Alopecia Mucinosa/Follicular Mucinosis
Robert A. Lee
DEFINITION
Alopecia mucinosa (AM) and follicular mucinosis (FM) refer to the same entity. AM describes the clinical manifestation, whereas FM denotes the classic histopathologic finding of mucin accumulation within the follicular epithelium.1,2 FM can be subcategorized into two clinicopathologic variants. The primary, benign/idiopathic form—primary FM (PFM)—is a spontaneously remitting process in children and young adults.3 The secondary, lymphoma-associated form—lymphoma-associated FM (LAFM)—occurs in elderly in the setting of mycosis fungoides (MF) or Sézary syndrome.3 These entities should be differentiated from FM secondary to various inflammatory or noninflammatory skin conditions.
EPIDEMIOLOGY
Patients with PFM are more frequently younger (mean age 39 years) and the female:male ratio is 3:1. In contrast, patients with LAFM are more often older (mean age 54 years) with a male:female ratio of 2:1.3
CLINICAL PRESENTATION
The clinical lesions of PFM are frequently solitary. They display various morphologies from hypopigmented macules and patches to erythematous, sometimes scaly papules and plaques, sometimes with follicular prominence.4 PFM lesions tend to concentrate around the head and neck and might present as acneiform eruptions5 (Fig. 42-1). Other locations, such as trunk and extremities, are not uncommon (Fig. 42-2). Nonscarring alopecia and pruritus can be present. The lesions are often persistent but tend to follow a benign albeit chronic course.4 Rarely, these cases evolve into MF6,7 or precede the development of Hodgkin disease.8,9,10,11
FIGURE 42-2. Follicular mucinosis. Erythematous and scaly patches and plaques on the back. Note central areas of alopecia. |
LAFM is generally distributed more diffusely as patches and plaques along with areas of alopecia.5 Most commonly, this is seen in the context of cutaneous T-cell lymphoma (CTCL) such as folliculotropic MF (FMF) or Sézary syndrome.3,12 To some authors, FM may be a variant of FMF.13 A recent systematic review of FMF suggests that FM can be found in 74% of biopsy specimens reviewed.14 Interestingly, some cases of FMF can present with circulating atypical lymphocytes without erythroderma. If the histologic findings show clearly a pattern of MF and there are abnormal lymphocytes in the blood, these cases are best treated at FMF. However, because MF itself may be indolent, this association with lymphoma may develop over time.6
TREATMENT
Treatment of PFM includes topical and systemic corticosteroids, oral tetracycline-class antibiotics, hydroxychloroquine, dapsone, isotretinoin, UV phototherapy, and indomethacin.6,15 The response of LAFM to skin-directed therapy is poor even in early-stage disease. Treatment modalities include psoralen plus ultraviolet A (PUVA) therapy with oral bexarotene or PUVA with interferon α.16