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. 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. This hyperhidrosis appears to be responsible for the secondary changes of erythema and erythematous papules.
Epidemiology
Frequency
United States
Granulosis rubra nasi is rare, and the precise frequency is unknown. Significant underreporting may be a factor.
International
The frequency is expected to be similar to that seen in the United States.
Mortality/Morbidity
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.
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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Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. St. Louis, Mo: Mosby; 2003.
Wenzel FG, Horn TD. Nonneoplastic disorders of the eccrine glands. J Am Acad Dermatol. Jan 1998;38(1):1-17; quiz 18-20. [Medline].
Heid E, Samain F, Jelen G, Boivin S. [Granulosis rubra nasi and pheochromocytoma]. Ann Dermatol Venereol. 1996;123(2):106-8. [Medline].
Binazzi M. [Further data on a case of hereditary granulosis rubra nasi.]. Rass Dermatol Sifilogr. Jan-Feb 1958;11(1):23-6. [Medline].
Hellier FF. Granulosa rubra nasi in mother and daughter. Brit Med J. 1937;2:1068.
Veltman G. [Uber das familiare Vorkommen der Granulosis rubra nasi.]. Arch Klin Exp Dermatol. 1949;188:188-96.
Sonthalia S, Singal A, Sharma R. Hyperhidrosis, vesicles, and papules over the nose: Granulosis rubra nasi. Indian J Dermatol Venereol Leprol. Jan 2012;78(1):97-8. [Medline].
Grazziotin TC, Buffon RB, da Silva Manzoni AP, Libis AS, Weber MB. Treatment of granulosis rubra nasi with botulinum toxin type A. Dermatol Surg. Aug 2009;35(8):1298-9. [Medline].
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