Localized cutaneous mucinosis associated with multiple. To determine i whether primary idiopathic follicular mucinosis pfm and lymphoma. Follicular mucinosis is also known as alopecia mucinosa or mucinosis follicularis. Acd az of skin follicular mucinosis australasian college of. It has been proposed by many authors that follicular mucinosis is directly associated with mycosis fungoides mf. Follicular mucinosis is an uncommon inflammatory disorder that characteristically presents as follicular papules andor indurated plaques. N2 follicular mucinosis represents a term for a histopathologic reaction pattern in follicular epithelium. 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. With such associations now in the literature, it is important to remain highly vigilant when managing such seemingly benign and nonspecific skin changes. Mucin deposition is a common histological finding in patients with cle, but it is unusual for the mucin to be present in such a quantity to produce detectable skin lesions.
Alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. 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. Papular mucinosis genetic and rare diseases information. Skin coloured or erythematous plaques favouring scalp, face, neck and shoulders.
Follicular involvement in cutaneous tcell lymphoma commonly presents clinically with alopecia, follicular cysts, or comedolike lesions usually associated with follicular mucinosis and strong. The vast majority of thymic carcinomas of all sorts just happen, without any identifiable risk factors. Alopecia mucinosa, also known as follicular mucinosis, describes the appearance of mucin around hair follicles as seen under the microscope. Follicular mucinosis associated with mycosis fungoides. Answers from experts on causes of follicular mucinosis. The face, neck, and scalp are the most frequently affected sites, although lesions may occur on any site of the body. Feb 14, 2017 alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Folliculitis, follicular mucinosis, and papular mucinosis as. Top 21 on causes of follicular mucinosis healthtap healthtap. It can occur as a primary idiopathic benign disorder. Aug 01, 2011 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 may be classified into 3 main clinical variants. Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the. Follicular mucinosis is characterized by mucin deposits within the hair follicles and sebaceous glands. 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. The topic primary chronic pinkus mucinosis follicularis you are seeking is a synonym, or alternative name, or is closely related to the medical condition primary chronic alopecia mucinosa. Follicular mucinosis has, clinically, keratotic follicular papules specially distributed in the scalp and face, affecting the neck, trunk and extremities. It is still unclear whether follicular mucinosis is a transitional state evolving into mycosis fungoides in. E, diffuse erythema and alopecia of the eyebrows and eyelashes, not shown. Multiple lesions may indicate the carney complex in which the benign tumours may develop in the. Follicular mucinosis is a cutaneous disorder distinguished by the buildup of mucin in the sebaceous glands and the pilosebaceous follicles. Follicular mucinosis associated with mycosis fungoides lesley clarkloeser md, and joann latkowski md dermatology online journal 10 3.
Follicular mucinosis fmu is a relatively rare dermatosis, belonging to the cutaneous mucinoses group, that is, a complex of morphological and various etiopathogenic clinical entities, whose common denominator is represented by localized or diffused deposits of mucin in the skin or hair follicles. 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 presenting as an acneiform eruption. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. Papular mucinosis abnormal paraprotein in 90% of cases, usually of the igg type plasmacell dyscrasia of ten present skin biopsy shows a horizontal band of mucinous material between collagen bundles in the upper dermis increase in fibroblasts and dermal fibrosis glycosaminoglycan positive for alcian blue at ph 2. 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. Jul 17, 20 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. Alopecia mucinosa follicular mucinosis in a dog bell 1995.
Results in our study, major causes of cicatricial alopecia were lichen planopilaris 27. 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 is characterized clinically by grouped erythematous papules andor plaques that may be markedly indurated or nodular and histologically by mucin accumulation in hair follicles. Follicular mucinosis developing in a patient with folliculitis decalvans. Pathology of follicular mucinosis alopecia mucinosa 7. Inflammatory diseases of hair follicles, sweat glands, and. Histologically, the disorder is characterized by mucin deposition in the follicular. The presence of single or multiple welldelineated erythematous plaques with. Follicular mucinosis is associated with lymphoma, particularly mycosis fungoides, in 1530% of cases. Follicular mucinosis is a rare disorder affecting hairbearing skin, most commonly on the scalp, head and neck. A clinical and demographic study of single lesions in leprosy. A disease of the pilosebaceous unit, presenting clinically as grouped follicular papules or plaques with associated hair loss. Mucinosis article about mucinosis by the free dictionary. With such associations now in the literature, it is important to remain highly vigilant when managing such.
Papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. Follicular mucinosis is an epithelial reaction pattern characterized by the accumulation of mucin within hair follicles. Follicular mucinosis and follicular mycosis fungoides. Follicular mucinosis fm is a rare disorder of the skin characterized by follicular degeneration due to the accumulation of mucin within the. Follicular mucinosis, mycosis fungoides, and acute myeloid leukemia. Cutaneous mucinosis, characterized by deposition of mucin glycosaminoglycans in skin, may be primary or secondary to collagen vascular diseases, mesenchymal and neural tumors, eosinophilia myalgia syndrome, histiocytosis, etc. It is caused by mucinous infiltration of tissues, and usually involving the scalp, face, and neck. The cancerous t cells in the body initially migrate to the skin, causing various lesions to appear. Updated by dr ebtisam elghblawi, dermatologist, tripoli, libya. Unlike most nonhodgkin lymphomas which are generally b cell related, ctcl is caused by a mutation of t cells. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Focal cutaneous mucinosis see the pathology of focal cutaneous mucinosis. C, infiltrated plaque with alopecia and cystic lesions above the left eye.
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. Clinical appearances of follicular mycosis fungoides. Follicular mycosis fungoides, a distinct disease entity with. Cutaneous t cell lymphoma ctcl is a class of nonhodgkin lymphoma, which is a type of cancer of the immune system. Hair follicle expanded by intercellular mucin confirm with mucin stainsmucicarmine, colloidal iron, or alcian bluepas rule out alopecia mucinosa rule out concomitant. Alopecia mucinosa is a rare disorder characterized by mucin deposition mucinosis in pilosebaceous follicles and with much less frequency in sebaceous glands. Follicular mucinosis called alopecia mucinosa by pinkus 3 involves accumulation of an abnormal amount of mucin. Mucinosis definition of mucinosis by medical dictionary. Follicular mucinosis, mycosis fungoides, and acute myeloid. Idiopathic follicular mucinosis or mycosis fungoides. Papulonodular mucinosis pnm is a form of cutaneous mucinosis characterized by aberrant accumulation of glycosaminoglycans between collagen bundles. Last, we propose a concept, and a terminology that derives from it, that synthesizes all that is known now about alopecia mucinosa and follicular mucinosis, in particular the relationship of alopecia mucinosa to mycosis fungoides, including follicular, syringotropic, and erythrodermic manifestations of it.
It may be primary idiopathic, occurring most often in children, or it may be secondary to mycosis. Follicular mucinosis article about follicular mucinosis by. Acercamiento clinicopatologico a las mucinosis cutaneas primarias laura carreno t1, claudia morales h1, lybeny rivera l. Mar 19, 2012 papular mucinosis is a rare skin disorder characterized by deposits of mucin in the skin. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in. However, this brief report highlights the novel association of folliculitis, follicular mucinosis, and papular mucinosis in association with chronic myelomonocytic leukemia. 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 definition of follicular mucinosis by. 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. 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. B, concurrent grouped follicular papules on the trunk. The first page of the pdf of this article appears above.
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. The name of the condition comes from the accumulation of mucin jellylike, semiliquid material in the walls of hair follicles. A 62yearold man with a year history of mycosis fungoides presented with a 2month history of alopecia of the scalp. 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. D, the same patient had numerous cysts and comedolike lesions on the trunk. 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. Perelman department of dermatology, new york university abstract. It is characterised by bald patches of skin in which hair follicles are prominent. Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. Follicular mucinosis is one of the three types of cutaneous mucinoses degos 1959, the two other forms being papular mucinosis and pretibial myxedema scleromyxedema. This is a hair loss condition that can occur at any age.