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Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity.

Tuttle KS, Vargas SO, Callahan MJ, Bae DS, Nigrovic PA - Pediatr Rheumatol Online J (2015)

Bottom Line: Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate.These findings help to define enthesitis as a clinicopathologic entity.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology, Boston Children's Hospital, Boston, MA USA.

ABSTRACT

Context: Imaging of dactylitis in adult psoriatic arthritis suggests a pathophysiological role for enthesitis. However, histological definition of the dactylitic digit is unavailable.

Objective: We evaluated the role of enthesitis in dactylitis associated with psoriatic juvenile idiopathic arthritis (psJIA) in a child who underwent detailed imaging and histologic evaluation.

Design: Radiographs, ultrasound and high-resolution magnetic resonance imaging were employed to define the locus of disease in two dactylitic digits from a 14-year-old girl with psJIA. Biopsies were stained with hematoxylin and eosin, CD3, CD20, CD4, CD8 and CD117 and examined via light microscopy.

Results: Radiologic features of dactylitis included enhanced signal at digital entheses without accompanying synovitis or tenosynovitis. Histologically, finger and toe tissue exhibited hypervascular tenosynovium with a fibromyxoid expansion of fibrous tissue. This was accompanied by sparse to moderate perivascular lymphocytic inflammation consisting predominantly of T cells, with occasional admixed B cells and mast cells. Neutrophils and plasma cells were absent. Fibrocartilage exhibited reactive features including increased extracellular myxoid matrix, binucleation, and focal necrosis, without cellular inflammation. Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.

Conclusions: Dactylitis in psJIA bears a radiographic resemblance to the condition in adult psoriatic arthritis. Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate. These findings help to define enthesitis as a clinicopathologic entity.

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Related in: MedlinePlus

Photographs depicting the clinical phenotype. A. Dactylitis of the right fourth finger. B. Dactylitis of left fifth toe. C. Representative skin findings, here an annular plaque from right forearm characterized by erythema and overlying silvery scale, consistent with psoriasis. D. Scalp biopsy showing psoriasiform dermatitis, characterized by hyperkeratosis, parakeratosis, and neutrophils within the stratum corneum and spinous layer (hematoxylin and eosin, original magnification, 400×).
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Fig1: Photographs depicting the clinical phenotype. A. Dactylitis of the right fourth finger. B. Dactylitis of left fifth toe. C. Representative skin findings, here an annular plaque from right forearm characterized by erythema and overlying silvery scale, consistent with psoriasis. D. Scalp biopsy showing psoriasiform dermatitis, characterized by hyperkeratosis, parakeratosis, and neutrophils within the stratum corneum and spinous layer (hematoxylin and eosin, original magnification, 400×).

Mentions: The patient was a female who was diagnosed with JDMS at age 3 years. The patient had been treated with corticosteroids, hydroxychloroquine, and methotrexate, but had persistent disease activity and developed multiple calcinoses and joint contractures, although without dactylitis or arthritis. At age 9 years, the patient moved from Central America to the United States to obtain further medical care. Her JDMS proved highly resistant to therapy, with new calcifications appearing even after therapy with pulsed corticosteroids, high-dose oral prednisone, methotrexate, cyclosporine, mycophenolate, and rituximab. Additional medications had included probenecid, diltiazem, colchicine, and atovaquone. At age 14, shortly after completing a course of cyclophosphamide and a trip to Central America, she presented with atraumatic fusiform swelling and tenderness of the right fourth finger and left fifth toe (Figure 1A and B). Skin examination revealed white, scaling, erythematous plaques on the knuckles (Figure 1A), frontal and occipital scalp, and bilateral dorsal forearms (Figure 1C). A scalp biopsy showed psoriasiform dermatitis (Figure 1D), supporting the clinical diagnosis of psoriasis.Figure 1


Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity.

Tuttle KS, Vargas SO, Callahan MJ, Bae DS, Nigrovic PA - Pediatr Rheumatol Online J (2015)

Photographs depicting the clinical phenotype. A. Dactylitis of the right fourth finger. B. Dactylitis of left fifth toe. C. Representative skin findings, here an annular plaque from right forearm characterized by erythema and overlying silvery scale, consistent with psoriasis. D. Scalp biopsy showing psoriasiform dermatitis, characterized by hyperkeratosis, parakeratosis, and neutrophils within the stratum corneum and spinous layer (hematoxylin and eosin, original magnification, 400×).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4359473&req=5

Fig1: Photographs depicting the clinical phenotype. A. Dactylitis of the right fourth finger. B. Dactylitis of left fifth toe. C. Representative skin findings, here an annular plaque from right forearm characterized by erythema and overlying silvery scale, consistent with psoriasis. D. Scalp biopsy showing psoriasiform dermatitis, characterized by hyperkeratosis, parakeratosis, and neutrophils within the stratum corneum and spinous layer (hematoxylin and eosin, original magnification, 400×).
Mentions: The patient was a female who was diagnosed with JDMS at age 3 years. The patient had been treated with corticosteroids, hydroxychloroquine, and methotrexate, but had persistent disease activity and developed multiple calcinoses and joint contractures, although without dactylitis or arthritis. At age 9 years, the patient moved from Central America to the United States to obtain further medical care. Her JDMS proved highly resistant to therapy, with new calcifications appearing even after therapy with pulsed corticosteroids, high-dose oral prednisone, methotrexate, cyclosporine, mycophenolate, and rituximab. Additional medications had included probenecid, diltiazem, colchicine, and atovaquone. At age 14, shortly after completing a course of cyclophosphamide and a trip to Central America, she presented with atraumatic fusiform swelling and tenderness of the right fourth finger and left fifth toe (Figure 1A and B). Skin examination revealed white, scaling, erythematous plaques on the knuckles (Figure 1A), frontal and occipital scalp, and bilateral dorsal forearms (Figure 1C). A scalp biopsy showed psoriasiform dermatitis (Figure 1D), supporting the clinical diagnosis of psoriasis.Figure 1

Bottom Line: Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate.These findings help to define enthesitis as a clinicopathologic entity.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology, Boston Children's Hospital, Boston, MA USA.

ABSTRACT

Context: Imaging of dactylitis in adult psoriatic arthritis suggests a pathophysiological role for enthesitis. However, histological definition of the dactylitic digit is unavailable.

Objective: We evaluated the role of enthesitis in dactylitis associated with psoriatic juvenile idiopathic arthritis (psJIA) in a child who underwent detailed imaging and histologic evaluation.

Design: Radiographs, ultrasound and high-resolution magnetic resonance imaging were employed to define the locus of disease in two dactylitic digits from a 14-year-old girl with psJIA. Biopsies were stained with hematoxylin and eosin, CD3, CD20, CD4, CD8 and CD117 and examined via light microscopy.

Results: Radiologic features of dactylitis included enhanced signal at digital entheses without accompanying synovitis or tenosynovitis. Histologically, finger and toe tissue exhibited hypervascular tenosynovium with a fibromyxoid expansion of fibrous tissue. This was accompanied by sparse to moderate perivascular lymphocytic inflammation consisting predominantly of T cells, with occasional admixed B cells and mast cells. Neutrophils and plasma cells were absent. Fibrocartilage exhibited reactive features including increased extracellular myxoid matrix, binucleation, and focal necrosis, without cellular inflammation. Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.

Conclusions: Dactylitis in psJIA bears a radiographic resemblance to the condition in adult psoriatic arthritis. Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate. These findings help to define enthesitis as a clinicopathologic entity.

Show MeSH
Related in: MedlinePlus