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Alopecia mucinosa, also called follicular mucinosis, is a rare inflammatory disease affecting the pilosebaceous units in the skin. It presents clinically as follicular papules with or without erythematous scaly plaques and evident hair loss in the absence of other conditions causing alopecia. Histologically, it presents as a deposition of mucopolysaccharides (mucin) within the outer root sheath and sebaceous glands. We present here a case of alopecia mucinosa in a 26-year-old male.

We present the case of a young patient, without comorbidities, who developed an erythematous-squamous, disseminated, discrete itchy rash, associated with scarring alopecia at the level of the scalp and the external third of the eyebrows, in 3-year evolution, in which two skin biopsies showed: subacute spongiotic dermatitis and psoriasiform dermatitis. As new lesions continued to appear despite treatment with phototherapy, systemic, local corticosteroids, it was decided to perform another skin biopsy: loose areas, granular appearance that color blue with Alcian Blue staining, thus supporting the diagnosis of follicular mucinosis. The treatment consisted of Acitretin 30 mg / day, with favorable evolution. Follicular mucinosis is a rare inflammatory disease, characterized by mucin deposits located in the follicular epithelium and sebaceous glands. There are two clinical forms: a primary, benign, limited to the skin, and a secondary form, commonly associated with cutaneous T-cell lymphoma. The evolution of both forms of follicular mucinosis is a chronic one, and the correct therapeutic attitude consists primarily in the periodic surveillance of the patient, with possible repeat biopsy, to exclude the form associated with a mycosis fungoides. The management of such patients is extremely difficult, as a number of conditions must be evaluated, such as: screening for collagenosis, diabetes, inflammatory bowel disease, evaluation of thyroid function, tumor markers.

Follicular mucinosis (FM) is a rare inflammatory disorder histologically characterized by mucin deposition in the follicular epithelium. There is no standard therapy for FM and several treatments have been described in the literature. We present the case of a 59 year-old female affected by a recalcitrant FM with diffuse scalp alopecia, in which complete clinical remission was achieved after a combination of topical tacalcitol and photodynamic therapy.

Both primary and secondary forms of cicatricial alopecia have been described. The hair follicles are the specific target of inflammation in primary cicatricial alopecias. Hair follicles are destroyed randomly with surrounding structures in secondary cicatricial alopecia. This 2-part continuing medical education article will review primary cicatricial alopecias according to the working classification suggested by the North American Hair Research Society. In this classification, the different entities are classified into 3 different groups according to their prominent inflammatory infiltrate (ie, lymphocytic, neutrophilic, and mixed). Part I discusses the following lymphocytic primary cicatricial alopecias: chronic cutaneous lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia, and Graham–Little syndrome.

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. We report a case of an 11-year-old female with widespread lesions showing distinct clinical and histological features of FM with a brief review of the literature.

Follicular mucinosis coexisting with lymphoproliferative disorders has been thoroughly debated. However, it has been rarely reported in association with inflammatory disorders.Thirteen cases have been retrieved, and those with cutaneous lymphoma or alopecia mucinosa were excluded.Follicular mucinosis was found in the setting of squamous cell carcinoma, seborrheic keratosis, simple prurigo, acne vulgaris, dextrometorphan-induced phototoxicity, polymorphous light eruption (2 cases), insect bite (2 cases), tick bite, discoid lupus erythematosus, drug-related vasculitis, and demodecidosis. Unexpectedly, our observations revealed a preponderating accumulation of mucin related to photo-exposed areas, sun-associated dermatoses, and histopathologic solar elastosis. The amount of mucin filling the follicles apparently correlated with the intensity of perifollicular inflammatory infiltrate, which was present in all cases. The concurrence of dermal interstitial mucin was found in 7 cases (54%).The concurrence of interstitial dermal mucinosis or the potential role of both ultraviolet radiation and the perifollicular inflammatory infiltrates in its pathogenesis deserves further investigations. Precise recognition and understanding of this distinctive, reactive histological pattern may prevent our patients from unnecessary diagnostic and therapeutic strategies.

