Reticular Erythematous Mucinosis Research Articles

Reticular Erythematous Mucinosis: A Case Report and Brief Review

Reticular erythematous mucinosis is a rare entity characterised by mucin deposition in the skin. It has been reported in association with a few autoimmune diseases such as type 1 diabetes mellitus, systemic lupus erythematosus, systemic sclerosis, Hashimoto’s thyroiditis and idiopathic thrombocytopenic purpura. It is commonly misdiagnosed and treated as photodermatitis due to its subtle presentation. The case is being reported due to its rarity.

A Peculiar Reticular Rash of The Extremities: Think of Reticular Erythematous Mucinosis

A Peculiar Reticular Rash of The Extremities: Think of Reticular Erythematous Mucinosis

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

BackgroundSince 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.ObjectiveTo 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.MethodsWe 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.ResultsWe 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.ConclusionOver 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.

Identifying the potential origin of mucin in primary cutaneous mucinoses-A retrospective study and analysis using histopathology and multiplex fluorescence staining.

Primary cutaneous mucinoses (PCM) are rare diseases characterized by dermal or follicular mucin deposits. A retrospective study characterizing PCM to compare dermal with follicular mucin to identify its potential origin on a single-cell level. Patients diagnosed with PCM between 2010 and 2020 at our department were included in this study. Biopsy specimens were stained using conventional mucin stains (Alcian blue, PAS) and MUC1 immunohistochemical staining. Multiplex fluorescence staining (MFS) was used to investigate which cells were associated with MUC1 expression in select cases. Thirty-one patients with PCM were included, 14 with follicular mucinosis (FM), 8 with reticular erythematous mucinosis, 2 with scleredema, 6 with pretibial myxedema and one patient with lichen myxedematosus. In all 31 specimens, mucin stained positive for Alcian blue and negative for PAS. In FM, mucin deposition was exclusively found in hair follicles and sebaceous glands. None of the other entities showed mucin deposits in follicular epithelial structures. Using MFS, all cases showed CD4+ and CD8+ T cells, tissue histiocytes, fibroblasts and pan-cytokeratin+ cells. These cells expressed MUC1 at different intensities. MUC1 expression in tissue histiocytes, fibroblasts, CD4+ and CD8+ T cells, and follicular epithelial cells of FM was significantly higher than the same cell types in the dermal mucinoses (p < 0.001). CD8+ T cells were significantly more involved in expression of MUC1 than all other analysed cell types in FM. This finding was also significant in comparison with dermal mucinoses. Various cell types seem to contribute to mucin production in PCM. Using MFS, we showed that CD8+ T cells seem to be more involved in the production of mucin in FM than in dermal mucinoses, which could indicate that mucin in dermal and follicular epithelial mucinoses have different origins.

A case of reticular erythematous mucinosis successfully treated with thalidomide

A case of reticular erythematous mucinosis successfully treated with thalidomide

New and emerging conditions of acquired cutaneous mucinoses in adults.

Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti-colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement.

Reticular Erythematous Mucinosis Successfully Treated with Laser in a Male Patient with Systemic Lupus Erythematosus.

Reticular Erythematous Mucinosis Successfully Treated with Laser in a Male Patient with Systemic Lupus Erythematosus.

DP20: Reticular erythematous mucinosis: a rare case of primary dermal mucinosis

DP20: Reticular erythematous mucinosis: a rare case of primary dermal mucinosis

A case report of a localized reticular erythematous mucinosis like eruption of the lower legs mimicking cutaneous larvae migrans.

Reticular erythematous mucinosis is an uncommon type of cutaneous mucinosis. Typically, reticular erythematous mucinosis affects middle-aged women and usually appears as papules and plaques on the mid-upper trunk. Histologically, biopsies of reticular erythematous mucinosis reveal increased deposition of dermal mucin and perivascular lymphocytic inflammation. Infrequently, reticular erythematous mucinosis has been reported in males and in atypical locations other than the trunk. In these instances, reticular erythematous mucinosis can present a diagnostic challenge clinically and histologically. This case describes the clinical and histologic findings of a localized variant of reticular erythematous mucinosis in a 65-year-old male patient.

