Necrotic facial papules in an adolescent: C2 deficiency with eventual development of lupus erythematosus. Pediatr Dermatol 2003;20(4):318-22
Date
07/19/2003Pubmed ID
12869153DOI
10.1046/j.1525-1470.2003.20409.xScopus ID
2-s2.0-0042206515 (requires institutional sign-in at Scopus site) 2 CitationsAbstract
A 14-year-old girl was admitted to the hospital because of persistent throat pain, fever, fatigue, 25 pound weight loss, and leukopenia. On physical examination she was thin, ill-appearing, and had necrotic papules on the face and palpable cervical lymph nodes. Presumptive differential diagnosis included occult malignancy and infection. Numerous investigative procedures failed to elucidate a source. Vasculitis was eventually appreciated after repeat skin biopsy. Numerous serologic studies were performed and were notable for a very low level of the second component of complement without direct evidence of lupus erythematosus (LE) or other autoimmune conditions. A diagnosis of C2 deficiency-associated vasculitis was made. She was treated with high-dose prednisone and cyclophosphamide with resolution of her symptoms. Two years later she returned with marked malar erythema. Antinuclear and Smith antibodies were then detected and a diagnosis of LE was made. She was treated with hydroxychloroquine and sun-avoidance measures with clearance of the malar rash.
Author List
Lyon VB, Nocton JJ, Drolet BA, Esterly NBAuthor
James J. Nocton MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentComplement C2
Facial Dermatoses
Female
Humans
Lupus Erythematosus, Cutaneous
Necrosis
Vasculitis
