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Follicular Mucinosis

Alopecia Mucinosa

Follicular mucinosis is a cutaneous disorder distinguished by the buildup of mucin in the sebaceous glands and the pilosebaceous follicles. It is associated with a number of diseases, including systemic lupus erythematosus, mycosis fungoides, and several dermatoses, but it may also arise as a separate clinical entity. The presence of single or multiple well-delineated erythematous plaques with alopecia is the typical presentation. Histological examination of the lesions is necessary to make the diagnosis.


Presentation

Follicular mucinosis (also known as alopecia mucinosa) is a rare cutaneous disease characterized by the accumulation and deposition of mucin in hair follicles and sebaceous glands, with follicular keratinocytes being the presumable origin of excessive mucin [1] [2] [3] [4]. The pathogenesis of follicular mucinosis is yet to be revealed, and the clinical presentation varies only slightly across different subtypes that have been described in the literature [1] [4] [5] [6] [7]

  • Idiopathic (primary or benign) - In the absence of an underlying etiology that could be responsible for cutaneous symptoms, primary or idiopathic follicular mucinosis is predominantly encountered in children and young adults as solitary lesions in the form of erythematous plaques or nodules accompanied by alopecia on the head and neck area, including the face [2] [3] [7]. Plaques are well-demarcated, skin-colored or pink, show signs of scaling and spontaneously resolve within a period of months or years [2] [4] [7]. In some patients, a prolonged and more chronic course of the disease has been reported, distinguished by several relapses and dissemination of lesions on the trunk and the extremities [3].
  • Secondary - The presence of generalized erythematous plaques and alopecia is almost universally associated with some underlying disorder [2] [3] [7], and patients are much older compared to primary forms (40-70 years of age) [4]. Mycosis fungoides and Sézary syndrome (cutaneous T-cell lymphomas) are two most important pathologies linked to follicular mucinosis; systemic lupus erythematosus (SLE), various dermatoses (eczema, alopecia areata, insect bites, etc.) have all been recognized in secondary forms of this cutaneous disorder [1] [3] [5] [7].
Weakness
  • A weak interkeratinocyte staining for HA was also observed in the interfollicular epidermis.[ncbi.nlm.nih.gov]
Alopecia
  • Alopecia mucinosa What causes alopecia mucinosa? The cause of alopecia mucinosa is unknown, but it may have something to do with circulating immune complexes and cell-mediated immunity.[dermnetnz.org]
  • KEYWORDS: alopecia areata; alopecia mucinosa; follicular mucinosis[ncbi.nlm.nih.gov]
  • The presence of single or multiple well-delineated erythematous plaques with alopecia is the typical presentation. Histological examination of the lesions is necessary to make the diagnosis.[symptoma.com]
  • Abstract The pathological features of alopecia areata were described in association with those of follicular mucinosis in the scalp pathology of an 18-year-old woman.[ncbi.nlm.nih.gov]
  • Clinical and histological features of eosinophilic pustular folliculitis with follicular mucinosis and alopecia mucinosa are very similar. Alopecia mucinosa may be a clonal T-cell dermatosis.[ncbi.nlm.nih.gov]
Anhidrosis
  • All reported cases with syringolymphoid hyperplasia were men (10 of 10), with the clinical findings of alopecia (9 of 10) and anhidrosis (3 of 10). Only 3 of 10 cases had associated follicular mucinosis.[ncbi.nlm.nih.gov]
  • Lesional pruritis or intense itching, dysethesia (burning or tingling sensation), and anhidrosis (the inability to sweat in response to heat) are documented clinical symptoms of alopecia mucinosa.[keratin.com]
Tremor
  • The symptoms are usually non-specific, such as fatigue, weight loss, tremor , visual changes , heart irregularities, diarrhea , high blood presssure, etc.[healthtap.com]

Workup

Because of the wide differential diagnosis that may be seen with follicular mucinosis, particularly in older adults, the physician must conduct a thorough clinical investigation. Firstly, the course of symptoms, their severity, as well as their progression or possible relapse should be assessed. It must be noted that some of the underlying etiologies (eg. mycosis fungoides) might develop before, after, or together with follicular mucinosis, which is why a detailed personal history is essential in revealing disease that can point to the causative illness [1] [7]. A meticulous inspection of the skin that will allow detection of typical erythematous plaques and alopecia is perhaps even more important in narrowing the number of possible diagnoses. To confirm follicular mucinosis, however, a biopsy of the lesion and subsequent histopathological examination is necessary [1] [3] [4]. Confirmation of mucin deposition in the external root of the hair follicle and sebaceous glands and an abundant lymphocytic infiltrate in the follicles are main microscopic findings [1] [7]. Many studies have attempted to find histological criteria that could differentiate between idiopathic and secondary forms of follicular mucinosis, but the appearance of both subtypes on histology is virtually identical [2] [5]. Thus, the classification rests on clinical criteria and the ability of the physician to recognize main clinical features.

