Secondary alopecia mucinosa, which many now consider to be mycosis fungoides. Mycosis fungoides mf is by far the most frequent pcl 5. The disease is typically slowly progressive and chronic. It is considered a benign condition, occurs mainly in children and young adults and presents with several reddish papules or. Alopecia mucinosa, follicular mucinosis, follicular mycosis fungoides introduction mycosis fungoides mf is a cutaneous tcell lymphoma that is thought to be uncommon in india. Follicular mucinosis alopecia mucinosa dermatology advisor. Alopecia mucinosa an overview sciencedirect topics. The second is the form seen in association with lymphoma, usually mycosis fungoides. Mycosis fungoides pictures, staging, symptoms, treatment. Folliculotropic mycosis fungoides this is due to a cutaneous tcell lymphoma, and is characterised by a more generalised chronic form in a slightly older age group, with larger and more numerous plaques on the scalp, face and also the extremities. Amin et al reported a 33yearold female with a 7year history of cd8positive hypopigmented mycosis fungoides mf involving the trunk and extremities who presented with a large welldefined alopecic patch on her frontal scalp, resembling alopecia areata aa without hypopigmentation or erythema. The topic syringotropic mycosis fungoides with alopecia you are seeking is a synonym, or alternative name, or is closely related to the medical condition syringotropic mycosis fungoides. Request pdf rapidly progressing mycosis fungoides presenting as follicular mucinosis follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or.
Mycosis fungoides which is also referred to as alibertbazin syndrome is a rare skin cancer. First described in 1957, the term alopecia mucinosa referred to a characteristic disease process of follicular degeneration that lead to alopecia. Th is term became follicular mucinosis when it was shown that follicular mucinous degeneration was not. Attending the masquerade ball with ctcl and alopecia areata. The patch stage of mycosis fungoides a criteria for histologic. Follicular mycosis fungoides a report of four indian cases ncbi. Stage 4a is described as mycosis fungoides with tumors that can cover any amount of the skin surface. Cutaneous tcell lymphomas occur when certain white blood cells, called t cells, become cancerous.
Alopecia can be a manifestation of mycosis fungoides mf and sezary. In patch stage mycosis fungoides, the skin lesions are flat. D, the same patient had numerous cysts and comedolike lesions on the trunk. Stage 4 mycosis fungoides is divided into stage 4a and stage 4b. The author proposes possible explanations regarding the relationship between follicular mucinosis and mycosis fungoides. What is the clinical history of folliculotropic mycosis. Three cases with single lesion of alopecia mucinosa follicular mucinosis were. Feb 14, 2017 mortality is related to the coexistence of mycosis fungoides in secondary alopecia mucinosa. Pdf follicular mycosis fungoides a report of four indian.
Mycosis fungoides pictures, staging, symptoms, causes. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. Clinical spectrum of hair loss in patients with mycosis fungoides and sezary syndrome ss. A 62yearold man with a year history of mycosis fungoides presented with a 2month history of alopecia of the scalp. Although the terms mf and ctcl are often used interchangeably, this can be a source of confusion. Three cases with single lesion of alopecia mucinosa follicular mucinosis were treated with antileprosy treatment and showed rapid and complete resolution of the lesions with no recurrence on extended followup.
The most relevant feature, with and without associated follicular mucinosis, is the deep follicular and perifollicular localization of the neoplastic infiltrates, which makes them. Mycosis fungoides follows a slow, chronic indolent course and very often does not spread beyond the skin. We sought to describe the clinicopathologic presentation and molecular features of alopecia in patients with mfss. Alopecia mucinosa, often referred to as follicular mucinosis, was first reported by pinkus in 1957. Diagnosis of mycosis fungoides and sezary syndrome was made according to criteria. Haber h follicular mucinosis alopecia mucinosa pinkus.
