Kikuchi fujimoto syndrome M 06.10

Kikuchi-Fujimoto Syndrome is an extremely rare, self-limited benign disease, also known as "histiocytic necrotizing lymphadenitis", which is dermatologically characterized by an inconsistent variable macular exanthema that can be confused with systemic lupus erythematosus but may also be associated with this disease.

Especially in the Asian population (Japan). In Europe only isolated cases. Age of disease: < 30 years; w:m=4:1

A viral genesis is suspected (HHV-8, Epstein-Barr-Virus, HHV6, Parvovirus B19)

Acute or subacute course with feeling of illness, night sweats, weight loss, subfebrile temperatures (30-50%), bronchitis and laryngitis, cervical lymphadenopathy. The disease is self-limiting and persists for 1-4 months. The histological lymph node changes are diagnostic.

Signs of inflammation, usually not pathological, atypical lymphocytes may be detectable.

Kawasaki syndrome, Still syndrome (of the adult), lupus erythematosus, systemic, lymphoma, cutaneous B-cell lymphoma,

Symptomatic, anti-IL-1 antibodies.

1. Di Lernia V et al (2014) Subacute cutaneous lupus erythematosus onset preceded by Kikuchi-Fujimoto disease. Dermatol Pract Concept 31:47-49

2. Fueyo-Casado A et al (2011) Fever, cervical lymphadenopathy, and erythematous-violaceous facial plaques. Actas Dermosifiliogr 102:629-630

3. Kim SK et al (2011) Histiocytic necrotizing lymphadenitis in the context of systemic lupus erythematosus (SLE): Is histiocytic necrotizing lymphadenitis in SLE associated with skin lesions? Lupus 20:809-819

4. Koga T et al (2013) Sweet's syndrome complicated by Kikuchi's disease and hemophagocytic syndrome which presented with retinoic acid-inducible gene-I in both the skin epidermal basal layer and the cervical lymph nodes. Internal Med 52:1839-1843
5. Sondermann W et al (2014) Coincidence of Kikuchi-Fujimoto syndrome and adult M. Still in a young Caucasian female patient. Cases, facts, drugs. 17th meeting of the DWFA Cologne. 28-30.11.2014
6. Yamamoto T (2011) Cutaneous manifestations associated with adult-onset Still's disease: important diagnostic values. Rheumatol Int 32:2233-2237