Staphylococcus Aureus
  • Follicular mucinosis, Staphylococcus aureus, and oligoclonal expansion of the T-cell receptor V beta chain genes 6 and 7 were present in the skin.[ncbi.nlm.nih.gov]
  • One month prior to presentation a culture was taken that was positive for methicillin-sensitive Staphylococcus aureus-associated impetigo.[ncbi.nlm.nih.gov]
  • Covers new and more commonly presenting disorders in 12 new chapters on today s hot topics, such as methocillin-resistant staphylococcus aureus, atypical nevi, autoimmune progesterone dermatitis, and more.[books.google.com]
  • The exact pathogenesis is unknown, although the role of circulating immune complexes and cell-mediated immunity has been considered including a reaction to persistent antigens such as Staphylococcus aureus . 7) Microscopic features: The lesion is characterized[histopathology-india.net]
  • Staphylococcus aureus can be isolated from the pustules in most cases, but whether this is a primary or secondary process is unknown.[omicsonline.org]

Treatment

  • No single treatment has been shown to be consistently effective.[ncbi.nlm.nih.gov]
  • There is no standard treatment for PFM, and a wide variety of therapies have been described. There are no previous reports of treatment of PFM with imiquimod. 2012 Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • Three patients showed complete clearing after anti-leprosy treatment, and the fourth patient is currently taking anti-leprosy treatment and showed good clinical response.[ncbi.nlm.nih.gov]
  • We sought to describe clinical and histopathologic findings of children with FM with and without MF, as well as treatments administered and clinical outcomes.[ncbi.nlm.nih.gov]
  • Numerous treatments for primary follicular mucinosis have been described, which include isotretinoin and glucocorticoids, whereas treatment of the underlying disease is necessary in the secondary form.[ncbi.nlm.nih.gov]

Prognosis

  • […] the literature of follicular mucinosis appearing as an acneiform eruption of the face and neck, we were unable to find any case evolving in lymphoma, suggesting that this form is a particular clinical sub-type of follicular mucinosis with a favorable prognosis[ncbi.nlm.nih.gov]
  • When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis.[ncbi.nlm.nih.gov]
  • […] report a case of PFM with identical T-cell clone rearrangement at anatomically distinct sites, supporting the idea that some authors have proposed, that FM may represent a low-grade lymphoproliferative disease related to mycoses fungoides with favorable prognosis[ncbi.nlm.nih.gov]

Etiology

  • MeSH terms Alopecia/etiology* Child Diagnosis, Differential Eosinophilia/etiology Female Humans Lymphoproliferative Disorders/complications* Lymphoproliferative Disorders/diagnosis Lymphoproliferative Disorders/genetics Lymphoproliferative Disorders/pathology[ncbi.nlm.nih.gov]
  • The pathogenesis of follicular mucinosis is yet to be revealed, and the clinical presentation varies only slightly across different subtypes that have been described in the literature Idiopathic (primary or benign) - In the absence of an underlying etiology[symptoma.com]
  • Abstract Follicular mucinosis is a rare disorder of unknown etiology characterized by accumulation of mucin in the sebaceous glands and outer root sheaths of the hair follicles.[ncbi.nlm.nih.gov]
  • […] moderate to dense superficial and deep perivascular perifollicular lymphohistiocytic infiltrate with numerous eosinophils, marked mucin deposition and conspicuous disruption of the follicular epitheliumThe pathologic diagnosis includes, “infectious etiology[vgrd.blogspot.com]
  • Picture Diseasesof the Skin and its Appendages Lethal Mutations Surviving by Mosaicism References Clinical Aspects Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology[books.google.de]