Follicular mucinosis represents a term for a histopathologic reaction pattern in follicular epithelium. It is a characteristic of alopecia mucinosa. However, it may also occur in a variety of unrelated conditions. Epidermal nevi are considered to be hamartomatous disorders and they can show a predominant component of non-organoid (keratinocytes) and/or organoid nevi. All the cases of epidermal nevi described with mucin deposits until now are reported as mucinous nevus or mucinous eccrine nevus; in the first type of disorder, diffuse mucin deposition is only seen in the papillary dermis, and in the second type, the mucin is found around the proliferation of eccrine structures. We believe this is the first reported case of epidermal nevus along Blaschko's lines exhibiting typical microscopic findings of mucinosis exclusively distributed inside the follicular epithelia.

Background Some authorities consider alopecia mucinosa (AM)/follicular mucinosis (FM) to invariably represent mycosis fungoides (MF). This understanding of AM/FM derives from observations in adults. Objectives We sought to explore the clinicopathologic features and natural history of pediatric AM/FM. Methods Medical records were searched for children given the diagnosis of AM/FM from 1998 through 2009. Diagnosis of AM/FM was defined as the presence of well-demarcated hairless plaques with follicular prominence plus an abundance of mucin on histopathologic examination. Results Forty children with a clinical diagnosis of AM/FM were identified. Nine did not meet the inclusion criteria. In the 31 remaining cases (16 boys, 15 girls) the mean age at onset was 9 ± 3.5 years. Histopathologic examination showed folliculotropism in 28 patients (90%) and epidermotropism in 15 (48%). Twelve cases fulfilled the International Society of Cutaneous Lymphomas (ISCL) diagnostic criteria for early MF. The histopathologic findings were typical of MF in only in two of these cases. T-cell receptor gene rearrangement was positive in 3 of 6 (50%) of tested samples, one in a patient who fulfilled the ISCL criteria for early MF. Mean duration of follow-up was 6.2 ± 3.7 years. All skin lesions resolved and none persisted or recurred. Hodgkin lymphoma was diagnosed 6 months after diagnosis of AM/FM in one patient. Limitations This was a retrospective study. Conclusions Although some pediatric cases meet the diagnostic criteria for MF, AM/FM cannot be regarded unequivocally as early follicular MF in this age group. We suggest the current diagnostic criteria for early MF should exclude children with AM/FM. Long-term follow-up of children with AM/FM is nevertheless warranted.

Cutaneous T-cell dyscrasia represents a heterogeneous group of persistent clonal and usually epitheliotropic T-cell infiltrates with a seemingly low risk for progression to mycosis fungoides (MF). Mucin-poor folliculotropic T-cell lymphocytosis is the least well characterized with only a few anecdotal case reports. Cases of folliculotropic lymphocytosis were retrieved via a natural language search from 2 dermatopathology databases. Comprehensive light microscopic, phenotypic, and molecular studies were conducted. There were 12 females and 12 males, with mean age of 41 years. There were 2 main clinical presentations: facial erythematous infiltrative papules and/or nodules vs extremity and truncal solitary, large, scaly plaques. The mean duration of the lesions was 3 years. Progression to follicular MF was noted in 1 case. Migration of small lymphocytes into the superficial portions of the hair follicle unaccompanied by other inflammatory cells or mucin was observed; lymphocytes with a cerebriform appearance could be seen. The CD4/CD8 ratio was more than 5:1 with significant reductions in the expression of CD62L and CD7. Of 20 cases tested, 5 showed monoclonality. Folliculotropic T-cell lymphocytosis is a form of pilotropic T-cell dyscrasia distinct from MF and alopecia mucinosa; the course seems indolent in most cases.

Primary cicatricial alopecias are uncommon folliculocenteric inflammatory disorders that represent a ‘trichologic emergency’. Unless correctly diagnosed and abruptly treated, these diseases can lead to permanent destruction of hair follicles and irreversible hair loss. Based on the predominant inflammatory cell type present, they are classified into lymphocytic, neutrophilic and mixed. The etiology and pathogenesis of many of these disorders are incompletely understood. A systematic review of classification, pathogenesis, clinical features, histopathologic findings and therapeutic options will be highlighted in this article.

