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Indian Dermatology Online Journal 2019Scleromyxedema is a rare progressive cutaneous mucinosis of unknown etiology with equal prevalence in both men and women. It is usually associated with monoclonal...
Scleromyxedema is a rare progressive cutaneous mucinosis of unknown etiology with equal prevalence in both men and women. It is usually associated with monoclonal gammopathy in most of the cases. Various treatment modalities have been tried for scleromyxedema including steroids, intravenous immunoglobulin (IVIg), autologous hematopoietic stem cell transplantation, and melphalan, but none has proved to be fully effective. This paper reports a case of scelromyxedema in a patient who was found to have multiple myeloma on further evaluation. The patient was treated with six cycles of bortezomib, thalidomide, and dexamethasone, following which he had complete resolution of his skin lesions. While recent case reports have mentioned treatment of patients of scleromyxedema with varying combinations of IVIg, thalidomide, bortezomib, and dexamethasone, we describe a patient who has been successfully treated with a combination of bortezomib, thalidomide, and dexamethasone with complete resolution of all skin lesions.
PubMed: 30775299
DOI: 10.4103/idoj.IDOJ_135_18 -
Clinical Case Reports Dec 2020Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an...
Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease.
PubMed: 33363876
DOI: 10.1002/ccr3.3302 -
Actas Dermo-sifiliograficas Nov 2005Scleromyxedema is a type of mucinosis that presents with some well defined clinical and histopathological characteristics. We describe the findings observed with...
Scleromyxedema is a type of mucinosis that presents with some well defined clinical and histopathological characteristics. We describe the findings observed with transmission electron microscopy in a case that we recently studied. The patient was a 56-year-old female with a localized eruption of lichenoid papules on the face, upper trunk and limbs. It was accompanied by IgG lambda gammopathy. Optical microscopy showed the findings typical of scleromyxedema. Electron microscopy revealed the existence of a large number of fibroblasts with high activity levels in the synthesis and release of collagen fibers and a mucoid substance.
Topics: Female; Humans; Microscopy, Electron; Middle Aged; Myxedema; Skin Diseases
PubMed: 16476310
DOI: 10.1016/s0001-7310(05)73148-3 -
Proceedings of the Royal Society of... Mar 1961
Topics: Humans; Lichen Planus; Medical Records; Myxedema; Scleromyxedema
PubMed: 13749383
DOI: No ID Found -
Dermatology Online Journal Oct 2018Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We... (Review)
Review
Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We report a case of CMI and review the prior 13 cases in the literature. The patient was a 5-year-old girl who presented with asymptomatic dermal papules and plaques on her leg and back with no overlying color change. These lesions were first noticed during infancy and had become slightly more evident over time. The patient had a history of birthmarks and eczema. Her family history included eczema, allergies, photosensitivity, and Graves disease. Pre-biopsy clinical differential diagnosis included connective tissue nevus, granuloma annulare, myofibroma, lipofibroma, and lymphangioma. Biopsies revealed significant increase in interstitial mucin within the reticular and mid dermis, without significant sclerosis or fibroblastic proliferation. The relatively quiescent pattern of interstitial mucinosis with slight fibrocyte hyperplasia presenting as dermal papules-plaques on the trunk and extremities was most consistent with a diagnosis of CMI. We report another case of CMI in an otherwise healthy patient. Our patient is unique as she is the first CMI patient with a family history of Graves disease, although our patient appeared euthyroid. We will also review the literature on this rare entity.
Topics: Biopsy; Child, Preschool; Diagnosis, Differential; Female; Granuloma Annulare; Graves Disease; Humans; Medical History Taking; Mucinoses; Myofibroma; Nevus; Scleromyxedema; Skin Neoplasms
PubMed: 30677812
DOI: No ID Found -
Discrete Papular Lichen Myxedematosus and Scleromyxedema with Hypothyroidism: A Report of Two Cases.Case Reports in Dermatology 2019Scleromyxedema and lichen myxedematosus (LM) are rare disorders that fall along the spectrum of primary cutaneous mucinoses. Scleromyxedema is a systemic form that...
Scleromyxedema and lichen myxedematosus (LM) are rare disorders that fall along the spectrum of primary cutaneous mucinoses. Scleromyxedema is a systemic form that classically presents with generalized waxy papules, sclerodermoid eruption, and monoclonal gammopathy; LM is a localized form limited to the skin that classically presents with white, firm, waxy papules and lacks monoclonal gammopathy. According to diagnostic criteria established in 2001, the diagnosis of both conditions requires absence of thyroid disease. However, atypical cases that lack monoclonal gammopathy and that present with hypothyroidism have been reported, suggesting that these criteria may require revision. First, we report a case of a 58-year-old female with a history of Hashimoto thyroiditis and biopsy-proven scleromyxedema responsive to intravenous immunoglobulin therapy with delayed presentation of monoclonal gammopathy. Next, we report a case of a 54-year-old female with a history of hypothyroidism, Hodgkin's lymphoma in remission after radiation and chemotherapy, and concurrent rheumatoid arthritis, with biopsy-proven LM temporarily responsive to systemic steroids. Our cases demonstrate that patients with papular mucinoses can have a multitude of concurrent and prior rheumatologic and endocrine conditions, including thyroid disease, which should not preclude a diagnosis of scleromyxedema and LM.
