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Geriatric hair and scalp disorders
Published in Robert A. Norman, Geriatric Dermatology, 2020
Scarring or permanent alopecias can be divided into inflammatory and non-inflammatory types71. Non-inflammatory causes include hereditary, developmental and neoplastic tumors, both benign and malignant. Of these, those causes that are developmental and hereditary will most probably be expressed and, hence, diagnosed before the geriatric age group under consideration in this chapter. Neoplastic lesions can be either primary to the scalp or metastatic to the scalp. Of the malignant tumors that are primary to the scalp, basal cell carcinoma and squamous cell carcinomas are common. Malignant melanoma and malignant hamartomas can be primary to the scalp. Both B-cell and T-cell lymphomas may occur on the scalp. Cutaneous T-cell lymphoma (CTCL) may exhibit a tendency to affect the follicles preferentially. Alopecia mucinosa occurs either in association with CTCL or primary to the scalp.
Management of acquired primary cicatricial alopecia
Published in Pierre Bouhanna, Eric Bouhanna, The Alopecias, 2015
Salvador Villablanca, Juan Ferrando
Alopecia mucinosa is usually benign and self-limiting in children and young adults, although it occasionally results in permanent patchy alopecia. It may be associated with lymphoproliferative disorders in older patients with multiple lesions. It is characterized by scaly erythematous infiltrate plaques formed by the confluence of follicular papules, with alopecia and dilation of the ostium follicular, localized predominantly on the face, shoulders, neck, and scalp. Histopathologically, the entity is defined by the presence of follicular mucinosis, accompanying a lymphomononuclear infiltrate that in many cases (30% of cases in adults) has a clonal origin, and corresponds to a cutaneous T cell lymphoma, of relatively indolent course in many cases.10,36
Cicatricial alopecias: Pathogenesis, classification, clinical features, diagnosis, and management
Published in Jerry Shapiro, Nina Otberg, Hair Loss and Restoration, 2015
Alopecia mucinosa (AM), also known as follicular mucinosis (FM), presents in two forms: a primary idiopathical mucinosis and as a secondary form in association with cutaneous T-cell lymphoma or mycosis fungoides [72–74]. AM presents as indurated, well-demarcated erythematosus or skin-colored patches of scarring or nonscarring alopecia that can be accompanied by diffuse hair loss [75] and alopecia of the eyebrows [76]. AM can clinically be mistaken for alopecia areata or other cicatricial hair loss conditions. Grouped follicular papules, follicular cysts, and follicular hyperkeratosis might be present in some cases. Lesions on the neck, the trunk, and the extremities have been described [76]. Early lesions of AM show mucin deposition in the outer root sheath and replacement of the entire pilo-sebaceous unit by pools of mucin in more advanced lesions [4,76]. Strictly speaking, AM is not a primary cicatricial alopecia because the hair follicle is not replaced by a true scar [4].
Classification and recommended treatment options for folliculotropic mycosis fungoides
Published in Expert Opinion on Orphan Drugs, 2018
Suzanne van Santen, Maarten H. Vermeer, Rein Willemze
In 1906, Giovanni already described a 13-year-old patient with MF showing prominent involvement of the hair follicles and associated alopecia [5]. However, the history of FMF actually starts in 1957, when Pinkus introduced the term alopecia mucinosa for a benign reactive disease process that was clinically characterized by follicle-based patches or plaques showing alopecia, and histologically by the presence of mucin deposits in hair follicles and sebaceous glands and a variably dense inflammatory infiltrate [6]. Since the diagnosis was particularly based on the presence of mucin deposits in the hair follicle and alopecia was not always present, Jablonska et al. suggested the term follicular mucinosis for this condition [7]. It was soon recognized that follicular mucinosis does not only occur in alopecia mucinosa, also referred to as the benign or idiopathic form of follicular mucinosis, but can also be found in other skin diseases, in particular in MF (MF-associated follicular mucinosis) [8]. Differentiation between idiopathic and MF-associated follicular mucinosis can sometimes be difficult and the question whether they should be considered as two completely different conditions or as the two ends of a disease spectrum is still a matter of debate [9]. In 1996, Vergier et al. introduced the term pilotropic MF for cases presenting with follicular lesions histologically characterized by infiltration of hair follicle epithelium by neoplastic T cells (folliculotropism), but without the presence of follicular mucinosis [10]. While previous studies had focused particularly on the differentiation between idiopathic and MF-associated follicular mucinosis, van Doorn et al. focused on differences between FMF and classic MF. It was found that patients with FMF had distinctive clinicopathologic features, were more refractory to several standard SDTs and had a worse prognosis than classic type MF. It was therefore suggested that FMF, with or without associated follicular mucinosis, is a distinct disease entity [2]. These observations were confirmed by several other studies [3,4] and in recent cutaneous lymphoma classifications FMF has therefore been included as a distinct variant of MF [1]. Since SDTs were often inadequate and the prognosis was found to be roughly similar to that of tumor-stage classic MF, it was suggested that patients with FMF should be treated accordingly with more aggressive therapies [2,3]. However, more recent studies defined a subgroup of FMF patients with an indolent clinical behavior and an excellent prognosis [11,12]. These studies indicate that not all patients with FMF run an aggressive clinical course and that indolent and aggressive subgroups of FMF may require a different therapeutic approach.