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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.77 no.6 Rio de Janeiro Nov./Dec. 2002
Follicular mucinosis: literature review and case report*
Antônio de Pádua Marques da Fonseca; Sebastião Honório Bona; Wanda Silveira M. da Fonseca; Francisco Soares Campelo; Prisco Medeiros de Melo Rego
Adjunct Professor at the Dermatology Department of the "Universidade Federal do Piauí"
Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis characterized by mucin accumulation predominantly in the pilosebaceous follicle. Two forms are distinguished: an idiopathic, or primary form, which is benign and without associated disease, and a symptomatic form associated with lymphoproliferative disorder, most commonly the cutaneous T-cell lymphoma mycosis fungoides. The authors present a new case of the disease and a brief review of the literature. The patient was a 26-year-old female with an asymptomatic plaque of erythema and scaling with alopecia on her right eyebrow. The lesion resolved spontaneously six months later. We have followed the patient over twelve months without relapse.
Key words: lymphoma; mucinosis, follicular; mucinoses.
Follicular mucinosis (FMu) is a relatively rare dermatosis, belonging to the cutaneous mucinoses group, that is, a complex of morphological and various etiopathogenic clinical entities, whose common denominator is represented by localized or diffused deposits of mucin in the skin or hair follicles.1 There are two forms of the disease: an idiopathic or primary form, and another symptomatic form associated to diverse benign and malignant processes.
In what follows, a case of primary FMu is reported, which was studied at the Serviço de Dermatologia of the Universidade Federal do Piaui (FUFPI). The study highlights its clinical manifestation and histopathology, and puts forth a few considerations on the entity.2
A 26-year-old faiodérmica female patient, without personal or family antecedents of note, sought assistance at the dermatology outpatients' clinic due to the appearance of cutaneous lesions six months earlier, which were asymptomatic and located at the inner third of the right eyebrow.
The dermatological test showed evidence of an erythematous plaque, which was lightly infiltrated, had a circular configuration, was very well defined and measured approximately three centimeters in diameter. The area of the lesion was covered over by a fine scales and exhibited porous dilated follicles and destitute of skin (Figure 1).
Exploration of the inner sensitivity of the plaque demonstrated a reduction of tactile, painful and cold-related sensitivity, but an increased sensitivity to heat.
The anatomopathologic exam of the stained cutaneous lesions due to hematoxiline-eosine (HE) showed discreet orthokeratosis, spongiosis, cystic degeneration with vacuolization of the follicular epithelium and moderate inflammatory infiltrate, constituted principally by lymphocytes and histiocytes and, in lesser abundance, by plasmocytes and eosinophils, predominantly located in the perifollicular area (Figure 2). By means of alcian blue staining, the cystic spaces acquired a bluish character of tone, confirming the presence of mucin in its inside (Figure 3) and, consequently, the diagnosis of FMu.
The course of the form without undergoing any type of treatment was toward progressive resolution of the lesion, with complete disappearance six months after the initial consultation.
In 1957, Pinkus3 described a novel dermatological entity under the name of alopecia mucinosa, in a presentation of his observations on six patients carrying the novel infirmity. However, as the alopecia is only clinically evident when found to be affecting cutaneous areas with the terminal hairs, the FMu designation seems to be more adequate. The latter was proposed in 1959 by Jablonska Chorzelski and Lancucky,4 and has been adopted by most of the authors who occupy themselves with the subject.1,5
FMu has been observed in all races and ages, and in both sexes equally.1
Its cause remains unknown,6 but the current tendency is to consider it a standard of follicular epithelium reaction to diverse noxas.5 Studies carried out with the electron microscope by Ishibashi7 seem to confirm that mucin present in the follicle and in the sebaceous gland proceeds from the altered epithelial cells of these structures.
In Rabello's description8, the objective aspect of FMu is that of "flat or raised plaques, pink or reddish-purple in color, at times squamous or keratotic, edematous or infiltrated, with or without pruritus; these plaques are covered by open porous pilosebaceos, small follicular papules and alopecic areas." However, it is worth mentioning that this form of clinical manifestation is not always that typical. Variations occur covering a range from nodular forms to others that simulate folliculitis,10 alopecia areata,11 cicatricial alopecia,12 chronic eczema,13 acneiform lesions,14 urticaria15 and erythrodermic forms inclusive.16 Special mention has to be made of the so-called tropical follicle T-cell lymphocytosis, a form of follicular mucinosis poor in mucin exhibiting cellular infiltrate constituted by T lymphocytes, and compromises all associated follicles to hyperkeratinization, spongious and follicular hyperplasia in the absence of mucin deposits. The finding of typical lymphocytes that do not attack the hair follicle, as well as the immuno-histochemical study, help to distinguish it from tropical follicle T-cell lymphocytosis. Such entities may follow an indolent course or evolve into tropical follicle T-cell lymphocytosis, fungoid micosis or anaplastic lymphoma.17 Also worthy of consideration are the frequent reports of paresthesias at the efflorence level, consistent in the reduction of tactile, pain and cold sensitivity, but with increased sensitivity to heat.10,18,19,20 20 In the case report, the authors also had the opportunity to observe these facts.
