Granulosis Rubra Nasi

Updated: Jun 18, 2018
  • Author: Basil M Hantash, MD, PhD, MBA; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

Granulosis rubra nasi is described as a benign, autosomal dominant familial disease of children involving the eccrine glands of the nose, cheeks, and chin. German dermatologist Josef Jadassohn first described the condition in 1901. [1] This condition appears to be extremely rare.

The term is sometimes used as a diagnostic title for hyperhidrosis of the nose. [2]

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Pathophysiology

Pathogenesis is unknown. Granulosis rubra nasi is a nonneoplastic disorder of the eccrine glands, possibly representing a unique form of sweat retention. [3] Eccrine glands produce sweat, and an alteration in the rate of sweat secretion manifests as hypohidrosis or hyperhidrosis. [4] A marked increase in sweat production on the nose occurs in granulosis rubra nasi. Hyperhidrosis also is present on the central face, palms, and soles. [5] This hyperhidrosis appears to be responsible for the secondary changes of erythema and erythematous papules.

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Etiology

Most occurrences of granulosis rubra nasi are genetically determined, with an autosomal dominant or autosomal recessive pattern. [6, 7, 8]  The gene locus has not been identified.

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Epidemiology

Frequency

Granulosis rubra nasi is rare, and the precise frequency is unknown. Significant underreporting may be a factor.

Race

No racial predilection has been described.

Sex

No sexual predilection has been described.

Age

Granulosis rubra nasi usually starts in early childhood in patients aged 6 months to 10 years. Peak incidence is from age 7-12 years. Granulosis rubra nasi usually resolves spontaneously at puberty; however, it occasionally persists indefinitely, in which case telangiectasia becomes the predominant feature.

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Prognosis

The prognosis is excellent. Granulosis rubra nasi is primarily a cosmetic disorder. Patients have few physical complaints, and most occurrences resolve completely at puberty. Residual changes (when present) consist of superficial telangiectasias.

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Patient Education

Reassure patients that the condition is benign and usually resolves completely during puberty.

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