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].

• Inflammation

  When the mucinous material settles in the sebaceous glands and hair follicles, it causes inflammation that eventually disrupts the follicles’ ability to make hair. [explainz.com]

  Four cases of suspected leprosy showed, on biopsy, follicular mucinosis without any granulomatous inflammation. All the patients were adolescents (12-17 years) with a single lesion on the face. [ncbi.nlm.nih.gov]

  Figure 2: Histology slide showing Follicular Decalvans: heavy perifollicular chronic inflammation and ruptured folliculitis. He also had a thick, erythematous plaque with loss of hair over his right eyebrow, as shown in Figure 2. [omicsonline.org]

  Polyclonal (many types) hypergammaglobulinemia is a benign condition due to infection, inflammation causing non-specific gamma globulin production such as in hepatitis, immune and allergic disorders. ...Read more See 1 more doctor answer 4 What are the [healthtap.com]

  If there are signs of inflammation the use of a potent topical or intralesional corticosteroid may arrest/slow progression. [pcds.org.uk]

• Myxedema

  […] mucinosis [ mu″si-no´sis ] a state with abnormal deposits of mucin in the skin, often associated with hypothyroidism (myxedema). follicular mucinosis a disease of the pilosebaceous unit, presenting clinically as grouped follicular papules or plaques with [medical-dictionary.thefreedictionary.com]

  Content View Hits : 13611603 flag counter find us on Mucinoses There are six types of cutaneous mucinosis: (a) generalized myxedema, (b) pretibial myxedema, (c) lichen myxedematosus or papular mucinosis, (d) reticular erythematous mucinosis or plaquelike [dermaamin.com]

  [C17.300.550.590] Myxedema Scleredema Adultorum [C17.300.550.750] Scleredema Adultorum Scleromyxedema [C17.300.550.875] Scleromyxedema DISEASES Skin and Connective Tissue Diseases [C17] Skin and Connective Tissue Diseases Skin Diseases [C17.800] Skin [decs.bvsalud.org]

  ( ICD-10-CM Diagnosis Code E03.9 Hypothyroidism, unspecified 2016 2017 2018 2019 Billable/Specific Code Applicable To Myxedema NOS E03.9 ) The following code(s) above L98.5 contain annotation back-references Annotation Back-References In this context [icd10data.com]

• Weight Loss

  The symptoms are usually non-specific, such as fatigue, weight loss, tremor, visual changes, heart irregularities, diarrhea, high blood presssure, etc. [healthtap.com]

  Associated lymphoma tends (although not invariably) to be associated with age over 30 years, a wider distribution of lesions, and possibly systemic features such as night sweats, weight loss, or lymphadenopathy. [clinicalgate.com]

  referred to as anagen effluvium (figure 4) Acute telogen effluvium Normally occurs two to three months after a triggering event such as pyrexia (in particular a body temperature of 102.5 degrees Fahrenheit and above), child-bearing, major surgery, weight [pcds.org.uk]

• Swelling

  […] 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, clogs, or inflames your skin can cause symptoms such as redness, swelling [icdlist.com]

• Diarrhea

  The symptoms are usually non-specific, such as fatigue, weight loss, tremor, visual changes, heart irregularities, diarrhea, high blood presssure, etc. [healthtap.com]

• 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]

  Clinically Follicular Mucinosis (FM) appears as follicular papules and can have scarring alopecia but alopecia however is not always present [ 7 ]. [omicsonline.org]

  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]

  MUCINOSA 1965-92 (see under ALOPECIA 1965-90) Nota Online: use MUCINOSIS, FOLLICULAR to search ALOPECIA MUCINOSA 1966-92 Nota de historia: 93; was ALOPECIA MUCINOSA 1965-92 (see under ALOPECIA 1965-90) Identificador de DeCS: 30908 ID del Descriptor: [decs.bvsalud.org]

  KEYWORDS: alopecia areata; alopecia mucinosa; follicular mucinosis [ncbi.nlm.nih.gov]

• Eruptions

  Follicular Mucinosis Presenting as an Acneiform Eruption: Report of Four Cases. J Am Acad Dermatol. 1998;38(5 Pt 2):849-51. PubMed PMID: 9591801. TY - JOUR T1 - Follicular mucinosis presenting as an acneiform eruption: report of four cases. [unboundmedicine.com]

