Secondary erythromelalgia involving the ears probably preceding lupus erythematosus

Badeloe, Sadhanna; Henquet, C. J. M.; Nieuwhof, Chris; Frank, Jorge

doi:10.1111/j.1365-4632.2007.03500.x

Cited by 14 publications

(18 citation statements)

References 10 publications

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“…Other LE nonspecific lesions Other lesions include: calcinosis cutis, acanthosis nigricans, hypocomplementaemic urticarial vasculitis (very rare in JSLE patients) [62–64], whilst rheumatoid nodules, anetoderma and erythromelalgia have been reported in adult SLE [65–67]. …”

Section: Reviewmentioning

confidence: 99%

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

et al. 2015

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Patients diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) often have skin and oral lesions as part of their presentation. These mucocutaneous lesions, as defined by the American College of Rheumatology (ACR) in 1997, include malar rash, discoid rash, photosensitivity and oral ulcers. It is therefore essential to recognize mucocutaneous lesions to accurately diagnose JSLE. The mucocutaneous lesions can be divided into those with classical histological features (LE specific) and those strongly associated with and forming part of the diagnostic spectrum, but without the classical histological changes of lupus (LE nonspecific). A malar rash is the most commonly associated LE specific dermatological presentation. This skin manifestation is an acute form and also correlates with disease activity. Subacute (polycyclic or papulosquamous lesions) and chronic (discoid lesions) forms, whilst showing classical histological changes supportive of lupus, are less commonly associated with systemic lupus and do not correlate with disease activity. The most commonly associated skin lesions without classical lupus changes are cutaneous vasculitis, oral ulcers and diffuse non-scarring alopecia. These signs frequently relate to disease activity. An understanding of cutaneous signs and symptoms of lupus in children is important to avoid delay in diagnosis. They will often improve as lupus is adequately controlled and their reappearance is often the first indicator of a disease flare.

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Section: Reviewmentioning

confidence: 99%

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

et al. 2015

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“…2 The disease manifests itself primarily in the feet and the hands but it can also affect the ears although less frequently. 1 Heat exposure, physical exercises, gravity and the use of stockings/socks and gloves can act as triggers of the symptoms or intensify the discomfort while cold causes discomfort relief. [3][4][5] The disease takes typically a chronic course and it is associated with a decrease in the quality of life and considerable morbidity.…”

Section: Introductionmentioning

confidence: 99%

“…Familiar erythromelalgia of juvenile onset is associated with mutations in the gene SCN 9 A that codifies a voltage-dependent sodium channel and it is more frequent in male. 1,5 Secondary erythromelalgia can be associated with various disorders like thrombocytopenia, polycythemia, myeloproliferative disorders, hypertension, vasculitis, systemic lupus erythematous, scleroderma, rheumatoid arthritis, Raynaud's disease, HIV and gout.…”

Section: Introductionmentioning

confidence: 99%

“…6 Erythromelalgia can be classified as primary or secondary, depending that if the absence or presence respectively of associated diseases that may precede it, coincides with the beginning of the disease or occurs during its evolution. 1 The primary form can be classified in familiar (autosomal dominant) or sporadic, and in juvenile onset (before 20 years of age, frequently before 10 years) or adult onset. Familiar erythromelalgia of juvenile onset is associated with mutations in the gene SCN 9 A that codifies a voltage-dependent sodium channel and it is more frequent in male.…”

Section: Introductionmentioning

confidence: 99%

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Eritromelalgia primária: relato de caso

Albuquerque¹,

França

Kozmhinsky

et al. 2011

An. Bras. Dermatol.

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Erythromelalgia is a rare clinical syndrome characterized by heat, redness and intermittent pain in the extremities, being most frequent the bilateral development in the lower extremities. Local cooling brings relief to symptoms, while heating, physical exercises and use of stockings/socks intensify the discomfort. This condition can be primary or idiopathic or secondary to haematological disorders and vascular inflammatory and degenerative diseases. It is reported the case of an eighteen-year-old male who presented, at the early age of two, development of the symptoms of erythema ,heat and pain followed by desquamation of hands and feet, in outbreaks, with intervals 4 to 5 years long between the crises. Keywords: Erythema; Erythromelalgia; Hot temperature; Pain Resumo: Eritromelalgia é uma síndrome clínica rara, caracterizada por calor, rubor e dor intermitente nas extremidades, sendo frequente o acometimento bilateral das extremidades inferiores. O resfriamento local provoca alívio dos sintomas, enquanto aquecimento, exercícios físicos e uso de luvas e meias intensificam o desconforto. A desordem pode ser primária ou idiopática, ou secundária a distúrbios hematológicos e doenças vasculares inflamatórias e degenerativas. Relata-se o caso de um jovem de dezoito anos, com início precoce aos dois anos de idade dos sintomas de eritema, calor e dor, seguidos de descamação nas mãos e pés, em surtos, com intervalos longos de 4 a 5 anos entre as crises.

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“…Cutaneous manifestations related to, but not specific to SLE, include cutaneous vasculitis, periungual telangiectasia, urticarial vasculitis, livedo reticularis, atrophie blanche, and bullous lesions [10]. RP is also seen in 15% to 30% of patients [11] and EM is rarely observed [12]. EM may be primary or secondary in nature.…”

Section: Discussionmentioning

confidence: 99%

Coexistence of Erythromelalgia and Raynaud's Phenomenon in a Systemic Lupus Erythematosus Patient

et al. 2018

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Erythromelalgia (EM) is an uncommon disorder characterized by redness, heat, and painful extremities with intense burning sensation. Attacks of EM may be worsened by limb warming, exercise, or dependency of the affected extremity. Although the coexistence of EM and Raynaud's phenomenon (RP) may appear to be opposites in symptomatology and clinical presentation, recent studies provide an explanation based on a dysfunction of the regulation of vasomotor tone. Here, we report a case of EM in a patient with RP. (J Rheum Dis 2018;25:69-72)

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Secondary erythromelalgia involving the ears probably preceding lupus erythematosus

Cited by 14 publications

References 10 publications

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature

Eritromelalgia primária: relato de caso

Coexistence of Erythromelalgia and Raynaud's Phenomenon in a Systemic Lupus Erythematosus Patient

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