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. Follicular mucinosis is a disease characterized by follicular degeneration and mucin accumulation. Pdf follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. Follicular mucinosis fm is a primary or secondary finding in fmf, lupus. Histopathology of 2 separate cases, both diagnosed as follicular mucinosis pending further followup. Captopril is an angiotensinconverting enzyme inhibitor which has been widely used as an antihypertensive agent and vasodilator. Demodexinduced follicular mucinosis of the head and neck. Cutaneous mucinosis in a strain of brownegg laying. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in the walls of hair follicles. Follicular mucinosis associated with mycosis fungoides lesley clarkloeser md, and joann latkowski md dermatology online journal 10 3. Immunohistochemical stains were noncontributory, and tcell gene rearrangement was positive only in the case of mycosis fungoides. Alopecia mucinosa also known as follicular mucinosis, mucinosis follicularis, pinkus follicular mucinosis, and pinkus follicular mucinosisbenign primary form is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp, neck and face. Follicular mucinosis colloidal iron alopecia mucinosa adults 3040s follicular papules or plaques with hair loss and follicle degeneration three types.
Follicular mucinosis is a histologic term for a reaction pattern in follicular epithelium. The terms papular mucinosis and lichen myxoedematosus are generally used interchangeably to describe the occurrence of this condition as a localized and less severe form, while the term scleromyxoedema refers to a generalized, more severe form. Follicular mucinosis secondary to captoprilinduced photoallergy abstract. Light and electron microscopy, direct immunofluorescence, and immunoperoxidase cell marker studies were undertaken to characterize the nature of the disease. Dec 14, 2018 follicular induction is a phenomenon whereby the epidermis is induced by an underlying process to form primitive or mature hair follicles, and is commonly seen overlying dermatofibromas. Alopecia mucinosa, often referred to as follicular mucinosis. Unlike most nonhodgkin lymphomas which are generally b cell related, ctcl is caused by a mutation of t cells.
It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis. A clinical and demographic study of single lesions in leprosy. Abstractfollicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis. It usually occurs in middle aged men on the head and neck.
Alopecia mucinosa is a disease of unknown etiology, characterized clinically by plaques of folliculopapules and histologically by mucinosis of the pilosebaceous unit. Histologically, the disorder is characterized by mucin deposition in the follicular. Follicular mycosis fungoides is a type of mycosis fungoides with different clinicopathological and prognostic features. Mucinosis article about mucinosis by the free dictionary. Follicular mucinosis fm is a cutaneous mucinosis in which mucinosis folicular is a buildup of mucin at the external root sheath and with much less frequency in the sebaceous gland. Follicular mucinosis is a primary idiopathic disease or a secondary, lymphoma associated dermatosis.
The dermatologic eruptions consist of follicular papules andor indurated plaques that demonstrate distinct histologic changes in the hair follicles that lead to hair loss. Follicular mucinosis definition of follicular mucinosis. Alopecia mucinosa, also known as follicular mucinosis, describes the appearance of mucin around hair follicles as seen under the microscope. Follicular mucinosis alopecia mucinosa jama dermatology. Mar 19, 2012 papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. Follicular mucinosis responding to isotretinoin treatment.
Follicular mucinosis alopecia mucinosa this mucinosis is most common in children and adult males in their thirties or forties. Perelman department of dermatology, new york university abstract. Answers from experts on causes of follicular mucinosis. We report two cases of women in their twenties who presented with an acneiform rash on the face, arms and back. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. Dec 24, 2018 there are 3 conditions that belong to this group and they include alopecia mucinosa, cutaneous lymphoma related follicular mucinosis, and urticarialike follicular mucinosis. An effective standard therapy for the benign group is. Follicular mucinosis fm, also known as alopecia mucinosa, is a rare inflammatory disorder that characteristically presents as follicular. Follicular mucinosis presenting as an acneiform eruption. It is believed that follicular keratinocytes are responsible for mucin production, as a response to the stimulus of cytokines released by perifollicular t lymphocytes. Lymphoid infiltration was composed of smallmedium sized cells, with scattered hyperchromatic nuclei in six cases. How to discern folliculotropic mycosis fungoides from follicular. Follicular mucinosis is an epithelial reaction pattern characterized by the accumulation of mucin within hair follicles.
Follicular mucinosis alopecia mucinosa is an uncommon inflammatory dermatosis with a predilection for adults in the third and fourth decades of life. It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis fungoides or reticulosis. Successful treatment of primary progressive follicular mucinosis with. Possible druginduced follicular mucinosis in a dog.
The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in. The vast majority of thymic carcinomas of all sorts just happen, without any identifiable risk factors. Pdf follicular mucinosis responding to isotretinoin. Follicular mucinosis definition of follicular mucinosis by. Jan 04, 2019 mucinosis folicular pdf posted on january 4, 2019 by admin follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. Follicular mucinosis fm is a cutaneous disorder arising from the pilosebaceous unit that is most strongly associated with folliculotropic mycosis fungoides fmf, an aggressive form of mycosis fungoides mf. Follicular mucinosis is a rare disorder affecting hairbearing skin, most commonly on the scalp, head and neck. It is characterised by bald patches of skin in which hair follicles are prominent. Alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957.
