Alopecia Mucinosa

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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.

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]


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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]

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:

History

The presenting sign of alopecia mucinosa is hair loss in hair-bearing areas. Skin eruptions present as pruritic, pink–to–yellow-white, follicular papules and plaques. Lesions may be isolated or multiple. Mycosis fungoides is recognized at the time of diagnosis in approximately 15-30% of patients with alopecia mucinosa.

Physical

The clinical manifestations of alopecia mucinosa are grouped follicular papules and alopecia. Nodules, plaques, and patches of follicular papules may exist. Occasionally, mucinous material can be expressed from active lesions, and erythema and scaling are usually present. The face and the scalp are the most common sites of involvement.

The alopecia that develops on hair-bearing skin is of the nonscarring type (see the image below).


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Courtesy of San Antonio Uniformed Services Health Education Consortium (SAUSHEC) teaching files.

Usually, the alopecia is reversible unless follicular destruction has occurred due to excess mucin in the outer root sheath and sebaceous glands. In patients with permanent alopecia, the whole follicle degenerates, and the cystic cavity becomes blocked with keratinous debris. When the plugs persist, they are obvious features on healed, hairless patches of alopecia mucinosa.

Causes

Alopecia mucinosa represents various stages of follicular damage leading to hair loss. The reactive process is of unknown etiology. The role of circulating immune complexes and cell-mediated immunity has been considered.

Laboratory Studies

A history and physical examination are the first steps.

Procedures

Multiple skin biopsy samples are needed in the evaluation of patients with alopecia mucinosa because of the association with lymphoma.

Histologic Findings

The characteristic features of alopecia mucinosa are follicular degeneration with the accumulation of mucin within the follicles (see the image below). Early lesions contain an abundance of mucin between the decaying root sheath cells or pooling in localized collections. The mucinous degeneration begins in the pilosebaceous units. A periappendiceal, perivascular, or interstitial lymphocytic mixed inflammatory cell infiltrate often exists. In patients with chronic alopecia mucinosa, the histologic analysis demonstrates the presence of distorted follicles with variable viability.


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Courtesy of Dirk M. Elston, MD.

Differentiation between the variants of alopecia mucinosa is difficult; thus, several criteria are used to differentiate the histologic features of benign alopecia mucinosa and mycosis fungoides–associated alopecia mucinosa.[11] Although no single criterion is diagnostic,[12] cytologic atypia and a bandlike infiltrate are more common in mycosis fungoides. In addition, Pautrier microabscesses in the epidermis and upper follicle are rarely found in benign alopecia mucinosa but are common in mycosis fungoides. The benign inflammatory process is typically characterized by an infiltrate confined to the follicular, perifollicular, or perivascular zones with no extension of cells into the epidermis or papillary/reticular dermis. On the other hand, the typical infiltrate associated with mycosis fungoides involves the upper dermis invading the epidermis and includes characteristic tumor cells in the damaged follicle, epidermis, and/or dermis.

Medical Care

No uniformly effective therapy exists for alopecia mucinosa, although several treatments are routinely used. Treatments include topical, intralesional, and systemic corticosteroids. In addition, topical and systemic psoralen plus ultraviolet A light (PUVA) therapy,[13] topical nitrogen mustard, and radiation therapy have demonstrated some success. Isolated cases document the beneficial responses of dapsone, indomethacin, and interferons.[14] Because of the variable course of the disease and the likelihood of spontaneous resolution, therapeutic efficacy is difficult to prove.

Further Outpatient Care

Close follow-up care with all patients is required.

Complications

Although the question of whether alopecia mucinosa is a transitional state evolving into mycosis fungoides is unresolved, it is proven that alopecia mucinosa may precede the development of mycosis fungoides by several years. Thus, additional biopsy specimens and extremely close follow-up care are crucial in all variants of alopecia mucinosa.

Prognosis

The prognosis of alopecia mucinosa depends on the specific clinical variant, as follows:

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

Disclosure: Amgen/Pfizer Honoraria Consulting; GlaxoSmithKline None Investigator; Novartis Honoraria Consulting; Ranbaxy None Consulting; Pfizer None Consulting; BioLineRX, Ltd. Honoraria Consulting; Celgene Corporation Consulting; Clinuvel None Investigator; Eli Lilly & Co. Honoraria Consulting; Genentech None Consulting

Specialty Editors

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

Disclosure: Nothing to disclose.

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

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

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

Disclosure: Nothing to disclose.

Additional Contributors

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. Aug 1983;119(8):690-7. [View Abstract]
  2. Dalle S, Marrou K, Balme B, Thomas L. Neonatal follicular mucinosis. Br J Dermatol. Sep 2007;157(3):609-10. [View Abstract]
  3. Buchner SA, Meier M, Rufli T. Follicular mucinosis associated with mycosis fungoides. Dermatologica. 1991;183(1):66-7. [View Abstract]
  4. Clark-Loeser L, Latkowski JA. Follicular mucinosis associated with mycosis fungoides. Dermatol Online J. Nov 30 2004;10(3):22. [View Abstract]
  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. [View Abstract]
  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. [View Abstract]
  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. [View Abstract]
  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. [View Abstract]
  9. Roth DE, Owen LG, Hodge SJ, Callen JP. Follicular mucinosis associated with pregnancy. Int J Dermatol. Jun 1992;31(6):441-2. [View Abstract]
  10. Lockshin BN, Khachemoune A, Cohen C. Follicular mucinosis in a 4-year-old boy. Int J Dermatol. Dec 2004;43(12):950-2. [View Abstract]
  11. Nickoloff BJ, Wood C. Benign idiopathic versus mycosis fungoides-associated follicular mucinosis. Pediatr Dermatol. Mar 1985;2(3):201-6. [View Abstract]
  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. [View Abstract]
  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. [View Abstract]
  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. [View Abstract]
  15. Coskey RJ, Mehregan AH. Alopecia mucinosa. A follow-up study. Arch Dermatol. Aug 1970;102(2):193-4. [View Abstract]
  16. Emmerson RW. Follicular mucinosis. A study of 47 patients. Br J Dermatol. Jun 1969;81(6):395-413. [View Abstract]

Courtesy of Dirk M. Elston, MD.

Courtesy of San Antonio Uniformed Services Health Education Consortium (SAUSHEC) teaching files.

Courtesy of Dirk M. Elston, MD.

Courtesy of Dirk M. Elston, MD.

Courtesy of Dirk M. Elston, MD.

Courtesy of San Antonio Uniformed Services Health Education Consortium (SAUSHEC) teaching files.