A Case of Reticular Erythematous Mucinosis with Systemic Lupus Erythematosus

A Case of Reticular Erythematous Mucinosis with Systemic Lupus Erythematosus

Reticular erythematous mucinosis-Aspecial subtype of cutaneous lupus erythematosus?

Reticular erythematous mucinosis (REM syndrome) is arare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Extracutaneous diseases and involvement of internal organs do not occur in REM syndrome. Histopathological features include perivascular and periadnexal lymphocytic inflammatory infiltrates as well as increased deposition of mucin in the dermis. Due to the similar histology to lupus erythematosus (LE) tumidus, it is discussed whether REM syndrome can be assigned to the spectrum of cutaneous LE or can be assessed as a lupus-like disease. Moreover, both conditions respond well to treatment with hydroxychloroquine.

Evaluating results of an interferon-γ release assay in patients with autoimmune disease who are taking hydroxychloroquine

Evaluating results of an interferon-γ release assay in patients with autoimmune disease who are taking hydroxychloroquine

Reticular Erythematous Mucinosis (REM): Case Report and Review of Literature

Reticular erythematous mucinosis (REM) is a rare disorder with an unknown etiology. REM requires both a clinical and a pathological diagnosis. Multiple relevant associations and triggers are known. Herein, we report a classic case of REM, whose condition was found to be correlated with smoking and exacerbated by sun exposure. Our diagnostic and treatment approach is based on a worldwide review of current research, and this report aims to demonstrate a classical presentation of REM and available treatment options that have proved efficacious to date.

Pathomorphological changes in patients with Jessner>s lymphocytic infiltrate of skin, reticular erythematous mucinosis and lupus tumidus

Pathomorphological changes in patients with Jessner>s lymphocytic infiltrate of skin, reticular erythematous mucinosis and lupus tumidus

ステロイド内服・外用が奏効した Reticular Erythematous Mucinosis の1例

ステロイド内服・外用が奏効した Reticular Erythematous Mucinosis の1例

Pathomorphological changes in patients with Jessner&gt;s lymphocytic infiltrate of skin, reticular erythematous mucinosis and lupus tumidus

Jessner’s lymphocytic infiltrate of skin, reticular erythematous mucinosis and lupus tumidus are the group of rare, insufficiently explored dermatosis with common clinical and histological features, whose nosology is controversial today. Plasmacytoid dendritic cells play the key role in autoimmune disease induction The target of our study was the analysis of pathomorphological features of lesional skin and the research of plasmacytoid dendritic cells in the skin of patients with Jessner’s lymphocytic infiltrate of skin, reticular erythematous mucinosis and lupus tumidus. Material and methods. Histological and indirect immunohistochemistry examination (anti-CD123 antibodies) of lesional skin of 36 patients was held (JLIS - 12, REM - 10, LT - 14) and 10 healthy people. Result. The main pathomorphological changes of skin in all groups were found in dermis: dense perivascular and perifollicular infiltration and deposit of mucin between collagen fibers. Deposits of mucin in papillary dermis were found between patients with JLIS, REM and LT in 10/12 (83%), 7/10 (70%) and 12/14 (86%) of cases, there were no mucin found in skin of healthy people 0/10 (0%) In all dermatosis the distribution of CD123 cells in lesional skin, in immunohistochemistry study, was corresponded clusters model of infiltrate - plasmacytoid DC concentrate in groups around vessels of superficial and profundus networks and perivascular. The comparative analysis of density and localization of perivascular infiltration, number of CD123-cells and their correspondence in dermis perivascular infiltrates didn’t reveal statistically significant differences between JLIS, REM and LT. Results. Our results allow us to consider JLIS and LT as identical decease, and REM as similar in clinical-pathomorphological features dermatosis.

