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PubMed2001

Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema.

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.

Diagnosis, Differential↗

PubMed2002

Heparan sulphate proteoglycan in scleromyxedema promotes FGF-2 activity.

The cause of progressive dermal sclerosis, proliferation of fibroblasts, and collagen deposition in scleromyxedema is unknown. We analyzed the heparan sulphate proteoglycans (HSPG) in cutaneous nodules from a patient with scleromyxedema in order to ascertain their role in the binding of fibroblast growth factor (FGF) and promoting signaling complex assembly. Total heparan sulphate (HS) was detected with a monoclonal antibody to HSPG on paraffin sections. Binding of FGF to HS was assessed using FGF-2 followed by anti-FGF-2 antibody. The formation of HS-mediated signaling complex was studied using soluble FR1-AP, which contains the extracellular domain of FGF receptor-1 linked to alkaline phosphatase (AP) and monoclonal anti-AP-antibody. Anti FGF-2 and anti-AP antibodies were visualized using the DAKO Envision Plus system. The dermal nodule of scleromyxedema contained ample HS and these bound FGF-2 and FR1-AP. Specificity was confirmed by prior incubation with heparitinase (no staining) and omission of FGF-2 (no staining). Increased amounts of HSPG were present in the dermal nodules of scleromyxedema compared to adjacent normal dermis and these bound FGF-2, immobilized the soluble receptor protein FGFR-1 and, therefore, formed a ternary complex composed of HSPG, FGF-2 and FGFR-1 in vitro. Since this complex resembles the signaling complex formed on live cells, HSPG in the nodules of scleromyxedema are in a configuration that promotes FGF activity.

Adult↗

PubMed2004

Scleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-1a.

BACKGROUND: Animal studies have shown that some human monoclonal antibodies promote myelin repair in models of demyelinating disease. Scleromyxedema is a dermatologic disorder associated with a monoclonal gammopathy and neurologic manifestations. The reason for occurrence of cutaneous reactions in interferon treated patients is unknown. CASE DESCRIPTION: A 37-year-old woman was started on weekly interferon beta-1a (IFN beta-1a) following a diagnosis of multiple sclerosis (MS). After having been on interferon therapy for three years, she developed skin lesions secondary to scleromyxedema. Her IFN beta-1a was discontinued and intravenous immunoglobulin therapy was started for her scleromyxedema. At a six-month follow up, her skin lesions improved and there was no recurrence of neurologic symptoms. CONCLUSIONS: This is the first report of occurrence of scleromyxedema in a patient with MS. While this could be a chance association, it does raise the question if her neurologic manifestations could be secondary to scleromyxedema. Further research into the mechanism of IFN related cutaneous side effects is needed. Evidence regarding the remyelinating nature of human monoclonal antibodies raises interest in the potential therapeutic role these antibodies may have.

Adjuvants, Immunologic↗

PubMed2006

Scleromyxedema, a positive effect with thalidomide.

Scleromyxedema is a rare dermatological disorder marked by widespread symmetric 2- to 3-mm, firm, waxy, closely spaced papules involving the hands, forearms, face, neck, upper trunk and thighs. The most common extracutaneous manifestation of scleromyxedema is a benign plasma cell dyscrasia. Treatment of scleromyxedema is limited by the lack of long-term results, toxicity and significant adverse side effects. We report a severe case of scleromyxedema who had marked improvement when treated with thalidomide.

Angiogenesis Inhibitors↗

PubMed2005

Combination oral prednisone and intravenous immunoglobulin in the treatment of scleromyxedema.

BACKGROUND: Scleromyxedema is a clinical variant of the rare disease papular mucinosis that has both cutaneous and systemic manifestations. Treatment options are numerous and tend to be associated with serious potential side effects and frequent relapse. OBJECTIVE: We report a case of scleromyxedema treated with low-dose oral prednisone and intravenous immunoglobulin (IVIg). This is followed by a review of the literature. CONCLUSION: IVIg is being used for a growing number of inflammatory and immune disorders. It is being increasingly reported as a successful treatment for scleromyxedema. Although our patient succumbed to the disease, combination therapy with prednisone and IVIg provided temporary symptomatic, laboratory, and clinical improvement of the condition. Optimization of this therapeutic strategy is thus indicated for the management of scleromyxedema.

Aged, 80 and over↗

PubMed1994

Ophthalmic findings in scleromyxedema.