Papular mucinosis genetic and rare diseases information. Skin coloured or erythematous plaques favouring scalp, face, neck and shoulders. Results in our study, major causes of cicatricial alopecia were lichen planopilaris 27. Urticarialike follicular mucinosis ulfm is a type of alopecia mucinosa with urticarialike symptoms. Follicular mucinosis fm is an uncommon inflammatory disorder rarely described in dogs, all old retrievers and norwegian puffin dogs npds. Article information, pdf download for how to discern folliculotropic. Follicular mucinosis fm is a rare disorder of the skin characterized by follicular degeneration due to the accumulation of mucin within the. Follicular mucinosis on the forehead of a 15yearold girl. On the other hand, follicular mucinosis has been described in association with numerous processes, mainly. When follicular mucinosis fm is defined as an epithelial reaction pattern characterized by intrafollicular and perifollicular mucin accumulation, it cannot be considered a distinct disease entity, as this pattern is ubiquitously present in various inflammatory and neoplastic skin conditions. A benign idiopathic form, occurring as a spontaneously remitting process, which presents in children and young adults primary follicular mucinosis pfm and a secondary form, occurring. Follicular induction is a phenomenon whereby the epidermis is induced by an underlying process to form primitive or mature hair follicles, and is commonly seen overlying dermatofibromas.
Alopecia mucinosa also known as follicular mucinosis, mucinosis follicularis, pinkus follicular mucinosis, and pinkus follicular mucinosis benign primary form is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp, neck and face 649. Idiopathic follicular mucinosis or mycosis fungoides. It is a very rare condition that is observed in middleaged men. The cancerous t cells in the body initially migrate to the skin, causing various lesions to appear. Alopecia mucinosa an overview sciencedirect topics. Feb 14, 2014 follicular mucinosis is a disorder histopathologically defined by the accumulation of mucin in hair follicles and sebaceous glands, which undergo epithelial reticular degeneration.
Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. These lesions change shape as the disease progresses, typically. Primary mucinoses can be divided into dermal and follicular mucinoses dermal mucinoses include lichen myxoedematosus, reticular erythematous mucinosis, scleredema, mucinoses in thyroid disease, papular and nodular mucinosis in connective tissue disease, self. Also called follicular mucinosis edematous and erythematous plaques of alopecia on head and neck children. Acercamiento clinicopatologico a las mucinosis cutaneas primarias laura carreno t1, claudia morales h1, lybeny rivera l. It can be seen in mycosis fungoides, although idiopathic or forms associated with other diseases are also known. Mucinosis definition of mucinosis by medical dictionary. Cutaneous t cell lymphoma ctcl is a class of nonhodgkin lymphoma, which is a type of cancer of the immune system. Demodicidosis is a cutaneous infection caused by demodex, an ectoparasitic mite that permanently resides in or near the pilosebaceous unit of mammalian hair follicles. Mucinosis folicular secundaria a fotoalergia por captopril.
Follicular induction has been rarely described in cfm. Follicular mucinosis is a disorder histopathologically defined by the accumulation of mucin in hair follicles and sebaceous glands, which undergo epithelial reticular degeneration. The term follicu lar mucinosis was proposed by jablonska. Genetic mutation is a lottery over the lifetime, and some folks are lucky enough to escape all cancers through old age, but others may end up with an unusual one such as thymic carcinoma. Pdf follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized. The presence of single or multiple welldelineated erythematous plaques with.
Although adverse skin effects occur in 10 % of patients. Multiple lesions may indicate the carney complex in which the benign tumours may develop in the. Feb 14, 2017 alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. 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. A variety of treatments have been tried with some success but most lesions resolve within months to two years. 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. Updated by dr ebtisam elghblawi, dermatologist, tripoli, libya. Although kreibich 1 had reported a single case in 1926 and lehner and szodoray 2 did so in 1939, pinkus 3 in 1957 was first to regard the condition as an entity and propose its name. The face, neck, and scalp are the most frequently affected sites, although lesions may occur on any site of the body. Follicular mucinosis and follicular mycosis fungoides.
Follicular mucinosis fm can present as an acneiform eruption, and is usually a benign variant of primary fm unrelated to cutaneous t. Follicular mucinosis case report article pdf available. Follicular mucinosis 67, and dermal lymphoid infiltration showing minimalintensive folliculotropism accompanied by eosinophils was seen. Follicular mucinosis is an epithelial mucinosis, in which mucin infiltrates the outer root sheath of the follicles and sebaceous glands. 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 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 is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. Follicular mucinosis may be classified into 3 main clinical variants. Follicular mucinosis is a cutaneous disorder distinguished by the buildup of mucin in the sebaceous glands and the pilosebaceous follicles. Follicular mucinosis is one of the three types of cutaneous mucinoses degos 1959, the two other forms being papular mucinosis and pretibial myxedema scleromyxedema. The adult form tends to have more skin lesions and be last longer than the form typically found in children. A 62yearold man with a year history of mycosis fungoides presented with a 2month history of alopecia of the scalp.