Reticular erythematous mucinosis

This article presents modern data related to the clinical picture, pathologic features and treatment options of reticular erythematous mucinosis (REM). There is an opinion that REM, lupus erythematosus tumidus and Jessner - Kanof lymphocytic infiltration of the skin are similar nosological forms.

Reticular Erythematous Mucinosis and Lupus Erythematosus Tumidus — Different Diseases or Variations on a Theme?

Both reticular erythematous mucinosis (REM) and lupus erythematosus tumidus (LET) are characterized by erythematous papules and plaques and

MUCINOSES CUTÂNEAS PRIMÁRIAS – REVISÃO CLÍNICOPATOLÓGICA

Introdução: As mucinoses cutâneas são doenças raras, caracterizadas pela deposição excessiva de mucina na pele. Podem ser primárias ou secundárias e associarem-se a paraproteinémia, diabetes mellitus, doenças tiroideias ou auto-imunes. O objectivo deste estudo consistiu em rever e caracterizar as diferentes mucinoses cutâneas primárias diagnosticadas no nosso Serviço visando facilitar o seu reconhecimento clínico e patológico.Material e Métodos: Realizou-se estudo retrospectivo dos exames histopatológicos dos doentes com diagnóstico de mucinose primária, no período compreendido entre Janeiro de 2000 e Dezembro de 2012. Foram avaliados dados demográficos, clínicos e histológicos e realizada revisão da literatura.Resultados: Foram diagnosticados 15 casos de mucinoses em 8 mulheres e 7 homens. As mucinoses diagnosticadas foram: líquen mixedematoso (7/15), mucinose eritematosa reticular (3/15), escleredema (3/15) e mixedema pré-tibial (2/15). Dentro da primeira distinguiram-se as formas papulosa discreta (3/7), mucinose papular acral persistente (2/7), nodular (1/7) e atípica (1/7). Encontrou-se associação com hipertiroidismo (nos casos de mixedema prétibial) e cirrose biliar primária, hepatite auto-imune e mieloma múltiplo (em 2 dos casos de escleredema).Conclusões: O número limitado de casos diagnosticados explica-se pela raridade destas condições. Com base nos resultados obtidos realizou-se revisão anátomo-clínica com especial ênfase para o tipo de lesão, distribuição e evolução, assim como a presença ou ausência de manifestações sistémicas ou associações patológicas. Em relação à histopatologia os dados fundamentais para o diagnóstico diferencial são o padrão de distribuição da mucina, grau de proliferação fibroblástica, morfologia das fibras de colagénio, número de fibras elásticas e presença ou ausência de fibrose.

Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases

Reticular erythematous mucinosis (REM) is an uncommon disease, the nosology and specific characteristics of which are controversial because most reports deal with single cases or small series. To describe the characteristics of patients with REM regarding demographics, clinical and pathological features, comorbidities, treatment and course. A retrospective and prospective study was conducted on 25 patients diagnosed with REM in the setting of university-affiliated dermatology departments and dermatopathology centres. Of the 25 patients with REM, 16 were women (sex ratio 2 : 1) and the mean age was 46 years. The roles of sun exposure and oral contraceptives were ambiguous. Associated diseases included hypertension (n = 4), malignancies (n = 3), autoimmune diseases (n = 3) and Borrelia infection (n = 1). Immunological studies (including serology and direct immunofluorescence) were noncontributory. The response to antimalarial treatment was good in > 80% of cases. Worsening or recurrence of the lesion after treatment discontinuation, or in the course of the disease, occurred in 31% of patients. We present the largest REM case series to date. The reticular pattern with involvement of the midline of the chest and back, the predilection for middle-aged women, the controversial relationship with photosensitivity and the possible association with other conditions such as malignancies and thyroid dysfunctions are the main characteristics that makes REM a recognizable disease.