Juvenile Dermatomyositis Presenting With Anasarca

Saygı, Semra; Alehan, Füsun; Baskın, Esra; Bayrakçı, Umut Selda; Ulu, Esra Meltem Kayahan; Özbek, Namık

doi:10.1177/0883073808318544

Cited by 22 publications

(8 citation statements)

References 15 publications

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“…Onset can precede, accompany, or follow the diagnosis of DM. The mechanism for this more intense form of edema that exceeds that seen in typical DM is unclear; a hypothesis is that it represents excessive vascular permeability and subsequent leakage as a consequence of immune complex deposition (25,31,39). This more severe form of subcutaneous edema is not clearly correlated with autoantibody status, although subcutaneous edema defined as pitting or non-pitting extremity edema accompanying the active phase of the disease has been found to occur more often in anti-NXP-2 positive than in anti-NXP-2 negative patients (36% vs. 19%, P=0.01) (40).…”

Section: Diffuse Subcutaneous Edemamentioning

confidence: 99%

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Castillo¹,

Femia²

2021

Ann Transl Med

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Dermatomyositis (DM) is a strikingly heterogenous disease characterized by a broad and everevolving spectrum of cutaneous manifestations that transcend the classic "hallmarks" defined by Peter and Bohan in 1975. Despite the increasing preponderance and ubiquity of autoantibody, radiologic, and electrophysiologic testing, the diagnosis of DM still hinges largely on prompt detection of cutaneous manifestations of this condition. While pathognomonic cutaneous features of DM are more readily recognizable, many patients present with subtle and/or atypical skin manifestations, and diagnosis of DM may require clinician identification of these cutaneous clues. In this review, we highlight several of the lesserknown skin manifestations of DM, specifically, panniculitis, diffuse subcutaneous edema, erythroderma, calcinosis, ulceration, flagellate erythema, Wong-type DM, gingival telangiectasias, and the ovoid palatal patch. We describe the clinical and histopathologic presentation of these cutaneous findings. While manifesting less frequently than the heliotrope rash, Gottron's papules, and Gottron's sign, these cutaneous clues are equally important for clinicians to recognize in order to facilitate timely diagnosis and early intervention.

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Section: Diffuse Subcutaneous Edemamentioning

confidence: 99%

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Castillo¹,

Femia²

2021

Ann Transl Med

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“…Although localized edema is common in JDM, generalized edema has been reported rarely in the literature. [8][9][10][11] The generalized edema of JDM can be initially mistaken to be nephrotic syndrome, but a lack of proteinuria, normal albumin levels, and other skin manifestations are usually present.…”

Section: Discussionmentioning

confidence: 99%

A 5-Year-Old Girl with Fatigue, Diffuse Edema, and Weakness

Barsell¹,

Librizzi²

2015

Pediatr Ann

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A 5-year-old previously healthy girl presented to our hospital with 10 days of periorbital edema, abdominal pain, muscle weakness, and progressive fatigue. Four days prior to admission, her pediatrician prescribed diphenhydramine and fexofenadine for a possible allergic reaction, and polyethylene glycol 3,350 for constipation, but the patient showed no signs of improvement. She became progressively more fatigued, and 1 day prior to admission mild extremity edema was noted. The patient denied fever, vomiting, diarrhea, cough, joint pain or stiffness, recent rash, or trauma. She had visited Mexico 1 month prior. There was no significant family history. Outpatient testing revealed a negative urinalysis.In the emergency department, the patient was noted to be tachycardic (heart rate of 132 beats per minute) but otherwise well-appearing. Physical examination was significant for mild hepatomegaly, mild non-pitting edema of the bilateral upper and lower extremities, mild periorbital edema, and bilateral erythematous patches (2 cm × 2 cm) on her knees (Figure 1). Initial emergency department testing was significant for hypoalbuminemia (2.9 g/dL), transaminitis (aspartate aminotransferase [AST] of 231 units/L, alanine aminotransferase [ALT] of 139 units/L), and a mildly elevated erythrocyte sedimentation rate (24 mm/h).Initial differential diagnosis included autoimmune hepatitis, viral hepatitis, protein-losing enteropathy, and viral myocarditis. Coagulation studies and glucose challenge test were normal (suggesting normal synthetic liver function), hepatitis panel was negative, and the AST to ALT ratio of 231:139 made a primary hepatic etiology less likely. Sustained tachycardia prompted further cardiology testing; however, electrocardiogram and echocardiogram revealed normal cardiac function.Generalized fatigue and subjective muscle weakness remained a prominent symptom, and on further questioning the family then disclosed a history of intermittent facial rash and erythema above her eyelids (Figure 2). Therefore, a systemic inflammatory etiology was explored.

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“…To the best of our knowledge, there is only <30 cases in the literature till date, but none were associated with the clinical phenotype of SCLS such a subphenotype clinical variant of JDM is said to have a florid disease course, poorer prognosis with significant morbidity and mortality, and is postulated to represent a more aggressive disease course with underlying immune complex-mediated vascular endothelial damage as the contributor of significant generalized edema (anasarca). [ 6 7 8 9 ] To the best of our knowledge, this is the first published report of an 8-year-old boy with acute JDM, generalized edema, and complicated by fulminant, life-threatening systemic capillary leak syndrome (SCLS; Clarkson Syndrome). There is a report of a similar co-occurrence, being described in a 69-year-old Caucasian woman with acute exacerbation of chronic DM causally linked to C1 inhibitor deficiency.…”

Section: Introductionmentioning

confidence: 99%