Background

This rare disease has two variants, a localized and generalized form. Papular mucinosis (lichen myxedematosus) presents with localized multiple, asymptomatic pale waxy papules on the hands, forearms, face, neck, and upper trunk. It has been associated with HIV infection and the L-tryptophan eosinophilia-myalgia syndrome.

Scleromyxedema has generalized lichenoid papules with thickened skin and symmetric eruption of 2 to 3 mm, firm, waxy, closely spaced papules, most commonly located on the hands, forearms, face, neck, upper trunk, and thighs. This disorder is usually progressive although occasional resolution may occur. It affects middle-aged adults without sex predilection.

Sometimes the terms lichen myxedematosus, papular mucinosis, and scleromyxedema have been often used loosely and indiscriminately as synonyms. The importance of this diagnosis is the frequent association with a paraprotein, usually IgG lambda. This association is strongest with scleromyxedema. Rarely, multiple myeloma and Waldenstrom's macroglobulinemia may occur. Other rare associations include bizarre neurological events, dermatomyositis, scleroderma, atherosclerosis, multiple keratoacanthomas, and an underlying carcinoma.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
AGE RANGE-MEDIAN
Scleromyxedema	Middle-aged adults
SEX (M:F)
Scleromyxedema	Equal

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
DERMATOMYOSITIS
Scleromyxedema (lichen myxedematosus) associated with dermatomyositis. Launay D, Hatron PY, Delaporte E, Hachulla E, Devulder B, Piette F. Department of Internal Medicine, Claude Huriez Hospital, 1, place de Verdun, 59037 Lille Cedex. France.	Br J Dermatol 2001 Feb;144(2):359-62 Abstract quote A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.
HIV
Papular mucinosis with rapid spontaneous regression in an HIV-infected patient. Depaire-Duclos F, Renuy F, Dandurand M, Guillot B. Department of Dermatology, Hopital Caremeau, CHU Nimes, F-30029 Nimes Cedex, France.	Eur J Dermatol. 1998 Jul-Aug;8(5):353-4. Abstract quote   Papular mucinosis is a rare, papular eruption caused by the dermal deposition of mucin. Three clinical subtypes have been described. The evolution is usually chronic and no effective treatment is available. Papular mucinosis has recently been reported in patients with AIDS. We describe another case of papular mucinosis in an HIV patient with an uncommonly rapid, total regression.
OBESITY
Localized lichen myxoedematosus (papular mucinosis) associated with morbid obesity: report of two cases. Saez-Rodriguez M, Garcia-Bustinduy M, Lopez-Alba A, Noda-Cabrera A, Guimera-Martin-Neda F, Dorta-Alom S, Escoda-Garcia M, Fagundo-Gonzalez E, Sanchez-Gonzalez R, Martin-Herrera A, Garcia-Montelongo R. Department of Dermatology, Hospital Universitario de Canarias, University of La Laguna, Tenerife, Spain.	Br J Dermatol. 2003 Jan;148(1):165-8. Abstract quote   Cutaneous diseases are often found in obese patients but, to our knowledge, mucinous disorders have not been previously reported in association with obesity. Two cases of localized lichen myxoedematosus (papular mucinosis) in two women with morbid obesity are described. Both patients underwent a low-calorie diet for a 1-year period in one case, and for 4 months in the other one, as the only treatment. There was complete resolution of cutaneous lesions at the same time that an important weight loss was observed. Nevertheless, although spontaneous regression is not frequent, it could not be disregarded in either of these two cases.
RENAL HEMODIALYSIS	Lancet 2000;356: 1000-1

 

PATHOGENESIS	CHARACTERIZATION
PARAPROTEINS	Paraprotein levels correlate with neither extent nor progression of the disease However, whereas scleromyxedema serum enhances fibroblast proliferation an immunoglobulin purified from the paraprotein-containing serum proved unable to stimulate fibroblasts to proliferate in vitro which suggests a pathogenetic role of a circulating factor other than the paraprotein

LABORATORY AND RADIOLOGIC

CHARACTERIZATION

Paraproteinemia in scleromyxedema

J Am Acad Dermatol 1995;33:37-43. Associated with many systemic disorders and, almost constantly, with paraproteinemia (83.2%) Usually IgG with light chains Biclonal IgG and IgA paraproteinemia or polyclonal hypergammaglobulinemia have also been reported Although a mild plasmacytosis may be found in the bone marrow, the scleromyxedema monoclonal gammopathy progresses to multiple myeloma in only 10% of cases Other hematologic malignancies such as Hodgkin's and non-Hodgkin's lymphoma, Waldenström's macroglobulinemia, and leukemia

