Inverse Gottron papules in juvenile dermatomyositis: an under recognized clinical entity

Area of Science:

• Pediatric Rheumatology
• Dermatology
• Pulmonology

Background:

• Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy affecting children.
• Cutaneous manifestations are common in JDM, but inverse Gottron sign is rarely reported.
• Gottron papules typically appear on dorsal aspects of joints, not palmar surfaces.

Purpose of the Study:

• To report four cases of JDM with inverse Gottron sign.
• To review literature for similar cases of JDM with palmar Gottron papules.
• To investigate potential associations and clinical implications of this rare sign.

Main Methods:

• Retrospective case record review of children diagnosed with JDM.
• Identification of patients with Gottron papules on the palmar surface.
• Literature search for published cases of JDM with inverse Gottron sign.
• Clinical data collection including age, symptoms, comorbidities, investigations, and treatment.

Main Results:

• Four out of 127 JDM patients (3.1%) presented with inverse Gottron sign.
• Median age at presentation was 9 years (range 8-10).
• Inverse Gottron papules were located on palmar aspects of interphalangeal joints, creases, and lateral aspects.
• Three of the four patients showed interstitial lung disease (ILD) on HRCT chest.
• One patient had systemic scleroderma overlap, and another had patchy lipoatrophy.
• Two patients tested positive for ANA.

Conclusions:

• Inverse Gottron papule is an uncommon cutaneous manifestation of JDM.
• Palmar Gottron papules in JDM may be associated with interstitial lung disease.
• Screening for ILD in JDM patients with palmar Gottron papules is recommended.
• Further research is needed to clarify the clinical significance of this sign in JDM.