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) 4 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 BS, MD Vice-Chair, 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
