Summary a unique case of follicular mucinosis is described with erythroderma, boggy, purulent plagues on the scalp ami face, widespread alopecia, purulent paronychia and nail loss. May 09, 2018 as i know, cutaneous focal mucinosis cfm is a very rare reason of follicular induction of the epidermis. It has been described in association with various inflammatory and neoplastic cutaneous disorders. The primary form occurs in children and young adults. Disease bioinformatics research of mucinosis, follicular has been linked to mucinoses, mycosis fungoides, mycoses, skin neoplasms, lymphoma. Follicular mucinosis fm is a rare disorder of the skin characterized by follicular degeneration due to the accumulation of mucin within the. Follicular mucinosis fm is a primary or secondary finding in fmf, lupus. Follicular mucinosis has, clinically, keratotic follicular papules specially distributed in the scalp and face, affecting the neck, trunk and extremities. Although kreibich1 had reported a single case in 1926 and lehner and szodoray2.
Both these conditions have causative mechanisms that are not well understood and their similar timing of onset may indicate a related causative mediator. Follicular mucinosis is a chronic inflammatory disease of unknown etiology, the histopathological picture shows the presence of mucin in follicular epithelium and sebaceous glands. No uniformly effective therapy exists for follicular mucinosis, although. 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. Dec 24, 2018 follicular mucinosis is a type of skin condition in which an accumulation of mucin is observed around the hair follicles. A primary and acute disorder occurring in children and adolescents pinkus type.
It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis. Follicular mucinosis fm is a rare disorder of the skin characterized by follicular degeneration due to the accumulation of mucin within the pilosebaceous unit, with associated inflammatory changes. Follicular mucinosis lewars m, levin j, purcell s indian. Fiftynine patients with the histologic diagnosis of follicular mucinosis alopecia mucinosa were evaluated retrospectively. Three other patients had lymphoproliferative disorders, and two had kaposis sarcoma. Tan e, martinka m, ball n, shapiro j 2004 primary cicatricial alopecias. The illness was associated with an eosinophilia, an excess of abnormally shaped activated t cells in the peripheral blood and responded to oral steroid therapy. Follicular mucinosis and follicular mycosis fungoides. Provocation with standardized light testing was carried out on both normally pigmented skin and on areas of hypopigmentation representing a residual state after earlier skin lesions but without any signs of active mucinosis follicularis. The staining pattern and morphology of the folliculotropic portion were consistent with a diagnosis of lch. Follicular mucinosis developing in different locations in the same patient at similar times.
Follicular mucinosis is a cutaneous disorder distinguished by the buildup of mucin in the sebaceous glands and the pilosebaceous follicles. There are 3 conditions that belong to this group and they include alopecia mucinosa, cutaneous lymphoma related follicular mucinosis, and urticarialike follicular mucinosis. Cerroni l, finkpuches r, back b, kerl h 2002 follicular mucinosis. Since its first description by pinkus 1 in 1957 in the setting of alopecia mucinosa, 2 main forms have been classically recognized.
Immunohistochemical stains were noncontributory, and tcell gene rearrangement was positive only in the case of mycosis fungoides. Alopecia mucinosa responding to antileprosy treatment. The abnormal deposits that occur in mucinoses can be localised or widespread. Recently, i had the chance to observe a similar case. Pdf follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal. Follicular mucinosis alopecia mucinosa dermatology advisor. Follicular mucinosis is a histologic term for a reaction pattern in follicular epithelium. We present 2 cases of focal mucinosis with follicular induction and describe the histopathologic findings to create awareness of this association and to prevent confusion with other diagnoses such as dermatofibroma with follicular. Context beginning in 1957, patients have been described with localized alopecia characterized histopathologically by mucin deposition within hair follicles follicular mucinosis fm. Follicular mucinosis is a term that encompasses three related entities. To the best of our knowledge, the benefits of isotretinoin in the treatment of follicular. Alopecia mucinosa, urticarialike follicular mucinosis, and cutaneous lymphoma related. Feb 14, 2017 alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Cutaneous focal mucinosis has been rarely reported in association with follicular induction of the epidermis.