PURPOSE: To catalog ophthalmic findings in a cohort of patients with scleromyxedema. METHODS: Thirty-five biopsy-proven patients with scleromyxedema evaluated at the Mayo Clinic in Rochester, Minnesota, from 1960 to 1991 were identified. Eye examinations were performed on 17 of the patients. Ophthalmic findings not attributable to other systemic or ocular disease were recorded. RESULTS: The following abnormalities were considered secondary to scleromyxedema: corneal opacities (2 patients), thickened eyebrow or eyelid skin (4 patients), lagophthalmos (1 patient), and ectropion (2 patients). One patient who had concurrent polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome also had choroidal folds and papilledema. A corneal biopsy in one patient disclosed deposits of acid mucopolysaccharide, consistent with one of two previously published cases. CONCLUSION: A series of patients with scleromyxedema was reviewed. This systemic disorder infrequently may cause visually significant ophthalmic manifestations, including eyelid changes and corneal deposits. Further study is needed to characterize better the nature of the corneal opacities.

Adult↗

PubMed2001

[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus].

INTRODUCTION: Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance. CASE REPORT: A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up). COMMENTS: There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.

Combined Modality Therapy↗

PubMedDate not supplied

[Hashimoto's encephalopathy associated with scleromyxedema: coincidental or the overlapping of two syndromes with a possible autoimmune origin?].

INTRODUCTION: The encephalopathies that may accompany Hashimoto's thyroiditis and scleromyxedema, both of which are diseases that probably have an autoimmune origin, are clinically similar. The presence of both Hashimoto's thyroiditis and scleromyxedema in a patient with encephalopathy led us to compare the clinical symptoms and the different possible mechanisms accounting for the pathology. CASE REPORT: We describe the case of a 54-year-old male who was admitted to hospital because of several occurrences of transient neurological focus that finally developed into a clinical picture of aphasia, acalculia and right homonymous hemianopsia. The patient had previously been diagnosed as suffering from scleromyxedema, which responded poorly to treatment, as well as IgG kappa paraproteinemia and, six months before admission to hospital, Hashimoto's thyroiditis. No abnormalities were found in the complementary tests, except for slightly high protein levels in the cerebrospinal fluid, a diffuse slowing of brain waves in the electroencephalogram and alterations due to thyroiditis and paraproteinemia. Neurological symptoms improved after a few days of corticoid therapy. CONCLUSIONS: The presentation of encephalopathy as a stroke and its excellent response to treatment with corticoids pointed to a case of Hashimoto's encephalopathy, although we cannot completely rule out the possibility of it being a dermato-neuro syndrome, which is associated to scleromyxedema. In this paper we review the different pathological mechanisms proposed for the two clinical entities, as well as the clinical features that are similar in both syndromes and which could be an indication of some common mechanism shared by them.

Adrenal Cortex Hormones↗

PubMed1984

[Arndt-Gottron scleromyxedema and associated phenomena].

Scleromyxedema is an uncommon fibromucinous connective tissue disease characterized by accumulation of mucinous material in the dermis. A monoclonal paraprotein is regularly identified. A review of the literature (57 cases) shows the exceptional association of scleromyxedema with multiple myeloma (8.7%) and macroglobulinemia Waldenström (3.5%). A man with scleromyxedema, IgG lambda paraproteinemia, and sclerodactylia--as a special sign of scleromyxedema--is reported. Melphalan is the drug of choice in serious cases, but not effective in sclerodactylia.

Fingers↗

PubMed2007

[Marked improvement in scleromyxedema with high-dose intravenous immunoglobulin].

Scleromyxedema is a rare chronic skin disorder of unknown etiology, often accompanied by monoclonal gammopathies. Clinically the disease is characterized by generalized thickening and stiffening of the skin due to dermal mucin deposition. We report on a 56-year-old women with scleromyxedema, whose skin findings markedly improved following high-dose intravenous immunoglobulin therapy (Sandoglobulin((R)), 2 g/KG). The pronounced clinical benefit was documented by increased flexibility of the fingers, reduction of skin induration, and a decrease in number and size of mucinous papules. All previous therapeutic approaches were ineffective. High-dose intravenous immunoglobulin appears to be a promising treatment option for patients with scleromyxedema. Clinical studies are desirable, but difficult to perform in such a rare disease. Long-term follow-up on individual patients so treated is desirable.

Female↗

PubMed2005

Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation.

