Subcutaneous oedema of upper limbs heralding an aggressive form of dermatomyositis

Shukla, Mohit; Patel, Rishi; Kuzyshyn, Halyna; Feinstein, Daniel

doi:10.1136/bcr-2017-221908

Cited by 5 publications

(7 citation statements)

References 7 publications

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“…Although a few cases of edema in DM have been sporadically reported, our study is different in combining the rash ( pseudoangioedema) with myositis-specific autoantibody results to better guide the diagnosis, prognosis judgment, and differential treatment of DM. 5 In conclusion, significant pseudoangioedema may be associated with a poor prognosis and necessitates the evaluation of underlying comorbidities and aggressive management.…”

Section: Pseudoangioedema In Dermatomyositis Patients Indicates Sever...mentioning

confidence: 99%

Pseudoangioedema in dermatomyositis patients indicates severe disease and poor prognosis

Huang

Wang

et al. 2022

Journal of the American Academy of Dermatology

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Section: Pseudoangioedema In Dermatomyositis Patients Indicates Sever...mentioning

confidence: 99%

Pseudoangioedema in dermatomyositis patients indicates severe disease and poor prognosis

Huang

Wang

et al. 2022

Journal of the American Academy of Dermatology

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“…While periorbital and focal/localized subcutaneous edema especially in poikilodermatous sites is a common feature of DM, more widespread edema, sometimes to the point of anasarca, is considerably rare (24)(25)(26)(27)(28)(29). To date, only 20 or so cases involving juvenile and adult patients with DM presenting with diffuse limb, trunk, and/or generalized subcutaneous edema have been reported in the literature since it was first described by Nitsche in 1988 (24,(30)(31)(32)(33)(34)(35)(36)(37)(38). Most cases are idiopathic but an associated malignancy has been reported in a few (28,32,36,38).…”

Section: Diffuse Subcutaneous Edemamentioning

confidence: 99%

“…Fat-suppressed T2-weighted and/or short τ inversion-recovery (STIR) magnetic resonance imaging (MRI) has been found to be useful in identifying the extent of the subcutaneous and muscle edema and in monitoring treatment response (24)(25)(26)35). Presence of subcutaneous edema may indicate a more severe form of DM that requires more aggressive management and may carry a worse overall prognosis (24,26,31). In many cases, pulse dose steroids and/or IVIG was required to gain control of the disease (24)(25)(26)(27)(28)32).…”

Section: Diffuse Subcutaneous Edemamentioning

confidence: 99%

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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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“…Here the authors report a 69-year-old lady who presented with generalized edema and dysphagia, who after thorough evaluation was diagnosed to be a case of edematous dermatomyositis. It is an atypical presentation of the disease with less than 30 adult cases reported so far [ 5 ]. This form of dermatomyositis tends to be more aggressive and is associated with a higher risk of malignancy [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Dermatomyositis Masquerading As Generalized Body Swelling: A Case Report

Sunny,

Kashyap,

Kumar

et al. 2023

Cureus

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Dermatomyositis (DM) is a systemic autoimmune disease that primarily affects the skin and muscles. The hallmark skin manifestation is a violaceous rash on the face, neck, shoulders, upper chest, and extensor surfaces of the arms and legs, which is often accompanied by edema and can be exacerbated by exposure to sunlight. Generalized limb edema and dysphagia are rare presentations of dermatomyositis. Here we present a case of a 69-year-old woman presenting with generalized limb swelling, periorbital swelling, and dysphagia which was diagnosed as dermatomyositis based on a combination of clinical, laboratory, and imaging findings. The patient had an absence of complaints of limb weakness and a predominance of complaints of edema and dysphagia which posed a diagnostic challenge. The patient was treated with high-dose steroids and immunosuppressive therapy, leading to a significant improvement in her symptoms. Edematous dermatomyositis has been associated with underlying malignancy in 25% of the cases and this warrants close follow-up and malignancy screening for such patients. In some cases, subcutaneous edema might be the only manifestation of the disease. This case underscores the importance of recognizing DM as a potential differential diagnosis in patients presenting with generalized edema and dysphagia, particularly in the initial absence of classic skin findings. This rare presentation of dermatomyositis may be a hallmark of a severe form of the disease and requires prompt recognition and aggressive treatment.

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Subcutaneous oedema of upper limbs heralding an aggressive form of dermatomyositis

Cited by 5 publications

References 7 publications

Pseudoangioedema in dermatomyositis patients indicates severe disease and poor prognosis

Pseudoangioedema in dermatomyositis patients indicates severe disease and poor prognosis

Covert clues: the non-hallmark cutaneous manifestations of dermatomyositis

Dermatomyositis Masquerading As Generalized Body Swelling: A Case Report

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