Alopecia Mucinosa 

  • Author: Gervaise L Gerstner, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 13, 2012
 

Background

Alopecia mucinosa, often referred to as follicular mucinosis, was first reported by Pinkus in 1957.[1] The dermatologic eruptions consist of follicular papules and/or indurated plaques that demonstrate distinct histologic changes in the hair follicles that lead to hair loss. The accumulation of mucinous material in the damaged hair follicles and sebaceous glands creates an inflammatory condition and subsequent degenerative process. The face, the neck, and the scalp are the most frequently affected sites, although lesions may appear on any part of the body.

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Pathophysiology

Alopecia mucinosa is a disease process defined histopathologically by mucin deposition in hair follicles and sebaceous glands, which undergo epithelial reticular degeneration. The exact pathogenesis is unknown, although the role of circulating immune complexes and cell-mediated immunity has been considered. The 3 clinical variants of the disease consist of a primary acute disorder of young persons, a primary chronic disorder of older persons, and a secondary disorder associated with benign or malignant disease.

The primary disorder of young persons consists of focal cutaneous lesions with limited progression. Lesions are typically limited to the head, the neck, and the shoulders (see the image below). Most lesions spontaneously resolve between 2 months and 2 years. Pediatric cases comprise most of this type of alopecia mucinosa, with the remainder of patients being younger than 40 years. Neonatal follicular mucinosis has also been reported.[2]

Courtesy of Dirk M. Elston, MD. Courtesy of Dirk M. Elston, MD.

Primary chronic alopecia mucinosa of older persons affects people older than 40 years. Lesions have a widespread distribution, and they may persist or recur indefinitely. No associated disorders are identified.

The secondary alopecia mucinosa may be associated with either benign disease or malignant disease. These patients are usually aged 40-70 years, and the lesions are widespread and numerous. Alopecia mucinosa can occur secondary to benign disease including the inflammatory conditions lupus erythematosus, lichen simplex chronicus, and angiolymphoid hyperplasia. Secondary alopecia mucinosa is also associated with malignant disease, including mycosis fungoides, Kaposi sarcoma, and Hodgkin disease; mycosis fungoides is by far the most common association.[3, 4, 5, 6]

In most patients who exhibit both alopecia mucinosa and mycosis fungoides, these conditions appear to develop concomitantly; however, the concern exists that individuals exhibiting only alopecia mucinosa may also be at risk for subsequent development of lymphoma.

Drug-induced alopecia mucinosa has been associated with the use of adalimumab[7] and imatinib.[8]

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Epidemiology

Frequency

United States

Alopecia mucinosa is a rare condition. The precise data on its frequency are not available.

Mortality/Morbidity

Mortality is related to the coexistence of mycosis fungoides in secondary alopecia mucinosa. An estimated 15-40% of adults with alopecia mucinosa will eventually develop lymphoma, if they do not already have it. The malignant potential of alopecia mucinosa cannot be fully assessed because of the enigmatic nature of this and other cutaneous T-cell abnormalities. The morbidity of primary alopecia mucinosa is generally restricted to cosmesis; whereas, in cases of secondary alopecia mucinosa, morbidity is related to the associated disease process.

Race

No racial predilection is reported.

Sex

Although both sexes are affected by alopecia mucinosa, the disorder is more frequent in males than in females. Alopecia mucinosa in pregnancy is reported only once in the literature.[9]

Age

The 3 subsets of alopecia mucinosa breakdown by age, as follows:

  • Primary localized disease affects patients younger than 40 years and primarily occurs in the pediatric population.[10]
  • Primary generalized disease affects people older than 40 years.
  • Secondary disease with either a benign or a malignant association usually affects people in the fifth through eighth decades of life.
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Contributor Information and Disclosures
Author

Gervaise L Gerstner, MD  Assistant Clinical Professor, Department of Dermatology, Mount Sinai Hospital; Partner, Park Avenue Skin Care

Disclosure: Nothing to disclose.