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. Rongioletti F, Rebora A. Department of Endocrinologic and Metabolic Sciences, Section of Dermatology, University of Genoa, Italy.	J Am Acad Dermatol. 2001 Feb;44(2):273-81. Abstract quote   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.
GENERALIZED	Diagnosis of scleromyxedema should fulfill the following criteria: (1) Generalized papular and sclerodermoid eruption (2) Mucin deposition, fibroblast proliferation, and fibrosis (3) Monoclonal gammopathy (4) Absence of thyroid disease. Papules are commonly arranged in a linear mode Skin nearby is shiny resembling scleroderma Glabella is typically involved with deep longitudinal furrowing Erythema, edema, and a brownish discoloration Itching Sparing of the mucous membranes and the scalp As the condition progresses, erythematous and infiltrated plaques may occur with skin stiffening, sclerodactyly, and decreased mobility of the mouth and joints Elevated rim with a central depression due to the skin thickening is seen on the proximal interphalangeal joints (“doughnut sign”)
Lichen myxedematosus with systemic involvement: Clinical and autopsy findings Barbara Loggini, MD Raffaele Pingitore, MD Alessandro Avvenente, MD Giuseppina Giuliano, MD Paolo Barachini, MD Pisa, Italy	J Am Acad Dermatol 2001;45:606-8 Abstract quote Lichen myxedematosus is a rare disease that is characterized by the formation of lichenoid papules and plaques. Histologic examination shows deposition of mucinous material in the dermis. We report the case of a patient with cutaneous and systemic involvement and examine the clinical and postmortem data.
LOCALIZED	Diagnostic criteria: (1) Papular or nodular/plaque eruption (2) Mucin deposition with variable fibroblast proliferation (3) Absence of both monoclonal gammopathy and thyroid disease.
	Localized form is further subdivided into 5 variants: 1. 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 5. Nodular form.
NON-CLASSIFIABLE	A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form 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 4. Not well-specified cases
	Int J Dermatol 1987;26:91-5. J Am Acad Dermatol 1996;34:928-30. Cutis 1981;28:60-4. Cases showing scleromyxedema-like systemic involvement, such as dysphagia, hoarseness, pulmonary disturbances, and carpal tunnel syndrome or myositis without skin sclerosis or paraproteinemia One case of localized nodular mucinosis was associated with myalgias, carpal tunnel syndrome, and deep venous thrombosis was diagnosed as sclero-myxedema
	Dermatology 1992;185:134-6. Br J Dermatol 1979;100:727-30. Br J Dermatol 1996;135:467-70. Rare cases of localized LM are associated with monoclonal gammopathy. They include a patient with acral persistent papular mucinosis and IgA paraproteinemia and another case of discrete papular mucinosis with a nodular component and an abnormal serum protein In the latter patient the skin lesions resolved spontaneously after 11 years, and the abnormal protein disappeared several years later Another patient with discrete papular mucinosis had monoclonal plasma cell population in a papule, but no paraprotein
	Arch Dermatol 1966;93:3-12. Int J Dermatol 1978;10:833-9. Cutis 1981;28:60-4. Br J Dermatol 1993;129:88-91 Some cases of typical scleromyxedema lack the monoclonal gammopathy and may constitute cases of delayed appearance of serum paraprotein, which has occasionally been reported to occur after skin changes after 1 year Some cases with mixed features with one case is reported as having features of both acral persistent papular mucinosis and self-healing juvenile cutaneous mucinosis with carpal tunnel syndrome
ADDITIONAL CLINICAL SITES
ACRAL PERSISTENT PAPULAR MUCINOSIS
