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Journal Der Deutschen Dermatologischen... Mar 2024
Unusual variant of scleromyxedema presenting with severe systemic involvement and atypical adnexal proliferations - A histological pitfall with the risk of unnecessary surgeries.
Topics: Humans; Scleromyxedema; Granuloma Annulare; Diagnosis, Differential
PubMed: 38200613
DOI: 10.1111/ddg.15306 -
JAMA Dermatology Mar 2024
Topics: Humans; Scleromyxedema; Mucinoses
PubMed: 38170481
DOI: 10.1001/jamadermatol.2023.4976 -
JAAD Case Reports Dec 2023
PubMed: 38156097
DOI: 10.1016/j.jdcr.2023.10.010 -
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 -
RMD Open Nov 2023
Topics: Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Scleredema Adultorum; Scleromyxedema; Aged; Male
PubMed: 38016711
DOI: 10.1136/rmdopen-2023-003638 -
The New England Journal of Medicine Nov 2023
Topics: Humans; Immunoglobulins, Intravenous; Scleromyxedema
PubMed: 37991858
DOI: 10.1056/NEJMicm2302207 -
The American Journal of Dermatopathology Jan 2024Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be... (Review)
Review
Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.
Topics: Humans; Scleromyxedema; Skin; Skin Diseases; Hand; Upper Extremity
PubMed: 37982501
DOI: 10.1097/DAD.0000000000002589 -
Retinal Cases & Brief Reports Nov 2023We discuss a case of macular edema and retinal hemorrhage associated with scleromyxedema.
PURPOSE
We discuss a case of macular edema and retinal hemorrhage associated with scleromyxedema.
METHODS
A case report is presented.
RESULTS
A 64-year-old male with history of deep vein thrombosis and pulmonary embolism presented with new onset rash in the setting of switching anticoagulation treatments. He developed blurred vision was found to have macular edema and dot blot retinal hemorrhages which improved with systemic and topical corticosteroids.
CONCLUSIONS
Systemic autoimmune conditions including scleromyxedema should be considered in the workup of occult cystoid macular edema.
PubMed: 37910641
DOI: 10.1097/ICB.0000000000001510 -
The Journal of Dermatology Jan 2024Scleromyxedema (SMX) is a rare disease of unknown cause. It is a chronic, progressive, metabolic disorder characterized by a generalized papular and scleroderma-like...
Scleromyxedema (SMX) is a rare disease of unknown cause. It is a chronic, progressive, metabolic disorder characterized by a generalized papular and scleroderma-like rash, as well as a subtype of lichen myxedematosus. Dermato-neuro syndrome (DNS) is a rare neurological complication of SMX. It has flu-like prodromal symptoms; consists of a triad of fever, coma, and seizures; and can be life-threatening. We describe a patient with SMX complicated by DNS after infection with COVID-19. Her symptoms resolved after treatment with acyclovir and low-dose glucocorticoids, suggesting that DNS seizures may have a viral cause. Her skin lesions also improved after seven courses of intravenous immunoglobulin treatment, confirming that intravenous immunoglobulin is effective in these cases.
Topics: Humans; Female; Immunoglobulins, Intravenous; Scleromyxedema; COVID-19; Syndrome; Seizures
PubMed: 37830263
DOI: 10.1111/1346-8138.16994 -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221