Coauthor(s)

Mark G Lebwohl, MD  Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Amgen/Pfizer Honoraria Consulting; Centocor/Janssen Honoraria Consulting; DermiPsor Honoraria Consulting; GlaxoSmithKline Honoraria Consulting; HelixBioMedix Honoraria Consulting; Novartis Honoraria Consulting; Ranbaxy Lectures; Can-Fite Biopharma Honoraria Consulting; DermaGenoma Honoraria Consulting; Biosynexus Honoraria Consulting

Specialty Editor Board

Günter Burg, MD  Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland

Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

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

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.

References
  1. Pinkus H. Centennial Paper. Alopecia mucinosa. Inflammatory plaques with alopecia characterized by root-sheath mucinosis. By Hermann Pinkus, M.D., Arch Dermatol 1957. Arch Dermatol. Aug 1983;119(8):690-7. [Medline].

  2. Dalle S, Marrou K, Balme B, Thomas L. Neonatal follicular mucinosis. Br J Dermatol. Sep 2007;157(3):609-10. [Medline].

  3. Buchner SA, Meier M, Rufli T. Follicular mucinosis associated with mycosis fungoides. Dermatologica. 1991;183(1):66-7. [Medline].

  4. Clark-Loeser L, Latkowski JA. Follicular mucinosis associated with mycosis fungoides. Dermatol Online J. Nov 30 2004;10(3):22. [Medline].

  5. Lacour JP, Castanet J, Perrin C, Ortonne JP. Follicular mycosis fungoides. A clinical and histologic variant of cutaneous T-cell lymphoma: report of two cases. J Am Acad Dermatol. Aug 1993;29(2 Pt 2):330-4. [Medline].

  6. Bi MY, Curry JL, Christiano AM, Hordinsky MK, Norris DA, Price VH, et al. The spectrum of hair loss in patients with mycosis fungoides and Sézary syndrome. J Am Acad Dermatol. Jan 2011;64(1):53-63. [Medline].

  7. Dalle S, Balme B, Berger F, Hayette S, Thomas L. Mycosis fungoides-associated follicular mucinosis under adalimumab. Br J Dermatol. Jul 2005;153(1):207-8. [Medline].

  8. Scheinfeld N. Imatinib mesylate and dermatology part 2: a review of the cutaneous side effects of imatinib mesylate. J Drugs Dermatol. Mar 2006;5(3):228-31. [Medline].

  9. Roth DE, Owen LG, Hodge SJ, Callen JP. Follicular mucinosis associated with pregnancy. Int J Dermatol. Jun 1992;31(6):441-2. [Medline].

  10. Lockshin BN, Khachemoune A, Cohen C. Follicular mucinosis in a 4-year-old boy. Int J Dermatol. Dec 2004;43(12):950-2. [Medline].

  11. Nickoloff BJ, Wood C. Benign idiopathic versus mycosis fungoides-associated follicular mucinosis. Pediatr Dermatol. Mar 1985;2(3):201-6. [Medline].

  12. Gibson LE, Muller SA, Leiferman KM, Peters MS. Follicular mucinosis: clinical and histopathologic study. J Am Acad Dermatol. Mar 1989;20(3):441-6. [Medline].

  13. Fernandez-Guarino M, Harto Castano A, Carrillo R, Jaen P. Primary follicular mucinosis: excellent response to treatment with photodynamic therapy. J Eur Acad Dermatol Venereol. Mar 2008;22(3):393-4. [Medline].

  14. Meissner K, Weyer U, Kowalzick L, Altenhoff J. Successful treatment of primary progressive follicular mucinosis with interferons. J Am Acad Dermatol. May 1991;24(5 Pt 2):848-50. [Medline].

  15. Coskey RJ, Mehregan AH. Alopecia mucinosa. A follow-up study. Arch Dermatol. Aug 1970;102(2):193-4. [Medline].

  16. Emmerson RW. Follicular mucinosis. A study of 47 patients. Br J Dermatol. Jun 1969;81(6):395-413. [Medline].

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Courtesy of Dirk M. Elston, MD.
Courtesy of Dirk M. Elston, MD.
Courtesy of San Antonio Uniformed Services Health Education Consortium (SAUSHEC) teaching files.
 
 
 
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