Rearrange individual pages or entire files in the desired order. Secondary follicular mucinosis associated with systemic lupus erythematosus kathryn oreilly md phd, jeremy brauer md, aaron lloyd md, rishi patel md, ruth walters md, andrew g franks jr md dermatology online journal 16 11. Seven dogs had one or more asymptomatic nodules, papules, or plaques on the skin or oral. Mucinosa definition of mucinosa by medical dictionary.
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 induction or. Focal cutaneous mucinosis see the pathology of focal cutaneous mucinosis. We sought to describe clinical and histopathologic findings of children with fm with and without mf, as well as. Although kreibich1 had reported a single case in 1926 and lehner and szodoray2. Follicular mucinosis is a chronic inflammatory disease of unknown etiology, the histopathological picture shows the presence of mucin in follicular epithelium and sebaceous glands.
Alopecia mucinosa an overview sciencedirect topics. Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemia rashid rashid md phd, sharon hymes md dermatology online journal 15 5. Alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Secondary follicular mucinosis associated with systemic lupus. Pathology of follicular mucinosis alopecia mucinosa 7. Pdf follicular mucinosis on the forehead of a 15yearold girl. Mucinosis follicular occurs at any age, but more often in the age of 20 to 50 years. It is characterised by bald patches of skin in which hair follicles are prominent. Mar 19, 2012 papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. Updated by dr ebtisam elghblawi, dermatologist, tripoli, libya. Follicular mycosis fungoides, a distinct disease entity with. 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. Recently, i had the chance to observe a similar case. Cases with clinical followup and more than one biopsy, lymphocytic exocytosis in the follicular epithelium accompanied by follicular mucinosis, or clear folliculotropic lymphoid.
Altman division of comparative pathology, department of pathology, school of medicine, university of miami, miami, fl abstract. Abstract primary follicular mucinosis is a mucinosis folicular dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Young age of the patient, a solitary lesion on the face or neck, and the absence of atypical epitheliotropic lymphocytes favor benign follicular mucinosis. It is caused by mucinous infiltration of tissues, and usually involving the scalp, face, and neck. It is a very rare condition that is observed in middleaged men. A disease of the pilosebaceous unit, presenting clinically as grouped follicular papules or plaques with associated hair loss. On the other hand, the frequency of fmu in patients with cutaneous lymphoma has not been appreciated in the literature. Includes pretibial myxedema of hyperthyroidism, generalized myxedema of hypothyroidism, papillary mucinosis lichen myxedematosus, myxoid cyst, cutaneous focal mucinosis, follicular mucinosis and carneys syndrome related myxoid nodules. Follicular mucinosis presenting as an acneiform eruption. The differentiation between pinkus follicular mucinosis and mycosis fungoidesassociated follicular mucinosis can be very difficult. Alopecia mucinosa, also known as follicular mucinosis, describes the appearance of mucin around hair follicles as seen under the microscope. Alopecia mucinosa 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. As i know, cutaneous focal mucinosis cfm is a very rare reason of follicular induction of the epidermis. Papular mucinosis genetic and rare diseases information.
Data from 44 patients with fm were retrieved from the files of the department of dermatology of the university of graz, in austria. 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. Molecular genetic analysis confirmed the oligomonoclonal nature of the tcell infiltrate. 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. Papular mucinosis also known as scleromyxedema, generalized lichen myxedematosus, and sclerodermoid lichen myxedematosus is a rare skin disease. Follicular mucinosis is one of the three types of cutaneous mucinoses degos 1959, the two other forms being papular mucinosis and pretibial myxedema scleromyxedema. Information and translations of mucinosis, follicular in the most comprehensive dictionary definitions resource on the web. No annoying ads, no download limits, enjoy it and dont forget to bookmark and share the love.
Unlike most nonhodgkin lymphomas which are generally b cell related, ctcl is caused by a mutation of t cells. 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. Purely follicular mycosis fungoides without mucinosis. Frequently, all three forms are regarded as papular mucinosis. Follicular mucinosis fm and folliculotropic mycosis fungoides mf are rare in children, and data regarding long. Follicular mucinosis definition of follicular mucinosis. Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular forms with sclerosis are called scleromyxedema. It came back as follicular mucinosis which is considered rare but i am starting to wonder. Alopecia mucinosa is a clinicopathological entity which presents with several erythematous papules and plaques mainly over the head and face regions with associated loss of hair and histopathological findings of follicular mucinosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Follicular mucinosis risks, symptoms and leading causes. Follicular mucinoses mucin accumulates in the epithelial hair follicle sheaths and sebaceous glands. The pathologic changes consisted of fibroblastic proliferation in the corium, an increased production of. Idiopathic follicular mucinosis or mycosis fungoides.
It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis fungoides or reticulosis. There are 3 conditions that belong to this group and they include alopecia mucinosa, cutaneous lymphoma related follicular mucinosis, and urticarialike follicular mucinosis. Feb 14, 2017 alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Lichen myxoedematosus or papular mucinosis is a rare skin disorder characterised by mucin deposits in the skin. Mar 10, 2018 the topic follicular mucinosis you are seeking is a synonym, or alternative name, or is closely related to the medical condition alopecia mucinosa. There exists no treatment of choice for follicular mucinosis fm. However, this brief report highlights the novel association of folliculitis, follicular mucinosis, and papular mucinosis in association with chronic myelomonocytic leukemia. In both patients, histology showed a folliculotropic infiltrate of atypical lymphocytes that spared the epidermis, in the absence of follicular mucinosis. Histologically, the disorder is characterized by mucin deposition in the follicular epithelium. These methods, among others, have mostly identified monoclonality as a defining characteristic of lafm. The histogenesis of cutaneous local mucinosis cfm is controversial. Follicular mucinosis definition of follicular mucinosis by. What links here related changes upload file special pages permanent. Primary paediatric cutaneous mucinoses rongioletti 2020.