The FMu classification was recently expanded to include three forms:21
1. primary, short
2. primary, prolonged course;
3. secondary, associated to other processes.
The first, most common and benign, affects children and young adults with one to two lesions limited to the head and throat, which are spontaneously cured in a period of two months to two years, which in general do not recur. In the second form, occurring in patients slightly older in age to the preceding group, the lesions are more numerous, with some disseminated and having face, trunk and extremities as their sites of predilection. Despite the increasingly chronic course with recurrence over several years, the prognosis is, invariably, benign. The third variety, which usually incurs at 40 to 70 years of age, is manifested as generalized multiple plaques, infiltrated, associated to T-cell lymphomas especially fugoide micosis. The frequency with which lymphomas arise in FMu patients is very variable, once the criteria adopted in the distinct series are not uniform. This is how Coskey and Mehregan21 detected lymphoma in seven of the 50 FMu patients, or 14%; Emmerson,22 in eight of the 47, or 17%; Logan and Headington,23 in 21 of 80, or 26%; Mehregan et al., in nine of 33, or 27.2%; Gibson et al., in 19 of 59, 32%. On the other hand, the frequency of FMu in patients with cutaneous lymphoma has not been appreciated in the literature. Marti et al.25 found it in five of 43 patients, or 11%.
The histopathological image is characterized by mucin degeneration in the external sheath of the hair follicle and of the sebaceous gland.6 It was observed that the deposit of intercellular mucin and alterations of the epithelial cells, which become fusiforms or star-like, resemble embrionary or fibroblast cells. This could be followed by formation of cystic spaces partially mucin covered.10 The inflammatory infiltrate in the primary form of the FMu is perivascular and perifolicular.1 Its intensity is variable, being constituted of lymphocytes and eosinophils.22
Differentiation in purely histological bases between primary FMu and the FMu associated with lymphoma may be impossible. Generally, however, the presence of a high number of eosinophils in the inflammatory infiltrate and marked mucinous alterations in the follicular epithelium speak in favor of a benign form, while the presence of lymphocytic epidermotropism and of the dense perifollicular infiltrate of atypical cells point to FMu associated with lymphoma.1
In the presence of a plaque of alopecia with prominent follicles and minimal inflammatory alterations, the diagnosis of FMu must be considered.26 AAlopecia, despite its inconsistent character, has an enormous diagnostic value and has to be sought systematically above all in the scalp and eyebrows. Nonetheless, there do not appear to exist any dermatological clinical findings allowing a safe assertion of primary FMu or FMu associated with lymphoma.9 Repeated clinical and histopathological tests are necessary to achieve this objective.
Prior to initiating any treatment, it is convenient to consider possible spontaneous regression in the idiopathic forms. In case this does not occur, one may have recourse to one of the diverse therapeutic modalities employed by different authors. These have variable results: topical, interlesionary or systemic corticoids,6 dapsona,26 Puva27 interferons,28 surface radiotherapy22,30 and minocyclina.31 In the secondary forms, the therapy has to be orientated by the underlying disease. In a related case, as mentioned earlier, no specific medication whatsoever was prescribed for the cutaneous lesions, which showed spontaneous regression after six months.
The patient in question represents a typical case of primary FMu in its morphology as well as its topography, age of presentation and evolution. The differential diagnosis first associated it with tuberloid leprosy, especially due to the presence of disaesthesia at the level of the lesion and later with sarcoidosis and erythematous lupus. These diagnoses were disconsidered upon performing the anatomopathologic exam, which was shown to be characteristic of FMu.
To conclude, the authors draw attention to the need for periodic dermatological evaluations of FMu patients, due to the fact they may later develop a lymphoma. The patient here described is being followed up monthly at the outpatient's clinic, and is approaching the end of the first year of follow-up without showing signs of recurrence.
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Received in May,
25th of 2001.
Approved by the Consultive Council and accepted for publication in March, 23th of 2002
* Work done at the Dermatology Department of the "Universidade Federal do Piauí"