Acral persistent papular mucinosis. Harris JE, Purcell SM, Griffin TD. Lehigh Valley Hospital Dermatology Residency Program, Allentown, Pennsylvania, USA.	J Am Acad Dermatol. 2004 Dec;51(6):982-8. Abstract quote   Acral persistent papular mucinosis is a rare subtype of localized lichen myxedematosus. For half a century, this disease has endured a controversial and constantly evolving classification. We describe a patient who presented with discrete, flesh-colored papules on the hands, wrists, and forearms in a distribution consistent with acral persistent papular mucinosis. Histology was also constant with this disease, showing a well-circumscribed deposition of mucin in the upper and mid dermis that spared a small grenz zone. The changing nomenclature and diagnostic requirements of acral persistent papular mucinosis that have allowed it to remain a topic of debate are examined through a comprehensive review of the literature. All reported cases are reviewed.
CENTRAL NERVOUS SYSTEM	15% of patients Peripheral neuropathy may accompany, follow, or antedate the cutaneous manifestations. At least 10 cases of coma have been described. Coma is preceded by dysarthria, flu-like illness and weakness. CT scan finding is normal and postmortem examination reveals focal demyelinization, gliosis, and cerebral edema only occasionally Mucin deposition in the brain has never been reported
ESOPHAGUS	Dysphagia and nasal regurgitation are reported by 31.6% of patients Radiographic evidence of esophageal aperistalsis and histologic features of myositis Induration of the tongue has been occasionally described
EYE	Eyelids may be thickened, and ectropion and lagophthalmos may occur Some cases of corneal involvement have also been described occasionally with mucin and/or immunoglobulin deposition
HEART	Hypertension, atherosclerosis, and myocardial infarction have been described and mucin has been found in the walls of coronary vessels
JOINTS	Reported in 10.5% of the patients Arthralgia, migratory arthritis, and seronegative polyarthritis with occasional mucin deposition 9.6% of patients have carpal tunnel syndrome and 8.8% had Raynaud's phenomenon Sclerodactyly, acrosteolysis, and an association with other rheumatic diseases (scleroderma, rheumatoid arthritis, sicca syndrome)
KIDNEY	Scleroderma-like renal disease may occur with mucin deposition in the perivascular connective tissue of the kidney and in Bowman's capsule
LARNYX	Rare cases of laryngeal involvement have also been reported
LUNGS	Dyspnea is found in 16.7% of patients Restrictive or obstructive lung involvement and a reduced monoxide diffuse capacity have been reported. Rarely pulmonary hypertension developed, mucin deposition was found in the large pulmonary veins and artery
Severe lung involvement in systemic scleromyxoedema: a highly unusual finding. Morales P, Martinez MA, Vera F, Romero G. Dept of Pneumology and Pathology, La Fe University Hospital, Valencia, Spain.	Eur Respir J. 2002 May;19(5):976-9. Abstract quote   Scleromyxoedema is a rare systemic disorder characterized by a lichenoid papular rash. Although scleromyxoedema can involve any organ, very few cases of pulmonary involvement have been reported. Moreover, there are no reports in the literature on treatment of this condition, especially with lung transplantation. The authors report a case of scleromyxoedema in a young man with neurological, skin and respiratory involvement, the latter being mainly characterized by pulmonary emphysema. Due to the serious respiratory compromise, and to the stability of the systemic lesions, a bilateral lung transplantation was performed with successful results at 4.5 yrs. In conclusion, lung transplantation may be required in patients with severe respiratory failure caused by scleromyxoedema.
MUSCULAR	Slight to severe proximal muscle weakness is found in 27% of patients and is occasionally associated with slight elevation of muscle enzymes and inflammatory electromyographic findings Histology shows a nonspecific vacuolar myopathy with no or slight muscle fiber necrosis and interstitial inflammatory infiltrate Mucin deposition has been found in only 2 patients

