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].

• 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]

  Selected Image alopecia mucinosa alopecia mucinosa alopecia mucinosa alopecia mucinosa alopecia mucinosa alopecia mucinosa Use of Cookies We use cookies to ensure that we give you the best experience on our website. [dermquest.com]

  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]

• Facial Papule

  In both cases, pathological evaluation of the facial papules revealed predominantly mucinous degeneration of the follicular epithelium, with insufficient lymphocytic infiltration or atypia to diagnose mycosis fungoides. [ncbi.nlm.nih.gov]

• Photosensitivity

  There was no hisotry of photosensitivity. He had diabetes which was under control with diet and exercise. There was no history of drug intake prior to the onset of the disease. His vital parameters were within normal limits. [ijdvl.com]

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.

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. [ncbi.nlm.nih.gov]

Treatment of follicular mucinosis There are no specific standard treatment for primary alopecia mucinosa. Mucinosis follicularis Treatment with topical, intralesional and systemic corticosteroids show mixed results. [dynamicnaturesite.blogspot.com]

Early detection and treatment may be beneficial in limiting the complications, as well as to increase the success of treatment for underlying conditions such as lymphoma Active research is currently being performed to explore the possibilities for treatment [dovemed.com]

[…] 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]

What is the Prognosis of Alopecia Mucinosa? (Outcomes/Resolutions) The prognosis of Alopecia Mucinosa is generally positive with appropriate treatment. [dovemed.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]

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]

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

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]

Epidemiology and clinical features of atopic dermatitis in India. Indian J Dermatol 2011;56:471-5. [ PUBMED ] 3. Loffreda MD. Inflammatory diseases of hair follicles, sweat glands and cartilage. In: Elder DE, editor. [e-ijd.org]

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]

And hence, no guidelines or methods exist presently for the prevention of this condition. [dovemed.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.