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Alopecia Mucinosa

  • Author: Gervaise L Gerstner, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 08, 2015
 

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

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

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 three 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: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.

Specialty Editor Board

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

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate 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, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Acknowledgements

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.

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. 1983 Aug. 119(8):690-7. [Medline].

  2. Dalle S, Marrou K, Balme B, Thomas L. Neonatal follicular mucinosis. Br J Dermatol. 2007 Sep. 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. 2004 Nov 30. 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. 1993 Aug. 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. 2011 Jan. 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. 2005 Jul. 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. 2006 Mar. 5(3):228-31. [Medline].

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

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

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

  12. Gibson LE, Muller SA, Leiferman KM, Peters MS. Follicular mucinosis: clinical and histopathologic study. J Am Acad Dermatol. 1989 Mar. 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. 2008 Mar. 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. 1991 May. 24(5 Pt 2):848-50. [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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