BACKGROUND: Scleromyxedema is a rare chronic fibromucinous disorder that can have devastating clinical manifestations, including sclerosis of the skin with progressive pharyngeal and upper airway involvement, resulting in high mortality due to respiratory complications. Herein we describe a novel therapeutic approach. Because autologous hematopoietic stem cell transplantation is effective in other plasma cell proliferative disorders, it may be effective in this setting. OBSERVATIONS: We retrospectively evaluated 6 patients who were offered high-dose chemotherapy with stem cell rescue as treatment for scleromyxedema. One heavily pretreated patient was unable to mobilize stem cells. The remaining 5 patients mobilized stem cells and underwent successful transplantation. There was no treatment-related mortality. Hematologic responses were seen in 4 patients, including 2 complete remissions and 2 partial remissions, and all 4 had improvement in extracutaneous manifestations. All 4 patients subsequently had relapse of the monoclonal protein, and 3 developed skin relapses at 14, 37, and 45 months. CONCLUSIONS: High-dose chemotherapy with stem cell rescue is feasible for patients with scleromyxedema and, although not curative, offers durable remission in most patients. This therapy should be considered before treatment with alkylating agents or other treatments that could adversely affect the ability to collect stem cells.

Adult↗

PubMed1986

Scleromyxedema with systemic involvement mimics rheumatic diseases.

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema with systemic involvement may mimic rheumatic diseases.

Adult↗

PubMed1985

Erosive arthropathy associated with scleromyxedema.

Mucin deposition in the skin (scleromyxedema) is a rare disorder with a historically confusing nomenclature. The etiology and pathogenesis of the disease is poorly understood. The precise extent of potential systemic involvement is uncertain but the prevailing hypothesis is that scleromyxedema is a disorder of skin fibroblasts without internal organ involvement. Two patients with scleromyxedema skin lesions are described in whom an erosive arthropathy developed in joints contiguous to clinically involved skin. Because of the absence of other causes of destructive arthritis, it is postulated that the associated skin disease resulted in infiltration of adjacent synovium with subsequent erosions.

Aged↗

PubMed2001

[Extracorporeal photopheresis--treatment option in scleromyxedema?].

Scleromyxedema is an uncommon disease of unclear etiology. Therapy is difficult. Two patients with scleromyxedema were treated with extracorporeal photopheresis (ECP). The first patient has been treated unsuccessfully for 3 months with PUVA-bath-therapy and for one year with cyclophosphamide and prednisolone. Thus supplementary treatment with ECP was initiated, as the cyclophosphamide and prednisolone were gradually reduced. After 29 cycles of ECP, the skin lesions had almost disappeared and the associated myopathy also resolved. In the second patient initial monotherapy with ECP was started after PUVA-bath-therapy for 3 months did not show any effect. After temporary improvement with ECP every four weeks, the skin lesions relapsed, so oral cyclophosphamide was added. These two cases confirm the effect of ECP in scleromyxedema, but probably combination therapy is at least initially more successful.

Administration, Oral↗

PubMed1995

Scleromyxedema.

BACKGROUND: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. OBJECTIVE: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. METHODS: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. RESULTS: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. CONCLUSION: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.

Adult↗

PubMed1987

Scleromyxedema with corneal deposits.

Scleromyxedema (Arndt-Gottron syndrome) is a rare cutaneous disease in which hyaluronic acid is deposited in the dermis. The authors describe a patient with scleromyxedema and corneal deposits. A corneal biopsy demonstrated hyaluronic acid deposition in the corneal stroma and amyloid P component in Bowman's membrane. This is the first report of scleromyxedema involving the cornea. It is also the first report of amyloid P component deposition in the cornea occurring independent of corneal amyloid deposits.

Adult↗

PubMed2000

Scleromyxedema: possible association with seminoma.

Although scleromyxedema has been associated with neoplasm in rare instances, the literature showed no evidence of association with seminoma. We report a 43-year-old man who presented with a scleromyxedema and relapsed seminoma. The skin lesions of scleromyxedema cleared completely on treatment of seminoma with chemotherapy.

Adult↗

PubMed2000

Scleromyxedema with dermato-neuro syndrome.

Scleromyxedema is a rare connective tissue disease of unknown cause characterized by a generalized papular eruption, dermal fibroblast proliferation, and monoclonal paraproteinemia. A paroxysmal triad consisting of high fever, seizures, and coma with a flu-like prodrome can rarely occur in patients with scleromyxedema and is termed "dermato-neuro syndrome." We describe a 41-year-old patient with scleromyxedema in whom the dermato-neuro syndrome developed.

Adult↗