It was so named because alibert described the skin tumors of a severe case as having a mushroomlike appearance. This stage can either have cancer that has affected the lymph nodes while the blood may have the presence of cancerous lymphocytes or the stage can also be described as having cancerous lymphocytes in the blood with enlarged. Two children, a boy aged 14 years and a girl aged 12 years presented themselves, each, with a single hypopigmented, hypoesthetic patch on the face. Follicular mucinosis and follicular mycosis fungoides. In patients with mycosis fungoides the sign seems to be more frequent. Mycosis fungoides is a rare form of tcell lymphoma of the skin. Ctcl is a type of malignancy wherein the special kind of white blood cells called tlymphocytes become infectious and drawn or deposited to the skin. In examining 40 children with a clinical diagnosis of alopecia mucinosa follicular mucinosiswas diagnosed 6 months after diagnosis of alopecia mucinosa follicular mucinosis in one patient. Mycosis fungoides is rarely cured, but some people stay in remission for a long time. Alopecia mucinosa of the face associated with mycosis fungoides. The dermatologic eruptions consist of follicular papules andor indurated plaques that demonstrate distinct histologic changes in the hair follicles that lead to hair loss.
Alopecia mucinosa has been divided into the primary or idiopathic variety and the secondary type associated with cutaneous t cell lymphoma. To reflect the fact that the disease does not always present with or result in alopecia, the entity was renamed follicular mucinosa. The name mycosis fungoides is very misleadingit loosely means mushroomlike fungal disease. This condition is the most common variety of cutaneous t cell lymphoma ctcl. The disease, however, is not a fungal infection but rather a type of nonhodgkins lymphoma.
Secondary alopecia mucinosa, which many now consider to be mycosis fungoides, presents in older individuals, with or without other features of mycosis fungoides. Mycosis fungoides mf is the most common form of cutaneous tcell lymphoma ctcl. However, the benign form of alopecia mucinosa, which is not associated with mycosis fungoides, must be distinguished from mycosis fungoides associated with mucinosis. Follicular mucinosis associated with mycosis fungoides lesley clarkloeser md, and joann latkowski md dermatology online journal 10 3. Mycosis fungoides symptoms, diagnosis, treatments and causes. 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. Idiopathic follicular mucinosis or mycosis fungoides. It is characterised by bald patches of skin in which hair follicles are prominent.
Mf mainly affects the elderly, but rare cases in children and young adults have been reported 2. In early stages, its often treated with medicines or therapies that target just your skin. Alopecia mucinosa, follicular mucinosis, follicular mycosis fungoides doi. E, patchy hair loss on scalp with erythematous patches and plaques present at areas of baldness, patient 28. The incidence of mycosis fungoides was seen to be increasing till the year 2000.
Alopecia mucinosa typically presents as hair loss within patches or indurated plaques of perifollicular papules. Rapidly progressing mycosis fungoides presenting as. Apr 20, 2020 however, the benign form of alopecia mucinosa, which is not associated with mycosis fungoides, must be distinguished from mycosis fungoides associated with mucinosis. Alopecia mucinosa typically presents as hair loss within patches or. An estimated 1540% of adults with alopecia mucinosa will eventually develop lymphoma, if they do not already have it. One or more lesions may be present from onset or a single lesion may develop to multiple lesions over a few weeks or months. A genetic predisposition may play a role in some cases and some specific genetic alterations have been recently identified 6,7. Follicular mucinosis mycosis fungoides turk patoloji dergisiturkish journal of pathology introduction pinkus fi rst defi ned alopecia mucinosa as a mucinous follicular degeneration that leads to alopecia in 1957 1. Alopecia mucinosa, inflammatory disease or mycosis fungoides. Secondary causes of alopecia mucinosa such as mycosis fungoides may not be. The spectrum of hair loss in patients with mycosis.