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. Pdf follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal. Folliculitis, follicular mucinosis, and papular mucinosis. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in the walls of hair follicles. The localised form presents with firm, waxy papules with localised distribution and without systemic features. Cutaneous focal mucinosis cfm was originally described as an asymptomatic solitary lesion characterized by abundant focal cutaneous mucin deposits. 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. Cutaneous focal mucinosis a clinicopathological and histochemical study waine c. Papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin.
Secondary follicular mucinosis associated with systemic. 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. Histologically, the disorder is characterized by mucin deposition in the follicular. Cutaneous t cell lymphoma ctcl is a class of nonhodgkin lymphoma, which is a type of cancer of the immune system. Pdf follicular mucinosis is a rare dermatosis characterized by accumulation of mucin in the follicular epithelium and sebaceous glands. Reticular erythematous mucinosis also is a type of cutaneous mucinosis but with a classic clinical appear ance of a reticulated erythematous plaque on the chest or back, making it clinically distinct from papular mucinosis scleromyxedema and the presentation described in the current patient. Follicular mucinosis has, clinically, keratotic follicular papules specially distributed in the scalp and face, affecting the. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. Follicular mucinosis is a rare disorder affecting hairbearing skin, most commonly on the scalp, head and neck. Mucinosis definition of mucinosis by medical dictionary. Follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. Follicular mucinosis fm, also known as alopecia mucinosa, is a rare inflammatory disorder that characteristically presents as follicular papules or infiltrated plaques with prominent follicular. Apr 11, 2020 there exists no treatment of choice for follicular mucinosis fm. Follicular mucinosis alopecia mucinosa follicular mucinosis alopecia mucinosa is an uncommon inflammatory dermatosis with a predilection for adults in the third and fourth decades of life.
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. As of today we have 78,789,783 ebooks for you to download for free. Cutaneous focal mucinosis causing follicular induction of the epidermis. 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.
It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. This is a hair loss condition that can occur at any age. Urticarialike follicular mucinosis ulfm is a type of alopecia mucinosa with urticarialike symptoms. Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.
A case of cutaneous focal mucinosis with follicular inductio. Follicular mucinosis is a histologic term for a reaction pattern in follicular epithelium. Follicular mucinosis fm is a rare disorder of the skin characterized by follicular degeneration due to the accumulation of mucin within the. Follicular mucinosis and follicular mycosis fungoides. The most common of these rare conditions is alopecia mucinosa sometimes referred to as pinkus follicular mucinosis.
In fact, published reports on follicular mf are relatively scarce, generally concern case reports or small series of patients, and have mainly been focused on differentiation between mfassociated follicular mucinosis and alopecia mucinosa, the benign idiopathic form of follicular mucinosis. The face, neck, and scalp are the most frequently affected sites, although lesions may occur on any site of the body. Mucins look like stringy clear or whitish goo mainly composed of hyaluronic acid. However, multiple focal mucinous lesions associated with systemic diseases have also been designated as cfm. 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. Alopecia mucinosa is a disease of unknown etiology, characterized clinically by plaques of folliculopapules and histologically by mucinosis of the pilosebaceous unit. Historically two distinct entities of fm have been proposed. Definition of mucinosis, follicular in the dictionary.
Fm of children and young adults not associated with other diseases. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in. The purpose of this paper is to record our clinical, microscopic, and histochemical ob servations on 14 patients with focal mucino sis and to compare and discuss the relationship of focal mucinosis to the other cutaneous mucinoses. In recent years, the distinction between idiopathic follicular mucinosis fm and lymphomaassociated follicular mucinosis lafm has been made through assessment of tcell receptor gene rearrangement, flow cytometry, and immunohistochemistry. A literature search produced one case of follicular mucniosis as the presentingassociated sign of acute myeloblastic leukemia. Pinkus primary follicular mucinosis alopecia mucinosafollicular mucinosis. Folliculitis, follicular mucinosis, and papular mucinosis as. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the. Perifollicular mucinosis is a cutaneous condition characterized by mucinosis, and described in hivinfected patients.
Alopecia mucinosa is a rare inflammatory condition of hairbearing skin, resulting in a thickening of skin that is accompanied by hair loss. The dermatologic eruptions consist of follicular papules andor indurated plaques that demonstrate distinct histologic changes in the hair follicles that lead to hair loss. Cutaneous mucinoses encompass a variety of cutaneous disorders. Cutaneous mucinosis of infancy localized lichen myxoedematosus. Reticular erythematous mucinosis scleredema scleromyxedema papular mucinosis acral persistent papular mucinosis focal mucinosis digital mucous cyst mucocele cutaneous myxoma cutaneous mucinosis of infancy nevus mucinosis alopecia mucinosa follicular mucinosis mucopolysaccharidoses thursday, may 23.
Mucinous disorders of the skinbeyond the dermal mucinosis. Follicular mucinosis article about follicular mucinosis by. It is usually found in adults and rarely seen in children. Mucinosis, follicular is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings. Cutaneous focal mucinosis causing follicular induction of. Helwig, md, philadelphia a study of 14 patients with focal mucinosis revealed the usual clinical features\pm\asolitary, asymptomatic, fleshcolored to white papule or nodule.