Granulosis Rubra Nasi Clinical Presentation

  • Author: Basil M Hantash, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Feb 27, 2012
 

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.

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Physical

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.[4] Surgical removal of the pheochromocytoma was followed by involution of the hyperhidrosis, regression of the nasal dermatosis, and normalization of urinary catecholamines.
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Causes

Most occurrences of granulosis rubra nasi are genetically determined, with an autosomal dominant or autosomal recessive pattern.[5, 6, 7] The gene locus has not been identified.

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Contributor Information and Disclosures
Author

Basil M Hantash, MD, PhD  Chairman, Elixir Institute of Regenerative Medicine

Basil M Hantash, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery, Sigma Xi, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Rashid M Rashid, MD, PhD  Resident Physician, Department of Dermatology, University of Texas, Houston, MD Anderson Cancer Center, and Morzak Research Initiative

Rashid M Rashid, MD, PhD is a member of the following medical societies: American Academy of Dermatology, Council for Nail Disorders, Houston Dermatological Society, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Shyam Verma  MBBS, DVD, FAAD, Clinical Associate Professor, Department of Dermatology, University of Virginia; Adjunct Associate Professor, Department of Dermatology, State University of New York at Stonybrook, Adjunct Associate Professor, Department of Dermatology, University of Pennsylvania

Shyam Verma is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mark Crowe, MD, to the development and writing of this article.

References

  1. Maschkilleisson LN, Naradow LA. Jadassohns Granulosis rubra nasi. Derm Z. 1935;71:79-84.

  2. Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. St. Louis, Mo: Mosby; 2003.

  3. Wenzel FG, Horn TD. Nonneoplastic disorders of the eccrine glands. J Am Acad Dermatol. Jan 1998;38(1):1-17; quiz 18-20. [Medline].

  4. Heid E, Samain F, Jelen G, Boivin S. [Granulosis rubra nasi and pheochromocytoma]. Ann Dermatol Venereol. 1996;123(2):106-8. [Medline].

  5. Binazzi M. [Further data on a case of hereditary granulosis rubra nasi.]. Rass Dermatol Sifilogr. Jan-Feb 1958;11(1):23-6. [Medline].

  6. Hellier FF. Granulosa rubra nasi in mother and daughter. Brit Med J. 1937;2:1068.

  7. Veltman G. [Uber das familiare Vorkommen der Granulosis rubra nasi.]. Arch Klin Exp Dermatol. 1949;188:188-96.

  8. 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].

  9. 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].

  10. Pillsbury DM, Shelley WB, Kligman AM. Granulosis rubra nasi. In: Dermatology. Philadelphia, Pa: WB Saunders; 1956:835.

 
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