Atypical scleromyxedema improved by immunoglobulins intravenous

Scleromyxedema is a variant of papular mucinosis affecting the skin and internal organs that occurs in middle-aged people [1,2]. The different therapeutic approaches proposed for scleromyxoedema are still unsatisfactory. Intravenous immunoglobulin (IVIg) has been successfully employed in this dermatosis which the evolution is unpredictable [1,2]. The low number of cases and the lack of randomized study make the therapeutic choice difficult. There is currently no formal consensus regarding its therapeutic management [1]. We report a case of isolated scleromyxedema with favorable evolution under immunoglobulins maintained by synthetic antimalarials and Thalidomide.


INTRODUCTION
Scleromyxedema is a variant of papular mucinosis affecting the skin and internal organs that occurs in middle-aged people [1,2]. The different therapeutic approaches proposed for scleromyxoedema are still unsatisfactory. Intravenous immunoglobulin (IVIg) has been successfully employed in this dermatosis which the evolution is unpredictable [1,2]. The low number of cases and the lack of randomized study make the therapeutic choice difficult. There is currently no formal consensus regarding its therapeutic management [1]. We report a case of isolated scleromyxedema with favorable evolution under immunoglobulins maintained by synthetic antimalarials and Thalidomide.

CASE REPORT
This is a 62-year-old patient, with no pathological history, who presented about 20 days, small papules with normal skin color in some sites and ivory by others, about 2-3 mm in diameter, interesting the face, hands, forearms, neck, upper back as well as retro-auricular, followed by the installation of bilateral palpebral edema with palpebral occlusion, as well as hands and feet (Figs. 1-3). The patient had benefited from a biological assessment showing a negative proteinuria of 24 hours, the assessment in search of monoclonal gammopathy was negative, a correct thyroid assessment. Abdominal CT scan showing digestive thickening of D2 of inflammatory origin. Minimal pericardial effusion at transthoracic echography.
Cutaneous histology showed densified squamous collagen bundles, alcianophilous deposition with Alcian blue and metachromatic Toluidine blue, as well as proliferation of fibroblasts.
The diagnosis of diffuse scleromyxedema was retained and the patient was placed on intravenous immunoglobulin at a dose of 21 g/day for 5 days/month for 6 months with good improvement after just two months. This regression was maintained by thalidomide at a dose of 100 mg/day combined with synthetic antimalarials at a dose of 200 mg/day (Figs. 4-6). The recoil without recurrence is two years.

DISCUSSION
Scleromyxedema is a rare disease, that usually affects adults between the ages of 30 and 80 years with no race or gender predominance. The pathogenesis remains unclear, the main hypothesis is that circulating cytokines such as IL-1, TNF-alpha and TGF-beta, which are known to stimulate glycosaminoglycan synthesis and fibroblast proliferation in the skin, could play a role [3,4]. It belongs to the spectrum of cutaneous mucinoses and is characterized by a generalized papular eruption [4]. The rash, very often itchy, consists of firm papules of 2-3 mm in diameter, waxy in appearance [5], closely spaced, dome-shaped or flattopped papules involving the hands, forearms, head, neck, upper trunk and thighs. Papules are often arranged in a strikingly linear array, and the surrounding skin is shiny and indurate (sclerodermoid) in appearance [3]. Evolution is unpredictable. The entire skin can be reached with functional prognosis. The mucous membranes are spared [5]. Extracutaneous manifestations include neurological signs that can lead to coma and death. Cardiac, gastrointestinal, rheumatologic, muscular, vascular specially Raynaud's syndrome and respiratory manifestations are also described [1,4,5]. Hematologic involvement (monoclonal gammopathy) is very often associated. In 80% of cases, it is a IgG lambda monoclonal dysglobulinemia of undetermined significance, rarely evolving to a myeloma (less than 10%) [2,5]. Histological findings show fibroblast proliferation, mucin deposition, and fibrosis. Because of the rarity of the disorder, there are neither welldesigned clinical trials nor consensus about an effective treatment [4].
There is currently no formal consensus on therapeutic management, and the treatment is difficult despite the multiplicity of therapeutic possibilities: steroids, retinoids, PUVA, interferon, plasmapheresis, chemotherapy, electrotherapy, thalidomide or veinoglobulins [1,2]. Our patient has improved remarkably since the first treatment cure of immunoglobulins. The Thalidomide combined with synthetic antimalarials were interesting for maintenance treatment because of their immunomodulator and antiinflamatory effect.

CONCLUSION
Scleromyxedema remains a complex pathology that is difficult to treat. The initial efficacy with immunoglobulins is most often remarkable and the side effects are usually minor and transient. However, their use remains limited by their cost and availability. For maintenance treatment; Thalidomide and synthetic antimalarials sounds a good therapeutic modality.

Consent
The examination of the patient was conducted according to the Declaration of Helsinki principles.