Systemic lupus erythematosus presenting with a reticular erythematous                 mucinosis-like condition

Pozo, Jesús del; Peña, C Fernández; Almagro, Manuel; Yebra, Miguel; Martínez, Walter

doi:10.1191/096120300678828028

Cited by 27 publications

(26 citation statements)

References 8 publications

(5 reference statements)

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“…In the literature, only one woman with reticular lesions clinically and pathologically consistent with REM developed features fulfilling the diagnostic criteria of SLE, 6 years later. 10 Most of our patients were asymptomatic, although pruritus has been previously reported to occur in 20-30% of cases. The role of sunlight was confirmed to be controversial, as sun exposure worsened or triggered REM in five patients, but also improved the disease in another four.…”

Section: Discussionmentioning

confidence: 88%

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

et al. 2013

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SummaryBackground 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. Objectives To describe the characteristics of patients with REM regarding demographics, clinical and pathological features, comorbidities, treatment and course. Methods A retrospective and prospective study was conducted on 25 patients diagnosed with REM in the setting of university-affiliated dermatology departments and dermatopathology centres. Results 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. Conclusions 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.

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Section: Discussionmentioning

confidence: 88%

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

et al. 2013

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“…4,7,11 Some of these mucin deposition disorders occur in association with lupus erythematosus, and in some cases may be its initial presenting sign. 1,7,10 Papulonodular mucinosis, also known as cutaneous lupus mucinosis, is an uncommon eruption consisting of skin-colored papules and nodules usually on the upper extremities and trunk. 4,11 This is a specific eruption seen mostly in patients with SLE.…”

Section: Discussionmentioning

confidence: 99%

“…1 Cutaneous mucinosis is a rare group of skin disorders characterized by a prominent dermal mucin deposition [2][3][4][5][6] that may present in association with variants of lupus erythematosus. 2,4,[7][8][9][10] Papulonodular mucinosis, also known as cutaneous lupus mucinosis, is a wellknown example of this. 4,7,11 Periorbital swelling secondary to dermal mucin deposition has been described in lupus erythematosus [12][13][14][15][16][17] ; however, it is not widely recognized as one of its cutaneous manifestations.…”

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confidence: 99%

Periorbital mucinosis: A variant of cutaneous lupus erythematosus?

Morales-Burgos

Sánchez

Gonzalez-Chávez

et al. 2010

Journal of the American Academy of Dermatology

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“…It is probable that the clinico-pathologic entities papulonodular mucinosis and reticular erythematous mucinosis (REM) syndrome are also dermal variants of LE [30,31,32,33,34,35]. Thus, dermal LE is characterized either by an exclusive lymphocytic infiltrate (JLI), or by predominant mucin deposition (papulonodular mucinosis, REM syndrome), or a mixture or both (LT) [36].…”

Section: Discussionmentioning

confidence: 99%

Could Jessner’s Lymphocytic Infiltrate of the Skin Be a Dermal Variant of Lupus Erythematosus? An Analysis of 210 Cases

et al. 2006

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Background and Objective: The objective of this study was to evaluate the relationship between Jessner’s lymphocytic infiltration of the skin (JLI) and lupus erythematosus (LE), which has been the subject of debate since its initial description in 1953. Material and Methods: This is a retrospective study including all patients with a histopathologically ascertained diagnosis of JLI performed at the Laboratoire d’Histopathologie Cutanée of the Strasbourg University Hospital between 1993 and 2003. Information about patient characteristics and follow-up data were retrieved between 2004 and 2005. Special attention was paid to features indicative of LE. Results: 210 consecutive patients (102 women and 108 men) with a mean age 42 years were diagnosed with JLI in the reference period. 175 patients (83%) had multiple lesions and 32 patients (15%) had only a single lesion at the time of diagnosis (data not available in 3 patients). The head, neck and upper part of the thorax were involved in 171 patients (81%). An annular or arciform configuration and/or arrangement were present in 111 patients (53%). Lesions consisted of red (100%) papules or plaques (98%). Mean follow-up was 4 years. Sixteen patients (7.6%) had proven LE. Only 2 patients (1%) developed >4 ACR criteria of systemic LE. Furthermore, 1 patient had antiphospholipid antibody syndrome and 2 patients had rheumatoid arthritis. Conclusions: This high frequency of patients with typical features of LE strongly argues that JLI could be a dermal variant of LE and not an autonomous entity. It might be the cutaneous marker of a subset of LE patients with excellent prognosis.

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Systemic lupus erythematosus presenting with a reticular erythematous mucinosis-like condition

Cited by 27 publications

References 8 publications

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

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

Periorbital mucinosis: A variant of cutaneous lupus erythematosus?

Could Jessner’s Lymphocytic Infiltrate of the Skin Be a Dermal Variant of Lupus Erythematosus? An Analysis of 210 Cases

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