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

The objective of this study was to report four children having juvenile dermatomyositis (JDM) with inverse Gottron sign along with a brief review of literature of similarly published cases. This is a retrospective review of case records of all children diagnosed with JDM at a single tertiary care centre in North India. Children who were found to have Gottron papules over palmar surface were identified. A total of 127 children were diagnosed with JDM in the last 24 years (1994-2017). Out of these 127 patients, 4 were found to have Gottron papules over palmar surface. The median age at presentation of these four patients was 9 years (range 8-10). The distribution for inverse Gottron papules was over the palmar aspect of proximal and distal interphalangeal joints, palmar creases and lateral aspects of proximal and distal interphalangeal joints. One child also had features of systemic scleroderma overlap and one patient had patchy lipoatrophy. ANA were positive in two patients. High-resolution computed tomography (HRCT) chest was carried out in all four patients and three of them were found to have changes consistent with interstitial lung disease. Prednisolone was used for all patients, methotrexate in three, azathioprine in two and cyclophosphamide in one patient. Inverse Gottron papule is a hitherto uncommonly recognized cutaneous manifestation of JDM. The clinical implications of this sign are yet not clear in children with JDM. Children with JDM with palmar Gottron papules may be screened for ILD as this may change their management and prognosis.