Follicle center lymphoma with follicular mucinosis jama network. The present paper reports a study on a case of follicular mucinosis exacerbated by sunlight exposure. Apr 19, 2019 primary follicular mucinosis in childhood parul verma 1, asha kubba 2, aditi jha 3 1 department of dermatology, venereology and leprology, king george medical university, lucknow, uttar pradesh, india 2 department of pathology, kubba skin clinic, new delhi, india 3 department of dermatology and std, vmmc and safdarjung hospital, new delhi, india. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in the walls of hair follicles. At least 2 distinct diagnostic entities have been proposed. Several other forms of mucinosis are described and classified. The patient had treatment failure with clobetasol 0.
These lch cells also showed strong nuclear positivity using an. Follicular mucinosis is a histological reaction pattern wherein variable amounts of epidermally derived mucin accumulate within follicular infundibula and the outer root sheath epithelium. Results in our study, major causes of cicatricial alopecia were lichen planopilaris 27. Follicular mucinosis responding to isotretinoin treatment introductionfollicular mucinosis fm, first described in the 1930s as a follicular skin disease with intraand perifollicular deposits of mucin, is a chronic type of inflammatory dermatosis involving the sebaceous glands and. It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis fungoides or reticulosis. Follicular mucinosis is a rare disorder affecting hairbearing skin, most commonly on the scalp, head and neck. All involve accumulation in the skin of abnormal amounts of mucin. No uniformly effective therapy exists for follicular mucinosis, alth.
May 16, 2020 follicular mucinosis is a term that encompasses three related entities. Report of a case with diffuse alopecia and normalappearing scalp skin. Also called follicular mucinosis edematous and erythematous plaques of alopecia on head and neck children. Idiopathic follicular mucinosis or mycosis fungoides. Study follicular mycosis fungoides, a distinct disease. Mucinosis follicularis is an affection of the pilosebaceous apparatus, consisting of degenerative changes in the follicular epithelium and of mucin accumulation in the hair follicle and the sebaceous gland, often accompanied by loss of hair. Kim r, winkelmann rk 1962 follicular mucinosis alopecia mucinosa. I read with great interest the case report, titled cutaneous focal mucinosis causing follicular induction of the epidermis by tatsas et al, 1 which was published.
Follicular mucinosis lewars m, levin j, purcell s indian dermatol. Follicular mucinosis is a primary idiopathic disease or a secondary, lymphomaassociated dermatosis. Mucin is capable of absorbing times its own weight in water. Follicular mucinosis article about follicular mucinosis. The study of mucinosis, follicular has been mentioned in research publications which can be found using our bioinformatics tool below. Follicular mucinosis definition of follicular mucinosis. Follicular mucinosis fm is defined as the accumulation of a mucinous material within the hair follicle. A secondary disorder associated with benign or malignant skin disease. Follicular mucinosis responding to isotretinoin treatment introductionfollicular mucinosis fm, first described in the 1930s as a follicular skin disease with intraand perifollicular deposits of mucin, is a chronic type of inflammatory dermatosis involving the sebaceous glands and outer root sheaths of hair follicles.
Mucinosis definition of mucinosis by medical dictionary. A case of idiopathic follicular mucinosis treated with bexarotene gel. Follicular mucinosis definition of follicular mucinosis by. Article information, pdf download for how to discern folliculotropic mycosis. How to discern folliculotropic mycosis fungoides from follicular. This process may occur as a primary disorder or it may be associated with benign e. A case of idiopathic follicular mucinosis treated with. Fm is generally divided into a primary benign idiopathic form and a secondary form usually occurring in association with cutaneous lymphomas especially mycosis fungoides, among other.