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	The histopathology is distinct for scleromyxedema with collections of mucin associated with a marked proliferation of fibroblasts in the upper and mid-dermis There is only a scant perivascular infiltrate of lymphocytes Papular mucinosis has less distinct changes with less proliferation of fibroblasts

 

DIFFERENTIAL DIAGNOSIS	CHARACTERIZATION
NEPHROGENIC FIBROSING DERMOPATHY
Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. Kucher C, Xu X, Pasha T, Elenitsas R. Department of Pathology, University of Philadelphia Medical Center, Philadelphia, PA, USA.	J Cutan Pathol. 2005 Aug;32(7):484-90. Abstract quote   Background: Nephrogenic fibrosing dermopathy (NFD) clinically presents as indurated plaques and papules in patients with renal dysfunction. The differential diagnosis generally includes scleromyxedema (SMX), an idiopathic systemic disorder with cutaneous manifestations, in which patients also develop indurated papules and plaques. The two entities can be extremely difficult to distinguish microscopically. Histopathologic differences with immunophenotypic comparison, to our knowledge, have not been thoroughly studied. We compared these two entities with an emphasis on immunohistochemistry. Design: Nine biopsies diagnosed as NFD and seven biopsies diagnosed as SMX were retrospectively collected from the University of Pennsylvania Medical Center's surgical pathology and dermatopathology archives. Immunohistochemical staining for CD34, factor XIIIa, CD31, smooth muscle actin, CD68, and procollagen-I, as well as colloidal iron, were performed on each biopsy. Amount of expression for each of these markers, as well as degree of inflammation, for each biopsy was evaluated using a grading system of 0-3. Results: Overall, NFD and SMX showed similar expression for all markers except procollagen-I, which showed increased expression in SMX. Discussion: Although some immunophenotypic differences were found, our study did not demonstrate microscopic characteristics that can be easily used diagnostically to distinguish NFD from SMX. Clinical pathologic correlation is paramount in distinguishing these two entities.
SARCOMATOID CUTANEOUS LYMPHOMA
Cutaneous Sarcomatoid B-Cell Lymphoma. Ries S, Barr R, Leboit P, McCalmont T, Waldman J. From the *Department of Pathology, Memorial Medical Center of Long Beach, Long Beach, CA; daggerDermatopathology Laboratory, University of California Irvine Medical Center, Orange, CA; double daggerDermapathology Section, University of California, San Francisco, San Francisco, CA; and section signDepartments of Pathology and Dermatology, University of California at San Francisco, San Francisco, CA.	Am J Dermatopathol. 2007 Feb;29(1):96-98. Abstract quote A rare case of a spindle cell (sarcomatoid) B-cell lymphoma is described. The patient, a 48-year-old male, presented with a several month history of an enlarging lesion on the scalp. Although there have been a few recent reports of cutaneous sarcomatoid lymphomas, this case is especially unusual because it presented as a scarlike plaque rather than a tumor and microscopically exhibited a prominent myxoid matrix. Given these features, the lesion was initially interpreted as an atypical fibromucinosis. The differential diagnosis included fibromucinous lesion consistent with variant of lichen myxedematosus, spindle cell carcinoma, spindle cell melanoma, atypical fibroxanthoma, and atypical smooth muscle tumors. Initial immunoperoxidase studies demonstrated negative staining for CD68, factor XIIIa, CD57, cytokeratin(AE1/AE3), S100, EMA, and vimentin, essentially ruling out the previously mentioned neoplasms. Subsequently, strong positive staining for LCA(CD45RB) and CD20 was demonstrated characteristic of a B-cell lymphoma. The patient underwent local radiotherapy with complete resolution. Although all variants of cutaneous sarcomatoid B-cell lymphomas are rare, it is imperative to consider them in the differential diagnosis of otherwise difficult to categorize spindle cell proliferations. This includes neoplasms and, based on the current case, fibromucinoses as well.

