Abstract

Arthrogryposis type six is one of the distal arthrogryposes. It associates arthrogryposis of the hands and sensorineural deafness. Male-to-male transmission was observed. No gene has been identified to date. We report a unique case of a girl presenting with congenital arthrogryposis-like hand anomaly, sensorineural deafness, and acute onset of systemic lupus erythematosus (SLE).

Highlights

  • Arthrogryposis-like hand and deafness syndrome or distal arthrogryposis type 6 (DA6) is characterized by an arthrogryposis-like hand anomaly and a sensorineural deafness

  • We report a unique case of a girl presenting with congenital arthrogryposis-like hand anomaly, sensorineural deafness, and acute onset of systemic lupus erythematosus (SLE)

  • We describe a case of DA6 in a six-year-old girl who had an acute onset of systemic lupus erythematosus (SLE)

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Summary

Introduction

Arthrogryposis-like hand and deafness syndrome or distal arthrogryposis type 6 (DA6) is characterized by an arthrogryposis-like hand anomaly and a sensorineural deafness. We describe a case of DA6 in a six-year-old girl who had an acute onset of systemic lupus erythematosus (SLE). This case is -to our knowledge- the first to be described to date. An acute onset of a cutaneous rash alarmed the parents At examination, she had on top of bilateral hands arthrogryposis, a generalized discoid rash sparing the palms, and the soles of the feet. The child was still asymptomatic; Citation: Nassih H, El Qadiry R, Bourrahouat A, Sab IA (2020) Distal Arthrogryposis Type Six and Systemic Lupus Erythematosus, in a Girl: First Pediatric Case.

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