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