedema who suffer from lack of monoclonal gammopathy as well as individuals with localized forms of lichen myxedematosus who also have a monoclonal gammopathy and/or systemic symptoms .
possible treatment includes melphalan , interferon- , high - dose dexamethasone , autologous stem cell transplantation , thalidomide , cyclophosphamide , plasmapheresis , and intravenous immunoglobulin ( ivig ) .
a 41-year - old female patient presented with a 2-month history of pruritic lichenoid papules and tightening of the skin in her upper trunk , forehead , and arms .
glabella was involved with deep longitudinal furrows and folds ( fig 1 , fig 2 , fig 3 , fig 4 ) .
histopathologic evaluation of a skin biopsy specimen revealed proliferation of fibroblasts , thickening collagen bundles with mucin deposition in the upper and mid dermis , and sparse perivascular infiltrate of lymphocyte in the upper dermis consistent with a diagnosis of scleromyxedema ( fig 5 ) .
the laboratory test results were in the normal range including complete blood cell count , levels of inflammatory parameters ( erythrocyte sedimentation rate , c - reactive protein ) , electrolytes , calcium , glucose , antistreptolysin o , autoantibodies associated with scleroderma ( anti - topoisomerase i and anti - centromeric antibodies ) , and thyroid function tests .
given the lack of monoclonal gammopathy , we decided to begin treatment with thalidomide ( initial dosage 100 mg / daily ) and prednisolone ( 30 mg / daily ) . because of the patient 's anxiety and distress , we added doxepin ( 25 mg / daily ) .
treatment with thalidomide continued for 3 months , and prednisolone was tapered within the treatment period .
the patient exhibited remarkable improvement in symptoms such as pruritus and skin induration while skin lesions showed moderate response . during the treatment
scleromyxedema is a chronic and progressive disease of uncertain origin with increased collagen and fibroblast proliferation with irregular distribution .
the high prevalence of monoclonal gammopathy , which presents in up to 80% of patients , suggests a possible immune response of b - cell to antigenic mucin deposits in the dermis .
various therapeutic agents have been used : high - dose corticosteroids , a variety of immunosuppressive and chemotherapeutic drugs , puva and uva1 phototherapy , plasmapheresis , extracorporeal photopheresis , electron beam therapy , interferon- , retinoids , thalidomide , ivig , bortezomib , and autologous stem cell transplantation .
reported a case of scleromyxedema without underlying paraproteinemia that was treated with thalidomide for the first 3 months .
abarzua et al .   in 2014 reported a patient without paraproteinemia . despite treatment with thalidomide , the patient remained stable with maintenance of injuries . in a retrospective multicenter study in france , le moigne et al .  
showed one complete remission ( from 8 patients ) with thalidomide and another one with dexamethasone .
caudill and howell   explained complete remission of scleromyxedema in a 60-year - old man with ivig . in most of the mentioned reports ,
scleromyxedema is usually associated with multiple systemic disorders such as gastrointestinal , neurological , cardiac , muscular , renal , pulmonary , and ophthalmological disorders .
all of these associations represent events that can cause disability and may be fatal ; thus , immediate and aggressive intervention is required . in cases of serious disfiguring and refractory illness ,
encouraging results have been obtained with the use of ivig and autologous transplantation of stem cells .
based on lack of monoclonal gammopathy in our patient and no internal involvement , we treated the patient with thalidomide in combination with prednisolone for 3 months .
similar to the aforementioned studies , we observed only a moderate improvement of cutaneous lesions .
treatment of scleromyxedema is a challenging process and needs extensive studies to determine effective and first - step regimes .
according to moderate response of skin lesions and elimination of the subject , treatment of scleromyxedema is a challenging process and needs extensive studies to determine an effective and first - step regime .
based on the moderate response of skin lesions and elimination of symptoms , initial treatment with thalidomide and prednisolone may be beneficial .
however , adding other agents ( e.g. , ivig ) may lead to a more robust recovery . further clinical studies to determine an effective regimen are required .
scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology . 
 it is presenting with generalized papular eruption and sclerodermoid induration . 
 numerous treatment modalities have been reported to produce partial or permanent responses . 
 this study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomide and prednisolone .