Granulosis Rubra Nasi 

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

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]

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.

 

Presentation

History

Initially, hyperhidrosis is the most conspicuous feature of the disease; small beads of sweat are commonly noted on the tip of the nose. Excessive sweating may precede other changes by several years.

With persistent hyperhidrosis, diffuse erythema develops on the tip of the nose. Erythema gradually extends and may involve cheeks, upper lip, and chin. Erythema is covered by small beads of sweat that also may be evident over a wider area. Small erythematous macules, erythematous papules, and vesicles or papules may form at sweat duct orifices.

Check for symptoms of pain and itching. Check for family history; the condition typically is autosomal dominant familial. Particular sensitivity to sunlight may suggest an alternative diagnosis. Check for other symptoms, such as a racing feeling of the heart or a faint feeling, which may suggest an alternative diagnosis (eg, pheochromocytoma).

Many affected patients have poor peripheral circulation and hyperhidrosis of palms and soles. Granulosis rubra nasi usually resolves spontaneously at puberty; however, it occasionally persists indefinitely, in which case telangiectasia becomes the predominant feature.

Physical Examination

Physical examination may reveal the following:

  • Marked sweating on the tip of the nose is the most conspicuous feature.

  • Redness is seen on the tip of the nose and onto the cheeks, upper lip, and chin. Red areas may be covered by small beads of sweat.

  • Small erythematous macules and papules may occur.

  • Small vesicles or pustules may form at the sweat duct orifices (miliaria crystallina).

  • The tip of the nose is cool and not infiltrated.

  • Poor peripheral circulation and hyperhidrosis of the palms and soles may be noted.

  • If the condition does not resolve completely at puberty, residual telangiectasias and small cysts may occur.

Any evidence of redness, solar lentigos, actinic changes, actinic keratoses, or skin cancers on the face, ears, or exposed areas of the arms suggests an alternative diagnosis with photosensitivity or photo damage. Evidence of chronic photo damage in childhood is rare. Photosensitive dermatoses are usually easy to differentiate from granulosis rubra nasi because they are more extensive and do not show the characteristic hyperhidrosis. Photosensitive dermatoses may produce erythema on the nasal tip and can be associated with miliaria.

Tachycardia and diffuse sweating may suggest an alternative diagnosis such as pheochromocytoma. One report exists of a 19-year-old woman with hyperhidrosis, granulosis rubra nasi, and tachycardia.[9] Surgical removal of the pheochromocytoma was followed by involution of the hyperhidrosis, regression of the nasal dermatosis, and normalization of urinary catecholamines.

Complications

No complications are known.

 

DDx

Diagnostic Considerations

Consider photosensitive dermatoses, including lupus.

For rosacea, the presentation is often with vasomotor symptoms (eg, flushing) and a later age at onset. Additionally, it tends to affect the nose and other parts of the face.

Multiple eccrine hidrocystoma also presents with multiple translucent, dome-shaped papules on the periorbital and malar areas, with the frequent presence of craniofacial hyperhidrosis. However, this often occurs in middle-aged women and does not have the background erythema of rubra nasi.[10]

Consider the diagnosis of pheochromocytoma in patients with hypersudation and tachycardia, since one report exists of a pheochromocytoma in a 19-year-old woman with hyperhidrosis, granulosis rubra nasi, and tachycardia.[9] Surgical exeresis of the pheochromocytoma was followed by involution of the hyperhidrosis, regression of nasal dermatosis, and normalization of urinary catecholamines.

Differential Diagnoses

 

Workup

Histologic Findings

Occlusion of the eccrine ducts produces cystic dilation, which may resemble hidrocystoma. A mild mononuclear cell infiltrate occurs around sweat ducts and dilation of dermal blood vessels and lymphatics. This infiltrate is not seen in hidrocystomas.

 

Treatment

Medical Care

Response to treatment, including the use of topical steroids, is usually disappointing. Assure patients that the condition usually resolves completely during puberty.

Some peer-reviewed work includes use of novel approaches such as botulinum toxin type A, with as little as a single dose.[11, 10, 12]

Tacrolimus 0.03% topical ointment has shown effective in decreasing vesicles in a 20-year-old patient who has had persistent granulosis rubra nasi.[13]

Consultations

Consultation is not usually required; however, appropriate consultation is indicated if a diagnosis of photosensitive dermatosis or pheochromocytoma is possible.

Prevention

No preventative measures are known.