Reticular erythematous mucinosis – (REM syndrome) in twins

Fühler, Maren; Ottmann, Karl; Tronnier, Michael

doi:10.1111/j.1610-0387.2009.07138.x

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…Less common histologic findings associated with REM include mildly thinned epidermis with reduced rete ridges, 3,7 dermal vacuolated cells with a clear cytoplasm and a large basophilic hyperchromatic nucleus, 8 dermal and dermoepidermal junction deposits of IgA, IgM, and C3, 9 and intracytoplasmic tubular aggregates in endothelial and periendothelial cells of dermal vessels, as well as dermal histiocytes and epidermal keratinocytes via electromicroscopy. [10][11][12][13] Laboratory investigations with normal results performed at the time of dermatologic consultation included antinuclear antibody (ANA), extracted nuclear antigen (ENA) screen, and complete blood count (CBC).…”

Section: Case Presentationmentioning

confidence: 99%

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

Kenny

Lichtenwald

Wright

et al. 2021

SAGE Open Medical Case Reports

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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.

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Section: Case Presentationmentioning

confidence: 99%

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

Kenny

Lichtenwald

Wright

et al. 2021

SAGE Open Medical Case Reports

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“…The initial appearance of REM in children is uncommon [1][2][3]. The majority of reported cases were sporadic with only a few isolated familial reports described [4,5].…”

Section: Introductionmentioning

confidence: 99%

Reticular Erythematous Mucinosis in an African Woman with HIV Infection: Case Report and Literature Review

Tenea,

Campaini

2023

Case Rep Dermatol

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Reticular erythematous mucinosis is a rare and persistent form of primary idiopathic mucinosis, often referred to as plaque-like cutaneous mucinosis or midline mucinosis. It presents with reticulate patches or erythematous plaques with predilection for the anterior and posterior trunk. Affected patients are frequently asymptomatic. Pruritus or burning sensations were reported after exposure to the sun. The aetiology remains obscure; its pathogenesis is poorly understood, particularly in immunocompromised patients such as HIV-infected patients. The disease associations are not uniformly documented. Antimalarial agents significantly improve and shorten the course of the disease. We report a case of a 31-year-old African woman with underlying HIV infection who displayed the classical clinical and histological features of reticular erythematous mucinosis. This condition is rare among the HIV-infected patients, particularly in those of African descent, in whom lichen myxoedematosus/scleromyxoedema variants and acral persistent papular mucinoses were most frequently reported. The higher incidence of photosensitivity in HIV-infected individuals including the patients with skin of colour may play a potential role in reticular erythematous mucinosis. Its relationship with lupus erythematosus and photosensitivity in the context of HIV infection is discussed. To the best of our knowledge, this is the first reported case of reticular erythematous mucinosis in an African HIV-infected patient. This case highlights the need for diagnostic awareness in cases presenting with erythematous plaques and patches in a net-like pattern developing on the midline and sun-exposed areas of the trunk.

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“…However, to our knowledge, few case reports exist on the use of chloroquine or hydroxychloroquine in REM syndrome, with varying degree of response. 1,[11][12][13][14][15] We therefore initiated the present retrospective study using a clinical scoring system to evaluate the clinical characteristics and the efficacy of antimalarial therapy in a series of German patients with REM syndrome.…”

mentioning

confidence: 99%

Clinical Features and Efficacy of Antimalarial Treatment for Reticular Erythematous Mucinosis

Kreuter¹,

Scola²,

Tigges³

et al. 2011

Arch Dermatol

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Background: Reticular erythematous mucinosis (REM syndrome) is a rare cutaneous disease that predominantly affects the chest and upper back area of middleaged women. Although antimalarial treatment is generally considered the most effective approach, only a few case reports exist on its use in REM syndrome. Observations: A total of 11 patients with REM syndrome (10 women and 1 man), mean age, 44 years (age range, 37-54 years), were included in this retrospective analysis. Ten of the 11 patients were cigarette smokers (91%), and 6 had concomitant autoimmune diseases (55%). Since no clinical score exists for REM syndrome, we used the validated Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) to evaluate the efficacy of antimalarial treatment. Overall, a significant decrease in the clinical score was observed from a median of 4 (range, 2-8) before initiation of treatment to 0 (range, 0-4) after 3 months of antimalarial therapy and to 0 (range, 0-4) after 12 months of therapy (PϽ.001). Two patients withdrew from the study owing to adverse gastrointestinal tract effects (nausea and vomiting); 2 relapsed after finishing their antimalarial regimens; 3 patients were free of disease 2 years after the end of treatment; and 4 patients were lost to follow-up. Conclusion: Antimalarial agents significantly improve or completely clear the skin lesions of patients with REM syndrome and should be considered as a first-line therapy for this rare disease.

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Reticular erythematous mucinosis – (REM syndrome) in twins

Cited by 5 publications

References 11 publications

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

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

Reticular Erythematous Mucinosis in an African Woman with HIV Infection: Case Report and Literature Review

Clinical Features and Efficacy of Antimalarial Treatment for Reticular Erythematous Mucinosis

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