  Follicular mucinosis is usually regarded as an eruption consisting of follicular papules or infiltrated plaques with prominent follicular openings associated with loss of hair. [ncbi.nlm.nih.gov]

• Pruritus

  Genetic basis of cutaneous diseases Arne Kanig and Rudolf Happle Ichthyoses erythrokeratodermas and related disorders Keratodermas Kro Dariers disease and HaileyHailey disease Daniel Hohl Theodora Mauro and leanPhilippe Gorog Mosaicism and linear lesions Pruritus [books.google.com]

  In view of affected quality of life patient was started on oral cyclosporine 5 mg/kg day in 3 divided doses after recording two normal baseline blood pressure readings along with potent topical steroids, moisturizers and oral antihistamines for pruritus [e-ijd.org]

  There is usually little or no pruritus. [ijdvl.com]

  Severe pruritus was seen in 68% of patients [ 7 ]. 2 adult patients who presented with alopecia and disseminated follicular erythematous papules, comedones and cysts were described. [omicsonline.org]

  He reported pruritus of these lesions, but review of systems was otherwise negative. [omicsgroup.org]

• Skin Rash

  The medication we're trying next has some possible side effects including a skin rash worse than she has, increased joint and muscle pain, and loss of pigment in her retina which can result in blindness. [gofundme.com]

  The signs and symptoms may include: Presence of red skin rashes (or weals) on hair-bearing skin Fluid accumulation may be observed around the rashes Loss of hair at the affected skin regions is not typically noted, even though hair-bearing skin is involved [dovemed.com]

• Dysesthesia

  […] cutaneous diseases Arne Kanig and Rudolf Happle Ichthyoses erythrokeratodermas and related disorders Keratodermas Kro Dariers disease and HaileyHailey disease Daniel Hohl Theodora Mauro and leanPhilippe Gorog Mosaicism and linear lesions Pruritus and dysesthesia [books.google.com]

  Rare cases of local dysesthesia have been reported. [3] A coexisting lymphoma is associated with follicular mucinosis in about 15% of cases. [4] Case Report A 62-year-old male presented with multiple, discrete skin coloured to reddish papules on the face [ijdvl.com]

• Seizure

  Some medications that commonly cause telogen effluvium are anti-seizure medications, beta-blockers for high blood pressure, and anti-thyroid medications. In this situation, these medications should only be stopped after discussion with your doctor. [americanskin.org]

• Headache

  / above the ears The presence of retained hairs along the frontal and / or temporal rim, termed the “fringe sign,” is a common finding in both early and late traction alopecia, and is a useful clinical marker of the condition Can be associated with headaches [pcds.org.uk]

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.

No single treatment has been shown to be consistently effective. Topical bexarotene 1% gel should be considered for patients with idiopathic follicular mucinosis resistant to standard treatment. [ncbi.nlm.nih.gov]

What is the treatment for alopecia mucinosa? There is no proven effective treatment for alopecia mucinosa. Usually, primary and acute alopecia mucinosa occurring in children resolves spontaneously. [dermnetnz.org]

[…] 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 [moh-it.pure.elsevier.com]

Folliculotropic MF seems to represent a specific clinicopathologic entity which may have a poorer prognosis than classic MF. [ncbi.nlm.nih.gov]

Alopecia mucinosa is a disease of unknown etiology, characterized clinically by plaques of folliculopapules and histologically by mucinosis of the pilosebaceous unit. [jamanetwork.com]

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]

Ofuji papuloerythroderma is a distinctive clinical entity of unknown etiology, which may occasionally be associated with B cell and T cell lymphoma or visceral malignancy. [ncbi.nlm.nih.gov]

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]

Calificadores permitidos: BL blood CF cerebrospinal fluid CI chemically induced CL classification CN congenital CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology [decs.bvsalud.org]

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]

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]

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]

Precise recognition and understanding of this distinctive, reactive histological pattern may prevent our patients from unnecessary diagnostic and therapeutic strategies. [ncbi.nlm.nih.gov]

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]

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.