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The lymphoid follicle variant of dermatomyositis.

Radke J, Pehl D, Aronica E, Schonenberg-Meinema D, Schneider U, Heppner FL, de Visser M, Goebel HH, Stenzel W - Neurol Neuroimmunol Neuroinflamm (2014)

Bottom Line: Electron microscopy was used to confirm the light microscopic diagnosis of DM.Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy.The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis.

View Article: PubMed Central - PubMed

Affiliation: Departments of Neuropathology (J.R., D.P., F.L.H., H.H.G., W.S.) and Rheumatology and Clinical Immunology (U.S.), Charité Universitätsmedizin Berlin, Germany; and Departments of (Neuro) Pathology (E.A.), Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children's Hospital (D.S.-M.), and Neurology and Neurophysiology (M.d.V.), Academic Medical Centre, University of Amsterdam, the Netherlands.

ABSTRACT

Objective: To investigate the clinical and morphologic spectrum of early adult-onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin.

Methods: Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ectopic lymphoid structures in muscle biopsies obtained from 2 patients diagnosed with DM. Clinical presentation and morphologic features, as well as treatment and follow-up, were assessed and documented. Electron microscopy was used to confirm the light microscopic diagnosis of DM. Clonality analysis of B-cell populations using PCR was performed.

Results: Muscle biopsy of both patients fulfilled the morphologic European Neuromuscular Centre criteria of DM. Analyses of muscle biopsy samples revealed ectopic lymphoid follicle-like structures that showed a remarkable similarity to secondary lymphoid organs (SLOs) with distinct T- and B-cell compartmentalization. Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy.

Conclusions: The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis.

No MeSH data available.


Related in: MedlinePlus

Contrast-enhanced MRI of affected muscles in both patientsContrast-enhanced MRI (T1) revealed areas of hyperintensity with diffuse gadolinium uptake in the affected muscle. Patient 1: left biceps (C); patient 2: right deltoid and left triceps brachii muscles (A, B).
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Figure 1: Contrast-enhanced MRI of affected muscles in both patientsContrast-enhanced MRI (T1) revealed areas of hyperintensity with diffuse gadolinium uptake in the affected muscle. Patient 1: left biceps (C); patient 2: right deltoid and left triceps brachii muscles (A, B).

Mentions: Patient 1, a 27-year-old woman, had a 7-year history of unilateral swelling of her left biceps muscle and hand as well as wrist pain and morning stiffness of her finger joints. A contrast-enhanced MRI of her left biceps showed diffuse gadolinium uptake, thickening of the muscle fascia, and muscle edema (figure 1). On neurologic examination she showed normal symmetrical muscle strength and normal fine motor skills. Thorough examination of the skin did not reveal any abnormalities. Laboratory examination of her blood serum showed elevated immunglobulin E, C-reactive protein, and antinuclear and anti-cyclic citrullinated peptide antibodies. Serum creatine kinase (CK) was normal. There was no evidence of rheumatoid arthritis. Based on the international consensus criteria,1 biopsy of the left biceps gave a diagnosis of DM with accompanying B-cell-rich follicle-like structures. An initial high-dose (1 mg/kg/d) prednisolone treatment over 4 weeks followed by a low-dose treatment (5 mg/d) led to rapid improvement and persistent resolution of her symptoms over a 1-year period.


The lymphoid follicle variant of dermatomyositis.

Radke J, Pehl D, Aronica E, Schonenberg-Meinema D, Schneider U, Heppner FL, de Visser M, Goebel HH, Stenzel W - Neurol Neuroimmunol Neuroinflamm (2014)

Contrast-enhanced MRI of affected muscles in both patientsContrast-enhanced MRI (T1) revealed areas of hyperintensity with diffuse gadolinium uptake in the affected muscle. Patient 1: left biceps (C); patient 2: right deltoid and left triceps brachii muscles (A, B).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4202675&req=5

Figure 1: Contrast-enhanced MRI of affected muscles in both patientsContrast-enhanced MRI (T1) revealed areas of hyperintensity with diffuse gadolinium uptake in the affected muscle. Patient 1: left biceps (C); patient 2: right deltoid and left triceps brachii muscles (A, B).
Mentions: Patient 1, a 27-year-old woman, had a 7-year history of unilateral swelling of her left biceps muscle and hand as well as wrist pain and morning stiffness of her finger joints. A contrast-enhanced MRI of her left biceps showed diffuse gadolinium uptake, thickening of the muscle fascia, and muscle edema (figure 1). On neurologic examination she showed normal symmetrical muscle strength and normal fine motor skills. Thorough examination of the skin did not reveal any abnormalities. Laboratory examination of her blood serum showed elevated immunglobulin E, C-reactive protein, and antinuclear and anti-cyclic citrullinated peptide antibodies. Serum creatine kinase (CK) was normal. There was no evidence of rheumatoid arthritis. Based on the international consensus criteria,1 biopsy of the left biceps gave a diagnosis of DM with accompanying B-cell-rich follicle-like structures. An initial high-dose (1 mg/kg/d) prednisolone treatment over 4 weeks followed by a low-dose treatment (5 mg/d) led to rapid improvement and persistent resolution of her symptoms over a 1-year period.

Bottom Line: Electron microscopy was used to confirm the light microscopic diagnosis of DM.Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy.The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis.

View Article: PubMed Central - PubMed

Affiliation: Departments of Neuropathology (J.R., D.P., F.L.H., H.H.G., W.S.) and Rheumatology and Clinical Immunology (U.S.), Charité Universitätsmedizin Berlin, Germany; and Departments of (Neuro) Pathology (E.A.), Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children's Hospital (D.S.-M.), and Neurology and Neurophysiology (M.d.V.), Academic Medical Centre, University of Amsterdam, the Netherlands.

ABSTRACT

Objective: To investigate the clinical and morphologic spectrum of early adult-onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin.

Methods: Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ectopic lymphoid structures in muscle biopsies obtained from 2 patients diagnosed with DM. Clinical presentation and morphologic features, as well as treatment and follow-up, were assessed and documented. Electron microscopy was used to confirm the light microscopic diagnosis of DM. Clonality analysis of B-cell populations using PCR was performed.

Results: Muscle biopsy of both patients fulfilled the morphologic European Neuromuscular Centre criteria of DM. Analyses of muscle biopsy samples revealed ectopic lymphoid follicle-like structures that showed a remarkable similarity to secondary lymphoid organs (SLOs) with distinct T- and B-cell compartmentalization. Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy.

Conclusions: The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis.

No MeSH data available.


Related in: MedlinePlus