Shulman disease (eosinophilic fasciitis) in X-linked agammaglobulinemia

Pituch‐Noworolska, Anna; Mach-Tomalska, H; Szaflarska, Anna; Adamek, Dariusz

doi:10.5114/pjp.2016.61456

Cited by 13 publications

(8 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The disease may be triggered by several factors. Intensive exercise was documented in three cases [ 10 , 12 , 15 ], infectious agents ( Borrelia burgdorferi, Mycoplasma pneumoniae, Parvovirus B19) in four cases [ 7 – 9 , 28 ], followed by other autoimmune diseases in one case [ 15 ] and hematologic disorders in one case [ 23 ]. There was one patient with medical history of emotional tension [ 18 ], one case with confirmed exposure to a chemical and biological agents [ 21 ] and in one case the symptoms were preceded with surgery [ 29 ] (Table 2 ).…”

Section: Discussionmentioning

confidence: 99%

Diffuse fasciitis with eosinophilia in boys: a case-based review

et al. 2023

View full text Add to dashboard Cite

Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified. In inconclusions cases Magnetic Resonance Imaging (MRI) and skin to muscle biopsy may be useful. The pathogenesis and ethiology remain unknown, but extensive physical exertion, certain infectious factors, such as Borrelia burgdorferi, or medications may serve as a trigger. EF affects equally women and men, mainly in their middle age, however the disease can occur at any age. The standard therapy contents gluccocorticosteroids. As a second-line treatment, methotrexate is usually chosen. In this article we compare world reports of EF in paediatric patients with the cases of two adolescent male patients recently hospitalized in the Department of Paediatric Rheumatology.

show abstract

Section: Discussionmentioning

confidence: 99%

Diffuse fasciitis with eosinophilia in boys: a case-based review

et al. 2023

View full text Add to dashboard Cite

show abstract

“…Cutaneous manifestations in XLA mainly present with severe skin infections like chronic skin ulcers, refractory cellulitis, skin abscesses, skin edema and discoloration and folliculitis [ 142 , 143 ]. Wheal and erythema, typical atopic dermatitis, and skin cancer were also reported in XLA patients [ 144 , 145 , 146 ].…”

Section: Immunodeficiency Related Diseasesmentioning

confidence: 99%

Atopic Dermatitis-like Genodermatosis: Disease Diagnosis and Management

Pan

Zhao

2022

Diagnostics

View full text Add to dashboard Cite

Eczema is a classical characteristic not only in atopic dermatitis but also in various genodermatosis. Patients suffering from primary immunodeficiency diseases such as hyper-immunoglobulin E syndromes, Wiskott-Aldrich syndrome, immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome, STAT5B deficiency, Omenn syndrome, atypical complete DiGeorge syndrome; metabolic disorders such as acrodermatitis enteropathy, multiple carboxylase deficiency, prolidase deficiency; and other rare syndromes like severe dermatitis, multiple allergies and metabolic wasting syndrome, Netherton syndrome, and peeling skin syndrome frequently perform with eczema-like lesions. These genodermatosis may be misguided in the context of eczematous phenotype. Misdiagnosis of severe disorders unavoidably affects appropriate treatment and leads to irreversible outcomes for patients, which underlines the importance of molecular diagnosis and genetic analysis. Here we conclude clinical manifestations, molecular mechanism, diagnosis and management of several eczema-related genodermatosis and provide accessible advice to physicians.

show abstract

“…6. Shulman disease (eosinophilic fasciitis) in X-linked agammaglobulinemia (Pituch-Noworolska et al 2016) 1. The patient was diagnosed with XLA in the first year of life, followed by regular substitution of immunoglobulins.…”

Section: Decrease In Systemic Infections Suchmentioning

confidence: 99%

X-Linked Agammaglobulinemia

Chen¹

2016

Atlas of Genetic Diagnosis and Counseling

View full text Add to dashboard Cite

Shulman disease (eosinophilic fasciitis) in X-linked agammaglobulinemia

Cited by 13 publications

References 9 publications

Diffuse fasciitis with eosinophilia in boys: a case-based review

Diffuse fasciitis with eosinophilia in boys: a case-based review

Atopic Dermatitis-like Genodermatosis: Disease Diagnosis and Management

X-Linked Agammaglobulinemia

Contact Info

Product

Resources

About