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Prognostic Factors
Recurrence	Spontaneous improvement and resolution, even after 15 years, have been described
Treatment
Scleromyxedema	Topical and intralesional hyaluronidase Corticotropin Topical, intralesional, and systemic corticosteroids PUVA, Grenz ray, and electron beam therapy Retinoid Plasmapheresis Extracorporeal photochemotherapy Dermabrasion Topical dimethyl sulfoxide
	Many chemotherapeutic agents have been tried to interfere with the plasma cell dyscrasia. Low doses of melphalan with some clinical improvements, but have also been implicated in 30% of deaths due to hematologic malignancies and septic complications Granulocyte colony-stimulating factor Cyclosporine in another one Interferon alfa has been seen to produce both improvement and worsening in a case of localized LM
IMMUNOGLOBULIN
Scleromyxoedema: treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin. Kulczycki A, Nelson M, Eisen A, Heffernan M. Division of Allergy and Immunology, Department of Medicine, Washington School of Medicine. St Louis, MO, U.S.A.	Br J Dermatol. 2003 Dec;149(6):1276-81. Abstract quote   Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-lambda paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.
Intravenous immunoglobulins control scleromyxoedema. Righi A, Schiavon F, Jablonska S, Doria A, Blasczyk M, Rondinone R, Todesco S, Matucci Cerinic M. Department of Medicine, Division of Rheumatology, University of Florence, Italy.	Ann Rheum Dis. 2002 Jan;61(1):59-61. Abstract quote   BACKGROUND: Scleromyxoedema is a variant of papular mucinosis affecting the skin and internal organs. The different therapeutic approaches proposed for scleromyxoedema are still unsatisfactory. Intravenous immunoglobulin (IVIg) has been successfully employed in the treatment of connective tissue diseases and vasculitides. PATIENTS: The successful treatment of three cases of scleromyxoedema with IVIg is reported here. CONCLUSIONS: The relatively low risk of the drug and the high effectiveness seen in three patients suggest that IVIg is a new treatment potentially useful in scleromyxoedema.
Scleromyxedema: response to high-dose intravenous immunoglobulin (hdIVIg). Lister RK, Jolles S, Whittaker S, Black C, Forgacs I, Cramp M, Potter M, Rustin MH. Department of Dermatology, Royal Free Hospital, London, UK.	J Am Acad Dermatol 2000 Aug;43(2 Pt 2):403-8 Abstract quote We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2g/kg per month. The response to treatment was assessed using an objective skin scoring system initially established for patients with scleroderma. This system grades the overall severity of the induration and the reduction in mobility of the skin. Both patients initially had a dramatic response to treatment which was sustained in one patient. The first patient, a 30-year-old black man, showed a reduction in skin scores from 36/60 to 11/60 over a 3-month period, during which time he had 3 infusions of hdIVIg. After an unplanned 2-month break from treatment, severe neuromuscular complications developed. These improved initially with more frequent infusions of hdIVIg but oral corticosteroids were required to treat worsening myopathy. Unfortunately, the initial response to hdIVIg has not been sustained and his skin scores at 1 year returned to baseline. The second patient, a 60-year-old white man, showed a similarly dramatic reduction in skin scores from 36/60 to 15/60 over a 3-month period after having received only 2 infusions of hdIVIg. There has been sustained improvement after 10 months of therapy and the interval between hdIVIg infusions has been increased to 10 weeks without deterioration. HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis.
STEM CELL TRANSPLANTATION
Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. Lacy MQ, Hogan WJ, Gertz MA, Dispenzieri A, Rajkumar SV, Hayman S, Kumar S, Litzow MR, Schroeter AL. Division of Hematology and Internal Medicine, Department of Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minn 55905, USA.	Arch Dermatol. 2005 Oct;141(10):1277-82. Abstract quote   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.
Complete Remission of Scleromyxedema Following Autologous Stem Cell Transplantation Adrienne M. Feasel, MD; Michele L. Donato, MD; Madeleine Duvic, MD	Arch Dermatol 2001;137 No. 8 Abstract quote Scleromyxedema is characterized by dermal fibroblast proliferation and mucin deposition, associated with plasma cell dyscrasia. Therapy for systemic progression is often not effective, and the disease is potentially fatal. We describe a man with rapidly progressive scleromyxedema in whom multiple treatments had failed before complete remission was achieved with treatment with high-dose pulse dexamethasone, high-dose melphalan, and autologous stem cell transplantation.
THALIDOMIDE
Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. Sansbury JC, Cocuroccia B, Jorizzo JL, Gubinelli E, Gisondi P, Girolomoni G. Department of Dermatology and Pathology, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA.	J Am Acad Dermatol. 2004 Jul;51(1):126-31. Abstract quote   Scleromyxedema is a generalized, papular, and sclerodermoid form of lichen myxedematosus associated with monoclonal gammopathy and systemic changes. Despite anecdotal reports of success with various agents, no satisfactory treatments are currently available. We report 3 adult patients with recalcitrant scleromyxedema associated with paraproteinemia who were treated with thalidomide. All 3 patients had marked improvement of the skin lesions and joint mobility after the first 2 months of therapy, with further amelioration after 4 months, and reduction in paraprotein levels.

J Am Acad Dermatol 2001;44:273-81
Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001

Basic Principles of Disease
Learn the basic disease classifications of cancers, infections, and inflammation

Commonly Used Terms
This is a glossary of terms often found in a pathology report.

Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscope

Surgical Pathology Report
Examine an actual biopsy report to understand what each section means

Special Stains
Understand the tools the pathologist utilizes to aid in the diagnosis

How Accurate is My Report?
Pathologists actively oversee every area of the laboratory to ensure your report is accurate

Got Path?
Recent teaching cases and lectures presented in conferences

• Pathologists Who Make A Difference
• Search for a Physician Specialist
• Clinical Photo
• Clinical Photo
• Clinical Photo
• Microscopic Photo
• Microscopic Photo

Last Updated February 8, 2007

Send mail to The Doctor's Doctor with questions or comments about this web site.
Read the Medical Disclaimer.

Copyright © The Doctor's Doctor