A 29-year-old Japanese man had an asymptomatic, solitary, indurated, erythematous plaque measuring 30 mm x 30 mm on his jaw that had been present for a month. The skin lesion had follicular hyperkeratosis, and lacked hair. A skin biopsy specimen showed a dense perifollicular infiltration composed of lymphocytes, with an admixture of eosinophils in the full thickness of the dermis. The hair follicles and sebaceous glands had reticular epithelial degeneration by mucoid material of the outer root sheath and sebaceous epithelium. The mucoid material stained with Alcian blue at pH 2.5. The clinical and histological features were consistent with the diagnosis of follicular mucinosis. On immunohistochemistry, the outer root sheath cells with reticular epithelial degeneration were nestin-positive and keratin 15-negative. These results suggest that the outer root sheath cells with reticular epithelial degeneration come from the nestin-positive, multipotent, hair follicle stem cells.

Background:Follicular Mycosis Fungoides (FMF) is an under-recognized disease in India. Its clinical mimics include Hansen′s disease and Sarcoidosis.Aims:To describe the clinical and pathological features of FMF.Materials and Methods:All cases of FMF between January and December 2007 were retrieved. Cases of conventional epidermotropic MF with a minor follicular component were excluded. Slides were reviewed by two observers. The following criteria were assessed: degree and density of folliculotropism of lymphocytes, location of folliculotropism (infundibular / isthmic / bulbar), follicular mucin, eosinophils, granulomas, and conventional epidermotropism. Each feature was assigned a semi-quantitative grade.Results:There were four cases of FMF, with an equal gender distribution and a mean age of 17.5 years. All lesions were on the face. They presented as: hypopigmented patches (2) and erythematous plaques (2). Alopecia was seen in two cases. The clinical diagnosis was Hansen′s disease in all four, with a differential of Alopecia mucinosa / Sarcoidosis in two cases.The histological features seen were: disproportionate folliculotropism, lymphocyte tagging with haloes, follicular mucin, and nucleomegaly / convolution in all four cases, prominent eosinophils (2), epithelioid granulomas (1), eccrine infiltration (4), parakeratosis at the follicular ostia (2), and sebaceotropism (1). The infiltrate was bulbar (4) and isthmic (2). The rest of the epidermis showed no hint of conventional MF.Conclusion:The preferential features for FMF were involvement of face, dominant folliculotropism, nuclear atypia and convolution, and follicular mucin. Presence of granulomas and eosinophils necessitated exclusion of infectious causes. The absence of findings of MF in the rest of the epidermis should not deter pathologists from rendering this diagnosis.

In this 2-part article, the authors review the primary cicatricial alopecias. Primary cicatricial alopecia can be defined as predominantly lymphocytic, neutrophilic, or mixed based on the nature of the follicular infiltrate that is present around affected hair follicles. Lymphocytic primary cicatricial alopecias include chronic cutaneous lupus erythematosus (discoid lupus erythematosus), lichen planopilaris, classic pseudopelade of Brocq, central centrifugal cicatricial alopecia, alopecia mucinosa, and keratosis follicularis spinulosa decalvans. In this first part, the authors summarize the classification, epidemiology, diagnostic approach, and patient management of lymphocytic cicatricial alopecias. In part II, the authors will focus on neutrophilic cicatricial alopecias and mixed cicatricial alopecias.

