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Open Access Original research

Allergic manifestations and cutaneous histamine responses in patients with McCune Albright syndrome

Jill D Jacobson*, Angela L Turpin and Scott A Sands

Author Affiliations

Section of Endocrinology, Children’s Mercy Hospital, University of Missouri-Kansas City, School of Medicine, 2401 Gillham Road, Kansas, MO 64108, USA

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World Allergy Organization Journal 2013, 6:9  doi:10.1186/1939-4551-6-9

Published: 1 May 2013

Abstract

Background

McCune Albright syndrome (MAS) is a rare disorder characterized by precocious puberty, café-au-lait spots, and fibrous dysplasia. Its cause is an activating mutation in the GNAS gene, encoding a subunit of the stimulatory G protein, Gsalpha (Gsα). The action of any mediator that signals via Gsα and cyclic AMP can be up regulated in MAS. We had observed gastritis, gastroesophageal reflux, and anaphylaxis in McCune Albright patients.

Objective

As histamine is known to signal via histamine 1 (H1) and histamine 2 (H2) receptors, which couple with stimulatory G proteins, we attempted to mechanistically link histamine responsiveness to the activating GNAS mutation. We hypothesized that responsiveness to histamine skin testing would differ between MAS patients and healthy controls.

Patients and methods

After obtaining informed consent, we performed a systematic review of histamine responsiveness and allergic manifestations in 11 MAS patients and 11 sex-matched, Tanner-stage matched controls. We performed skin prick testing, quantifying the orthogonal diameters of wheals and erythema. We also quantitated G protein mRNA expression.

Results

The peak wheal and flare responses to histamine were significantly higher in MAS patients compared to controls.

Conclusions

This study suggests that MAS patients may be at risk for exaggerated histamine responsiveness compared to unaffected controls.

Keywords:
McCune Albright syndrome; Histamine responsiveness; Wheal and flare; Atopy; Anaphylaxis