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Juvenile Dermatomyositis anti-NXP2 with Capillaroscopic Improvement

Name: Juvenile Dermatomyositis anti-NXP2 with Capillaroscopic Improvement
Creator: Clara Udaondo
Published: 2026-07-03

Juvenile Dermatomyositis (anti-NXP2+)Early Scleroderma-Like Pattern with Treatment ResponseModerate to Severe

Images & measurements46

A 12-year-old girl with muscle weakness, markedly elevated CPK, and no skin disease, followed with two capillaroscopies during treatment response.

AuthorClara Udaondo

Added to Capillaroscopy.comJuly 3, 2026

Clinical context

The patient presented with several weeks of shoulder and pelvic girdle weakness, without skin involvement or other symptoms. Laboratory testing showed CPK elevation up to 8000 U/L, lymphopenia, thrombocytopenia, and low-titer positive ANA. Whole-body MRI showed muscle edema in the girdles. The initial myositis panel was negative, so the early scleroderma-like capillaroscopy pattern helped support the diagnosis of juvenile dermatomyositis despite the absence of skin disease or specific autoantibodies. An extended panel later detected anti-NXP2 antibodies. Treatment was started with hydroxychloroquine, mycophenolate mofetil, and intravenous immunoglobulin, followed by clinical improvement and CK reduction.

Capillaroscopy

The June 2024 capillaroscopy showed an early scleroderma-like pattern compatible with juvenile dermatomyositis, including reduced density, frequent enlarged capillaries, sparse giant capillaries, and hemorrhages. The December 2024 follow-up showed disappearance of giant capillaries and evolution toward a non-specific pattern, with improved capillary density.

Key Capillaroscopic Findings

Initial reduced density (5.83/mm) with frequent enlarged capillaries (68.8%)
Initial sparse giant capillaries (2.6%) and hemorrhages
Follow-up density improved to 6.72/mm, with disappearance of giant capillaries

Atlas images from this case

Juvenile anti-NXP2 dermatomyositis: initial overview

Juvenile anti-NXP2 dermatomyositis: follow-up overview

Longitudinal Capillaroscopic Comparison

June 2024 → December 2024

Capillary density (N/mm)5.83 (Reduced)→6.72 (Improved)

Giant capillaries2.6%→0%

HemorrhagesPresent (0.17/mm)→Present, mild (0.20/mm)

Enlarged capillaries (%)68.8%→38.1%

2 capillaroscopies

The study, field by field

Images & measurements

Explore the fields from each finger and open any image for a closer look. Measurements describe the complete examination at each visit.

Contributed by Clara Udaondo for educational publication on Capillaroscopy.com. For reuse permissions, contact the rights holder.

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Initial study

Early scleroderma-like pattern supporting the diagnosis

Recorded measurements

Capillary density: 5.83capillaries/mm
Enlarged capillaries: 68.77%
Giant capillaries: 2.60%
Hemorrhages: 0.17/mm

Values from the saved analysis: density and hemorrhages per total analyzed length; percentages of the total capillary count. These are not measurements of an individual image and may differ from averages across fields.

Left index finger

4 images

Overview
Initial nailfold overview in the juvenile anti-NXP2 longitudinal case. Compare the follow-up and the documented quantitative findings in the complete case.
Read the atlas commentary

Left middle finger

3 images

Left ring finger

3 images

Left little finger

2 images

Right index finger

4 images

Right middle finger

3 images

Right ring finger

5 images

Right little finger

3 images

Follow-up study

6 months after the initial study

Non-specific pattern after clinical improvement

Recorded measurements

Capillary density: 6.72capillaries/mm
Enlarged capillaries: 38.12%
Giant capillaries: 0.00%
Hemorrhages: 0.20/mm

Left index finger

4 images

Overview
Follow-up nailfold overview from the same juvenile anti-NXP2 case. Differences in overview appearance should be interpreted alongside acquisition conditions and the serial reports.
Read the atlas commentary

Left middle finger

2 images

Left ring finger

2 images

Left little finger

2 images

Right index finger

2 images

Right middle finger

2 images

Right ring finger

3 images

Right little finger

2 images

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