raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:83313

Boutonneuse fever

Synonyms: Mediterranean spotted fever

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

A rare spotted fever rickettsiosis caused by infection with the tick-borne bacterium <i>Rickettsia conorii</i>, characterized by the onset of fever after an incubation period of about a week, followed by a centripetally spreading maculopapular rash, which may evolve into a petechial form. Accompanying symptoms are headaches, myalgia and/or arthralgia, among others. The typical ''tache noire'' may be observed at the site of the tick bite for several days. The disease is endemic in Africa, Southern Europe, and India.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enBoutonneuse feverMediterranean spotted fever
frFièvre boutonneuseFièvre boutonneuse méditerranéenne
deBoutonneuse-FieberMittelmeer-Fleckfieber
esFiebre botonosaFiebre manchada mediterránea
itFebbre bottonosa
nlBoutonneuse koortsFièvre boutonneuse · Mediterrane gevlekte koorts
ptFebre de BoutonneuseFebre de Marselha · Febre maculosa mediterrânica · Febre por mordedura da carraça queniana · Tifo da carraça africana · Tifo da carraça indiana · Tifo da carraça queniana
plGorączka plamista BoutonneuseŚródziemnomorska gorączka plamista · Śródziemnomorska gorączka plamista
csMarseillská horečkaHorečka boutonneuse · Exantematózní horečka
ukПлямиста лихоманкаСередземноморська плямиста лихоманка
zh南欧斑疹热地中海斑疹热
trBoutonneuse ateşiAkdeniz benekli ateşi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10A77.1NTBTinclusionvalidated
ICD-111C31.1Especificvalidated
MONDO0024472Evalidated
MeSHD001907Evalidated
UMLSC0006060Evalidated
MedDRA10006045Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

Codes that Orphanet moved or referred to this one:

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

3 records
TypeClassValueAreaQualificationValidationSource
Point prevalence1-5 / 10 00024.2 / 100 000FranceValue and classNot yet validatedORPHANET
Point prevalence1-9 / 100 0009.8 / 100 000PortugalValue and classNot yet validatedORPHANET
Point prevalence1-5 / 10 000—EuropeClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

26 annotations · validated 2020-04-22
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0100872Abnormality of the plantar skin of foot
HP:0040211Abnormality of the skin of the palm
HP:0001945Fever
HP:0000988Skin rash
Frequent (79-30%)
HP:0002829Arthralgia
HP:0025289Cervical lymphadenopathy
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0002315Headache
HP:0003496Increased circulating IgM level
HP:0002716Lymphadenopathy
HP:0012733Macule
HP:0040186Maculopapular exanthema
HP:0003326Myalgia
HP:0000083Renal insufficiency
HP:0032156Skin detachment
HP:0200036Skin nodule
HP:0001873Thrombocytopenia
Occasional (29-5%)
HP:0002027Abdominal pain
HP:0002014Diarrhea
HP:0003237Increased circulating IgG level
HP:0001882Leukopenia
HP:0002018Nausea
HP:0000967Petechiae
Very rare (<4-1%)
HP:0000613Photophobia
HP:0002878Respiratory failure
HP:0002633Vasculitis

Run the differential on the 4 obligate / very frequent terms

Annotation source: 27380385[PMID]_29855412[PMID]

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Sources for this card