Mycosis fungoides mf is a clinical diagnosis that requires strong correlation with histopathologic and sometimes molecular findings to exclude benign inflammatory diseases, more aggressive primary cutaneous lymphomas, and extracutaneous lymphomas that can involve the skin. Folliculotropic mycosis fungoides is commonly first evident clinically with alopecia, follicular cysts, or comedolike lesions and is usually associated with follicular mucinosis and strong. Conventional mf begins as eczematoid or psoriasiform patches and. This third group was regarded by hempstead and ackerman 3 as belonging to the group of secondary follicular mucinoses because of the lack, at that time, of convincing examples of follicular mucinosis alopecia mucinosa progressing to mycosis fungoides or cutaneous lymphoma. Primary or idiopathic alopecia mucinosa was first described in 1957 by pinkus. Further, the who stated that the term mycosis fungoides should be exclusively reserved for classical cases typified by the evolution of cutaneous patches, plaques, and tumors, or for variants that show a similar clinical course. Mycosis fungoides nord national organization for rare.
Most often there are oval or ringshaped annular pink dry patches on covered skin. The name mycosis fungoides is historical and confusing. Alopecia can be a manifestation of mycosis fungoides mf and sezary syndrome ss, but the prevalence is unknown. Amin et al reported a 33yearold female with a 7year history of cd8positive hypopigmented mycosis fungoides mf involving the trunk and extremities who presented with a large welldefined alopecic patch on her frontal scalp, resembling aa without hypopigmentation or erythema. The authors noted that alopecia due to cutaneous lymphoma is an. Reviews of series of patients with alopecia mucinosa2,3 suggest that. Syringotropic mycosis fungoides is a rare variant of mycosis fungoides, which is a cutaneous tcell nonhodgkins lymphoma. It presents as grouped follicular papules within reddened, dry, patches or plaques. They may spontaneously disappear, remain the same size, or.
Perelman department of dermatology, new york university abstract. Mycosis fungoides is the most common form of a type of blood cancer called cutaneous tcell lymphoma. Follicular mycosis fungoides a report of four indian cases. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. Folliculotropic mycosis fungoides masquerading as alopecia areata. Warren heymann on ctcl and alopecia areata practiceupdate. The spectrum of hair loss in patients with mycosis fungoides and. All cases of mf are ctcl, but not all ctcl cases are mf. Follicular mucinosis associated with mycosis fungoides. Alopecia mucinosa, follicular mucinosis, follicular mycosis fungoides. Alopecia mucinosa most commonly affects face, neck and scalp, but any part of the body may be affected. Alopecia mucinosa of the face associated with mycosis. Clinical appearances of follicular mycosis fungoides. E, diffuse erythema and alopecia of the eyebrows and eyelashes, not shown.
Follicular mycosis fungoides, a distinct disease entity with. Cutaneous tcell lymphoma presenting as benign dermatoses. The spectrum of hair loss in patients with mycosis fungoides. Follicular mucinosis associated with mycosis fungoides comment first described in 1957, the term alopecia mucinosa referred to a characteristic disease process of follicular degeneration that lead to alopecia. More detailed information about the symptoms, causes, and treatments of mycosis fungoides is available below. B, concurrent grouped follicular papules on the trunk. Plaques are often composed of densely aggregated individual papules, many of which have a central spine. Alopecia can be a manifestation of mycosis fungoides mf and. Histopathology of 2 separate cases, both diagnosed as follicular mucinosis pending further followup. Follicular mucinosis, mycosis fungoides and other cutaneous tcell lymphomas are the most important differential diagnoses both clinically and histopathologically. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is. C, infiltrated plaque with alopecia and cystic lesions above the left eye. It may mimic many benign processes, such as eczema, psoriasis and contact dermatitis. Follicular mucinosis associated with mycosis fungoides comment.
A to d, patchy hair loss clinically identical to alopecia areata from patients 8, 7, 2, and 6, respectively. Alopecia mucinosa is classified into two categories. Alopecia mucinosa responding to antileprosy treatment. Patches or plaques are usually 25 cm in diameter but can be larger. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma, which is characterised in its early stages by epidermotropism of small to mediumsized t lymphocytes with cerebriform nuclei. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells.
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