Pfapa syndrome E85.0

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 09.07.2022

History
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Marshall, 1987

Definition
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Most frequent, non-heritable or only sporadically inherited, periodic fever syndrome in children in our latitudes PFAPA is an acronym that summarizes the main symptoms of the disease: periodic fever, aphthae with stomatitis, pharyngitis and lymphadenitis.

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Dermatology

Keratosis areolae mammae naevifo...

Typically unilaterally occurring, nevoidal malformations of the nipple court.

Etiopathogenesis
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Unclear, family disposition does not seem to exist.

Manifestation
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Infancy; childhood

Clinical features
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The disease typically begins in early childhood with recurrent fever attacks (often > 39 °C), non-exudative pharyngitis, cervical lymphadenopathy, aphthae (similar in aspect to habitual aphthae). The fever episodes last on average 3-6 days and recur with almost fixed periodicity of 2-6 weeks (mostly about 3-4 weeks). Often the parents describe: "I can set the clock accordingly". Between the fever episodes, there is freedom of appearance.

Laboratory
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During the fever episodes, the inflammatory signs common to all periodic fever syndromes, such as leukocytosis, elevated CRP (50-150 mg/l) and accelerated BSG (30-60 mm/hr), are found in the laboratory. Elevated serum IgG levels have been described in isolated cases.

Diagnosis
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There are no specific parameters for the diagnosis of PFAPA, so it is a pure exclusion diagnosis.

Differential diagnosis
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Differentiation from other forms of periodic fever such as Familial Mediterranean fever; Muckle-Wells syndrome; Tumor necrosis factor receptor-associated periodic syndrome (TRAPS); Hyper IgD syndrome (HID); Familial cold urticaria.

Differentiation from other diseases associated with aphthous changes, especially Behçet 's disease and habitual aphthae.

Therapy
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The therapy is symptomatic and is based on a single administration of cortisone (prednisone 2 mg/kg bw) at the beginning of the febrile episode. With this therapy, the acute attack can be stopped, but the result can also be a shortened fever-free interval. Long-term but slow improvement can be achieved in 30% of cases with cimetidine in therapeutic doses (administered for a period of 6 to 12 months).

If cimetidine remains ineffective, tonsillectomy with simultaneous adenotomy is another therapeutic option (it is recommended by most authors). Studies in this area, however, with only a very small number of cases, show a positive curative effect in about 72% of patients.

Progression/forecast
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Favorable; prolongation of fever-free intervals, then remission.

Literature
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Atas B et al (2003) PFAPA syndrome mimicking familial Mediterranean fever: report of a Turkish child. J Emerg Med 25: 383-385
Berlucchi M et al (2003) Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature. Ann Otol Rhinol Laryngol 112: 365-369
Galanakis E et al (2002) PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 86: 434-435
Parikh SR et al (2003) Utility of tonsillectomy in 2 patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Arch Otolaryngol Head Neck Surgery 129: 670-673
Lee WI et al (1999) PFAPA syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis, adenitis). Clin Rheumatol 18: 207-213
Marshall GS et al (1987) Syndromes of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatrics 110: 43-46