Primary follicular mucinosis in childhood verma p, kubba a. Aug 28, 2019 follicular mucinosis is a term that encompasses three related entities. A 52yearold man with no notable medical history except diabetes type 2 presented with a lump on the abdominal skin 8 years ago. Muscardin lm et al 2003 acneiform follicular mucinosis of the head and neck region. Alopecia mucinosa is a disease of unknown etiology, characterized clinically by plaques of folliculopapules and histologically by mucinosis of the pilosebaceous unit. Cutaneous focal mucinosis causing follicular induction of. Follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. A primary and chronic disorder occurring in people older than 40 years. Contextbeginning in 1957, patients have been described with localized alopecia characterized histopathologically by mucin deposition within hair follicles foll. Cases with clinical followup and more than one biopsy, lymphocytic exocytosis in the follicular epithelium accompanied by follicular mucinosis, or clear folliculotropic lymphoid. Follicular mucinosis successfully treated with isotretinoin.
The mucinoses are a diverse group of disorders which have in common the deposition of basophilic, finely granular and stringy material mucin in the connective tissues of the dermis dermal mucinoses, 1,2 in the pilosebaceous follicles follicular mucinoses, or in the epidermis and tumors derived therefrom epithelial mucinoses. Jan 04, 2019 follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. This paper reports the case of a 60yearold man with a dermatosis of 4 months, with the appearance of follicular papules on the. The dermatologic eruptions consist of follicular papules andor indurated plaques that demonstrate distinct histologic changes in the hair follicles that lead to hair loss. This is a jellylike complex carbohydrate substance, called hyaluronic acid, that occurs normally as part of the connective tissue in the dermis or midlayer of the skin. Follicular mucinosis is a general term used to describe a reactive disease process characterized histopathologically by mucin deposition in hair follicles and sebaceous glands that are undergoing epithelial reticular degeneration, t.
Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the. Focal cutaneous mucinosis see the pathology of focal cutaneous mucinosis. Follicular mucinosis journal of the american academy of. Follicular mucinosis fm is an epithelial reaction pattern characterized by follicular mucin accumulation. Among 33 patients with the histologic diagnosis of follicular mucinosis alopecia mucinosa made at our institution between 1982 and 1989, 9 had mycosis fungoides diagnosed concomitantly. Follicular mucinosis altmeyers encyclopedia department. A case of cutaneous focal mucinosis with follicular inductio. This article reports the first case of bexarotene gel to successfully treat persistent idiopathic follicular mucinosis. Kluk j et al 20 follicular mucinosis treated with topical 0. Follicular mucinosis is a dermatosis characterized by mucin depot in the skin, the follicular and sebaceous epithelium, which has been associated with lymphoproliferative disorders such as mycosis fungoides.
Although it is the sine qua non for alopecia mucinosa as a disease sui generis, it may occur in a variety of unrelated conditions, which may be inflammatory, hamartomatous, hyperplastic, or neoplastic. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk. Follicular mucinosis as a histological finding by itself, does not denote a specific clinicopathological entity and has been described as an incidental finding in several unrelated conditions like angiolymphoid hyperplasia, 56 familial reticuloendotheliosis, 7 spongiotic dermatitis, 1 and in diffuse papular and eczematous eruptions. An effective standard therapy for this disease is unknown. We present a case of a 14 yearold woman with a 10 month history of primary follicular mucinosis with erythematous plaques infiltrated on her cheek and forehead. The disease occurs either as a primary idiopathic form or as a secondary symptomatic lesion in association with systemic disease such as mycosis fungoides or other lymphoma. Follicular mucinosis article about follicular mucinosis by. Fm is generally divided into a primary benign idiopathic form and a secondary form usually occurring in association with cutaneous lymphomas especially mycosis fungoides, among other entities. The cutaneous mucinoses are a heterogeneous group of uncommon conditions whose main characteristic is abnormal mucin deposition in the skin, which may be localised or widespread. Mucinoses are a diverse group of uncommon skin disorders. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis characterized by mucin accumulation predominantly in the pilosebaceous. The presence of single or multiple welldelineated erythematous plaques with. In our case, isotretinoin led to a dramatic improvement of the skin lesions in about two weeks. Pdf follicular mucinosis responding to isotretinoin.
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