Objectives: Ofuji's disease (OD) or eosinophilic pustular folliculitis and human immunodeficiency virus-associated eosinophilic folliculitis (HIV-EF) both show eosinophil-rich folliculocentric infiltrates, and it is not clear whether they are distinguishable pathologically. Follicular mucinosis (FM) has been observed in lesions of EF; such cases need to be differentiated from alopecia mucinosa (AM). Methods: We compared various pathologic features in 13 cases of OD, 10 cases of non-OD-typed papular EF (seven HIV-positive and three HIV-negative), and five cases of AM. Results: All cases of EF showed eosinophilic infiltrates affecting mainly the isthmus or/and sebaceous gland. Eosinophil-rich pilosebaceous pustules or/and microabscesses were noted in 69% of the biopsy specimens of OD, 73% of papular EF, 71% of HIV-EF, and 0% of AM. Mucin deposits, often abundant, were found in sebaceous lobules or/and isthmus in 41% of OD and 100% of AM. Compared with AM, OD with FM tended to show more numerous eosinophils, less abundant mucin, and most significantly, eosinophilic infundibular pustule or sebaceous microabscess. Conclusion: Our study suggests that OD and HIV-EF are indistinguishable pathologically and the diagnosis requires clinical correlation. FM was not uncommonly seen in lesions of OD. OD with FM may be difficult to differentiate from FM, especially in cases presenting with non-annular or non-pustular lesions, but the diagnosis might be facilitated by finding eosinophil-rich pustule, microabscess, or infiltrate in pilosebaceous units microscopically.

Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.

Follicular mucinosis can develop as a primary form or associated with other benign or malignant skin conditions. We present a case of primary follicular mucinosis in which complete clinical remission was achieved after treatment with topical imiquimod 5%. Primary follicular mucinosis (PFM), also called idiopathic alopecia mucinosa, is considered a distinct entity, the clinical course of which is usually benign, but its pathogenesis remains unclear. There is no standard treatment for PFM, and a wide variety of therapies have been described. There are no previous reports of treatment of PFM with imiquimod.

Since the original descriptions of follicular mucinosis, accumulating experience shows that patient age, distribution of lesions, and duration or extent of disease do not reliably distinguish benign primary follicular mucinosis from secondary follicular mucinosis, associated with cutaneous lymphoma. More recently, it has been suggested that individuals with follicular mucinosis demonstrating a clonal T-cell receptor gene rearrangement may be at higher risk for the development of lymphoma. Long-term follow-up of 7 patients younger than 40 years with primary follicular mucinosis are reported. In all cases, there was no clinical or histologic evidence of associated dermatoses or lymphoma at the time of diagnosis. Five of the patients have clonal T-cell gene rearrangement as determined by Southern blot analysis. Clinically, at the time of diagnosis, lesions of primary follicular mucinosis ranged from papules confined to the face to widespread cutaneous plaques. After a mean follow-up of 10 years (range, 5-23 years) from the onset of disease, the majority of patients continue to have cutaneous manifestations of follicular mucinosis despite various treatments. There is no evidence of progression to cutaneous T-cell lymphoma in any patient despite the presence of a clonal T-cell receptor gene rearrangement. Continued prolonged follow-up of patients with clonal primary follicular mucinosis is necessary to determine the significance of infiltrates harboring a T-cell receptor gene rearrangement. However, in our experience with this group of selected patients, primary follicular mucinosis has been a clonal disorder with limited or "benign" cutaneous manifestations. (J Am Acad Dermatol 2002;47:856-62.)

The diagnosis of disorders of the hair and scalp can generally be made on clinical grounds, but clinical signs are not always diagnostic and in some cases more invasive techniques, such as a biopsy, may be necessary. This 2-part article is a detailed review of the histologic features of the main types of alopecia based on the traditional classification of these disorders into 2 major groups: scarring and nonscarring alopecias. Scarring alopecias are disorders in which the hair follicle is replaced by fibrous scar tissue, a process that leads to permanent hair loss. In nonscarring alopecias, the follicles are preserved and hair growth can resume when the cause of the problem is eliminated. In the second part of this review, we describe the histologic features of the main forms of scarring alopecia. Since a close clinical-pathological correlation is essential for making a correct histopathologic diagnosis of alopecia, we also include a brief description of the clinical features of the principal forms of this disorder.

We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.

A diagnosis of alopecia mucinosa, occurring as a single scalp lesion, was made in a 40-year-old white woman who had a history of trauma. Follicular mucinosis, Staphylococcus aureus , and oligoclonal expansion of the T-cell receptor Vβ chain genes 6 and 7 were present in the skin. Epidermotropic T-cell skin diseases with oligoclonal T-cell proliferations may be the result of HLA- and cytokine-determined reaction patterns to persistent antigens. (J Am Acad Dermatol 1997;36:828-31.)