Follicular mucinosis is a term that encompasses three related entities. Alopecia mucinosa, Urticaria-like follicular mucinosis, and cutaneous lymphoma related. On hair-bearing skin (e.g. scalp), overlying alopecia is notable, hence the term “ alopecia mucinosa” (see Figure 5). Plaques are often composed of densely. Alopecia mucinosa is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp.
|Published (Last):||27 November 2005|
|PDF File Size:||14.98 Mb|
|ePub File Size:||12.99 Mb|
|Price:||Free* [*Free Regsitration Required]|
When incidentally encountered microscopically, it may be simply viewed as a histologic reaction pattern. Lesions are often asymptomatic but may itch or burn; they develop over weeks or months not days.
FM generally presents as pink-red papules, patches, or plaques Figure 1Figure 2Figure 3Figure 4. On hair-bearing skin e. Plaques are often composed of densely aggregated individual papules, many of which have a central spine. The number of lesions ranges from one or two to several dozen; they are typically distributed on the face, scalp, or neck; the trunk and extremities are less often affected.
Mucosal surfaces, genitalia, and acral palms and soles areas are usually spared. Skin biopsies are required for diagnosis. The presence of intrafollicular mucin can be confirmed with colloidal iron tissue stains.
Perifollicular and perivascular lymphocytes are typically present in all types of FM. In any subtype of FM, lesional skin polymerase chain reaction PCR based molecular testing for T-cell clonality may be positive, thereby suggesting monoclonality of the infiltrating T lymphocytes. However, monoclonality does not distinguish lymphoma-associated FM from idiopathic FM. Analysis of lesional skin for T-cell clonality using PCR for T cell receptor[TCR] gene rearrangements should be considered in patients with known or suspected FM, but is not required for diagnosis.
Due to its rarity, epidemiologic studies are limited; no predisposing factors have been alopeciw, and no racial or gender predilection has been established. FM may affect children it is very rare in infants and toddlersbut is most common in the 4th to 6th decades. FM has been reported as an incidental finding in a variety of inflammatory skin disorders, such as acne, insect bite reactions, and lichen planus.
It has also been rarely alopecja in patients with neoplastic diseases, most commonly hematologic-based malignancies. The etiology is not known. Accumulation of mucin, which is composed of hyaluronate and sulfated glycosaminoglycans, in the follicle results in disruption of cellular attachments and destruction of the pilosebaceous subunit.
With loss of the follicle, mucinoss may be evident clinically. Controversy exists as to whether the disease is a zlopecia process or a reactive aloprcia. It may be due to dysfunctional T cells inducing mucin production by fibroblasts surrounding follicular epithelium, or due to excess production of mucin by follicular keratinocytes. akopecia
In general, patients with idiopathic FM tend to be younger and have fewer and more localized lesions on the head or neck. No reliable distinguishing clinical, histological, or molecular parameter exists. Patients with FM require a thorough review of systems, full skin and lymph node exam, and long-term follow up.
Given the association with CTCL, as well as much rarer associations with other hematologic malignancies e. Therapy should be tailored to the individual patient, with consideration given to the extent of involvement lesion location, size, number. Therapeutic options and efficacy are based on retrospective case series or anecdotal reports; no controlled trials exist. If associated with an underlying neoplastic or inflammatory disorder, treatment of the associated disease is warranted.
Dose, frequency, and duration of therapy is alopeciq where available and is based on published literature. For solitary or localized lesions, topical agents are first line because they are xlopecia risk, easy to administer, and low cost. Intralesional corticosteroids or excision should be considered for refractory or localized lesions, where cosmetically acceptable.
Photodynamic therapy is an option, but availability is more limited and may be more costly. Suggested therapies are based on anecdotal reports or small case series. Intralesional corticosteroids—triamcinolone acetonide, 2. For more numerous or widespread lesions, or if the patient shows no response or is intolerant to the options above, the following are first-line oral therapeutic options: Hydroxychloroquine mg orally, three times daily for 10 days, followed by mg orally, twice daily; response noted within 6 months, begin taper after lesions have cleared.
Treatment based on single case series of six patients. Minocycline mg orally, twice daily; response noted within 6 months; begin to taper slowly over months after lesions have cleared. Avoid use in patients under age 8. Treatment based on several case reports. The following options have been anecdotally reported to be effective; selection is based on individual patient tolerance mucibosa comorbidities, and on the comfort level of the prescribing physician.
Consider if patient is intolerant or unresponsive to first-line agents. Indomethacin 25mg orally, twice daily; monitor for gastrointestinal adverse effects, electrolytes, and creatinine. Caution if used in patients with gastritis, peptic ulcer disease, or renal insufficiency. Response within 3 months; recurrence may occur upon discontinuation. Isotretinoin is pregnancy category X.
If given to females of childbearing potential, monitor closely for pregnancy. Routine laboratory monitoring is suggested i. Dapsone mg orally, daily; response within 3 months; taper not defined, recurrence may occur upon discontinuation.
Alopexia monitoring required i. Contraindicated in patients with glucosephosphate dehydrogenase deficiency. Discontinue therapy or consider slow titration over months or years after complete response is evident. If recurrence is noted upon withdrawal, consider reinstitution of either the same or a new therapy. Of note, FM may resolve spontaneously. Long-term dermatologic follow up is necessary; a reasonable frequency for skin and lymph node exam is every months initially, then annually.
Periodic laboratory studies i. If lesional morphology should change considerably, skin biopsies should be repeated. Mucinsa presence of lesional skin T-cell clonality in FM does not portend a poorer prognosis. Clonal FM may regress completely. Long-term clinical monitoring of patients with FM is suggested. J Cut Med Surg. J Am Acad Dermatol.
Follicular Mucinosis (Alopecia Mucinosa)
Guitart, J, Alopeci, C. And are there other choices? No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Who is at Risk for Developing this Disease? What is the Cause of the Disease? Are You Confident of the Diagnosis? Characteristic findings on physical examination Lesions are often asymptomatic but may itch or burn; they develop over weeks or months not days.
Idiopathic follicular mucinosis on the trunk. Mycosis fungoides associated follicular mucinosis on posterior scalp. Expected results of diagnostic studies Skin biopsies are required for diagnosis. X40, B X Diagnosis confirmation In any subtype of FM, lesional skin polymerase chain reaction PCR based molecular testing for T-cell clonality may be positive, thereby suggesting monoclonality of the infiltrating T lymphocytes.
No serological or imaging studies are useful for establishing the diagnosis of FM. Etiology The etiology is not known. Pathophysiology Controversy exists alipecia to whether the disease is a neoplastic process or a reactive process. Topical corticosteroids mid to high potency —response expected within 3 months Tretinoin 0. SECOND LINE The following options have been anecdotally reported to be effective; selection is based on individual patient tolerance and comorbidities, and on the comfort level of the prescribing physician.
Quinacrine mg; not available in the United States Corticosteroids, oral—dose not delineated in reports Methotrexate, oral Radiation therapy—skin-directed electron beam or conventional radiation Interferon alfa-2a, intralesional 3 million units biweekly X 5, then monthly Patient Management Discontinue therapy or consider slow titration over months or nucinosa after complete response is evident.