PubMed: 31011315
DOI: 10.1159/000498881 -
Anais Brasileiros de Dermatologia 2016Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal...
Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.
Topics: Cell Proliferation; Dermis; Fibroblasts; Humans; Male; Middle Aged; Mucins; Scleromyxedema
PubMed: 28099617
DOI: 10.1590/abd1806-4841.20165725 -
Skin Therapy Letter Jun 2013The skin is often a window to systemic disease that is available to the trained eye of the dermatologist. Herein, we focus on four dermatoses with associated systemic...
The skin is often a window to systemic disease that is available to the trained eye of the dermatologist. Herein, we focus on four dermatoses with associated systemic conditions of interest: scleromyxedema and monoclonal gammopathy, nephrogenic systemic fibrosis in the setting of renal insufficiency, dermatitis herpetiformis and celiac disease, and psoriasis as a risk factor for cardiovascular disease. Dermatologists can play a crucial role in recognizing the cutaneous manifestations linked with these conditions. Identifying the related underlying disorder will contribute to appropriate diagnosis and improved management.
Topics: Cardiovascular Diseases; Celiac Disease; Dermatitis Herpetiformis; Humans; Nephrogenic Fibrosing Dermopathy; Paraproteinemias; Psoriasis; Renal Insufficiency; Scleromyxedema
PubMed: 24310641
DOI: No ID Found -
JAAD Case Reports May 2019
PubMed: 31193003
DOI: 10.1016/j.jdcr.2019.03.005 -
Frontiers in Medicine 2023Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after...
The realistic positioning of UVA1 phototherapy after 25 years of clinical experience and the availability of new biologics and small molecules: a retrospective clinical study.
BACKGROUND
Since the early 1990s, Ultraviolet (UV) A1 phototherapy has been described as an effective and safe treatment of a multitude of skin disorders. However, after 30 years, its use has remained limited to few dermatological centers.
OBJECTIVE
To analyze the changes over the years and the current position of UVA1 phototherapy through a Real-World Evidence (RWE) study at a single tertiary referral center.
METHODS
We reviewed the medical files of 740 patients treated between 1998 and 2022. Treatment results were collected, efficacy was assessed by a grading scale and acute adverse effects were registered.
RESULTS
We treated patients with 26 different diseases. We registered marked improvement (MI) or complete remission (CR) in 42.8% of patients with morphea, 50% with Urticaria Pigmentosa, 40.7% with Granuloma annulare and 85.7% with skin sarcoidosis. Good results were obtained also in the treatment of chronic Graft Versus Host Disease (GVHD), Eosinophilic Fasciitis, Sclero-atrophic Lichen, skin manifestations of systemic lupus erythematosus and psoriasis of HIV+ patients. Systemic Sclerosis, Romberg's Syndrome, Bushke's Scleredema, Nephrogenic Fibrosing Dermopathy, REM Syndrome, Follicular Mucinosis, Pretibial Myxedema, Scleromyxedema, pemphigus foliaceus, chronic cutaneous lupus erythematosus, erythroderma of Netherton Syndrome and Necrobiosis Lipoidica were no or poorly responsive. In clinical indications where UVA1 was used as a second line phototherapy after narrow-band (NB)-UVB, we saw good MI or CR rates in Mycosis Fungoides (57% of patients), Atopic Dermatitis (33.9%), Pitiryasis Lichenoides chronica (50%), Pityriasis Lichenoides et varioliformis acute (75%) and Lymphomatod Papulosis (62.5%). Short-term adverse events were uncommon and mild.
CONCLUSION
Over the past decade, the annual number of treated patients has progressively declined for several reasons. Firstly, UVA1 phototherapy has taken a backseat to the cheaper and more practical NB-UVB phototherapy, which has proven effective for common indications. Secondly, the emergence of new, safe, and effective drugs for conditions such as atopic dermatitis, GVHD, and connective tissue disorders. Finally, our research has shown that UVA1 therapy is often ineffective or minimally effective for some rare diseases, contrary to previous case reports and small case series. Nonetheless, UVA1 continues to be a valuable treatment option for patients with specific skin disorders.
PubMed: 38076241
DOI: 10.3389/fmed.2023.1295145