Epidemiology

  • Epidemiology Coexisting lymphoma associated with very poor prognosis Clinical features Also called follicular mucinosis Edematous and erythematous plaques of alopecia on head and neck Children: benign, self-limited Adults: associated with cutaneous T[pathologyoutlines.com]
  • Epidemiology Follicular mucinosis is a rare condition with both genders being involved; however males tend to be diagnosed more than females.[xpertdox.com]
  • Epidemiology and genetics of alopecia areata. Clin Exper Dermatol. 2002;27:405-09. Hordinsky M, Sawaya M, Roberts JL. Hair loss and hirsutism in the elderly. Clin Geriatr Med. 2002;18:121-33. Papadopoulos AJ, Schwartz RA, Janniger CK.[rarediseases.org]
  • (effective October 01, 2015) Signs and Symptoms Itchy, painful, peeling and reddened skin Ulcerated skin lesion s, which may become infected Diagnostic Exams Flow cytometry Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology[seer.cancer.gov]
  • Lichen simplex chronicus Hypertrophic lichen planus Discoid lupus erythematosus Acne vulgaris Pseudolymphoma Melanocytic nevi Arthropod bite Atopic dermatitis Grover's disease Actinic prurigo Infundibulofolliculitis OUTLINE EPIDEMIOLOGY CHARACTERIZATION[thedoctorsdoctor.com]
Sex distribution
Age distribution

Pathophysiology

  • Case Overview alopecia mucinosa Member Rated 0 Patient case no. 1778 Date added 28 April 2003 Patient details Age --Undetermined-- Localisation Head / face / cheek Primary Lesions Papule / erythematous Alopecia / non scarring Pathophysiology deposition[dermquest.com]
  • Case Overview follicular mucinosis Member Rated 0 Patient case no. 2401 Date added 15 June 2003 Patient details Age --Undetermined-- Localisation Head / face / forehead Description Primary Lesions Nodule Papule / erythematous Pathophysiology deposition[dermquest.com]
  • Author information 1 Section of Dermatology, Fondazione IRCCS Cà Granda-Ospedale Maggiore Policlinico di Milano, Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy. [email protected] Abstract Follicular mucinosis represents[ncbi.nlm.nih.gov]
  • Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology and Pathogenesis Hypomelanotic Hair Disorders 2Modelsfor Cultivating Follicular Keratinocytes In Vitro Pathophysiological[books.google.de]
  • Pathophysiology It has been suggested that the keratinocytes that form the affected follicles produce intracellular mucin and they eventually degenerate, which is induced by the T lymphocytes of the infiltrate. [6] Causes The etiology of alopecia mucinosa[wikidoc.org]

Prevention

  • Precise recognition and understanding of this distinctive, reactive histological pattern may prevent our patients from unnecessary diagnostic and therapeutic strategies.[ncbi.nlm.nih.gov]
  • And hence, no guidelines or methods exist presently for the prevention of this condition.[dovemed.com]
  • The exact cause of Primary Chronic Alopecia Mucinosa is unknown, and hence, currently, there are no guidelines or methods to prevent the condition.[dovemed.com]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]

References

Article

  1. Passos PCV da R, Zuchi MF, Fabre AB, Martins LEAM. Follicular mucinosis - Case report . An Bras Dermatol. 2014;89(2):337-339.
  2. Lewars M, Levin J, Purcell S. Follicular mucinosis. Indian Dermatol Online J.. 2013;4(4):333-335.
  3. Fonseca APM, Campelo FS, Bona AH, Rêgo PMM, Fonseca WSM. Follicular mucinosis: literature review and case report. An Bras Dermatol. 2002;77:701–706.
  4. Westphal DC, Pennini SN, de Souza PP, Maquiné GÁ, Schettini APM, Santos M. Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area. An Bras Dermatol. 2015;90(3 Suppl 1):147-149.
  5. Rongioletti F, De Lucchi S, Meyes D, et al. Follicular mucinosis: a clinico pathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides - associated follicular mucinosis. J Cutan Pathol. 2010;37:15–19.
  6. O'Reilly K, Brauer J, Loyd A, Patel R, Walters R, Franks AG Jr. Secondary follicular mucinosis associated with systemic lupus erythematosus. Dermatol Online J. 2010;16(11):7.
  7. Rupnik H, Podrumac B, Zgavec B, Lunder T. Follicular mucinosis in a teenage girl. Acta Dermatovenerol Alp Pannonica Adriat. 2005;14(3):111-114.

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Last updated: 2018-06-22 02:37