Acneiform follicular mucinosis of the head and neck region

ARTICLE

In 1957 Pinkus described a particular skin disease characterized by small alopecic areas all over the body, sometimes associated with follicular papules and fine scaling. The lesions were rarely infiltrated. The histopathologic picture showed the presence of mucin in the follicular epithelium and degenerative changes of keratinocytes. These histopathologic features were the marker of this disease, which was defined as Ôalopecia mucinosa' [1]. Other authors introduced the term Ôfollicular mucinosis' (FM) [2] to highlight the histopathologic picture of the disease more than the clinical features, which are often variable.

Braun-Falco described for the first time a relationship between FM and malignant lymphoma of the skin [3]. Two main types of FM are distinguished: the primary or idiopathic FM and the secondary or so-called symptomatic FM. The primary FM occurs in young patients, it presents with localized lesions not associated with other diseases and has a good prognosis, while the secondary FM occurs in elderly patients and is generally associated with mycosis fungoides (MF). More rarely, FM is associated with other cutaneous diseases although it is not specific for any of them [4, 5]. Recently [6], FM was referred to as a single disease ranging from a non-aggressive, chronic or autoresolutive form to a long-standing [7] or rapidly evolving cutaneous T-cell lymphoma [8], in the spectrum of the variant forms of MF.

The clinical manifestations of primary FM are protean. We describe two cases of FM, presenting as an acneiform eruption of the head and neck region [5, 9, 12], and review all cases described in the literature presenting similar clinical characteristics.

Case report

Case 1

A 45-year-old woman presented with several skin-colored papulo-cystic elements (2-5 mm in diameter) on her face, mainly located on the cheeks, but also on the chin and forehead, evolving since 4-5 months (Fig. 1). The eruption had failed to respond to several therapies. Histopathologic examination of a papular lesion showed a dense lymphocytic infiltrate in the dermis with a perifollicular distribution, composed mainly of CD4-positive T-lymphocytes admixed with histiocytes and numerous eosinophils, sometimes degenerated. Focal mucinosis with degenerated keratinocytes was observed in the follicular epithelium. Few lymphocytes were present in the mucinous areas. During three years of follow-up, spontaneous resolution of some lesions was observed.

Case 2

A 30-year-old woman presented with an acneiform facial eruption since three months. Multiple, 2-5 mm erythematous papulo-cystic elements were evident on her face and neck (Fig. 2). The patient had been treated with tretinoin gel 0.01 % and systemic tetracyclines without improvement. Histopathologic examination of a lesion showed
focal parakeratosis of the epidermis and a dermal periadnexal and perivascular dense lympho-histiocytic infiltrate with numerous eosinophils, which were more abundant in the deep dermis. The lymphocyte population was composed of small cells with dense chromatin. The follicular epithelium showed mucinosis, altered morphology and presence of rare lymphocytes and eosinophils (Fig. 3). After the biopsy the patient showed a tendency to spontaneous improvement without any therapy, and during two years of follow-up the lesion number and size have continued to decrease.

In both cases, results of routine laboratory tests were normal and analysis of the T-cell receptor gamma (TCRgamma) gene rearrangement, performed on formalin-fixed, paraffin-embedded tissue sections, using the polymerase chain reaction (PCR) technique [10], revealed a polyclonal pattern.

Discussion

Six cases of FM appearing as an acneiform eruption of the head and neck region have been described in the literature (Table I) and none of them was associated with a lymphoproliferative disease, confirming that the localization of FM to the head and neck region is generally related to a primary FM. Considering all reported cases, including ours, a rearranged TCR beta chain was only detected in two of the four cases described by Wittenberg [9], and in both cases, despite the short follow-up, there was a tendency to a spontaneous clinical improvement.

The etiopathogenesis of FM has not yet been elucidated. The exclusive localization to the face can suggest a relationship with external factors, such as irritating and allergic chemical substances, and environmental factors, such UV-radiations. The clinical aspect of this form is peculiar, since it can mimic an acneiform eruption, both for the presence of cystic lesions and for the patients' age. A past history of seborrheic and/or acneic disorders as well as a failure to respond to therapy for acne are elements that help to define the diagnosis. In our second patient, therapy with oral tetracyclines and topical tretinoin did not achieve any improvement. In addition, clinical differential diagnosis includes the Jessner's lymphocytic infiltrate/lupus tumidus, which can appear as infiltrated single or multiple plaques or nodules of the face [11].

By histopathologic examination, a folliculitic-acneic process can be ruled out because of the absence of neutrophils in the infiltrate, dilatation and scarring processes of the follicles. Presence of eosinophils in the infiltrate and mucin in the follicular epithelium but not in the dermis rules out a lupus tumidus, which can overlap Jessner's lymphocytic infiltrate [12].

To determine the prognostic evolution of FM, it is necessary to discriminate between the idiopathic type and the MF-associated type. The following criteria should be evaluated: age, extension, localization, histopathologic features and molecular findings. The evolution of MF-associated FM can be subtle, therefore it is important to monitor the disease course, re-evaluating the diagnostic criteria at all times, in order to determine the possible onset of MF. Reviewing the literature regarding the differentiation of the idiopathic FM from the MF-associated type, Emmerson [13] reported forty cases of FM, collected over a period of sixteen years, and differentiated three main groups. The first group (55 %) showed a spontaneous resolution in 2-24 months, the second one (25 %) had a chronic course but did not evolve into MF and the third one was associated with MF, with clinical features similar to Ôfollicular MF'. He was unable to find any case associated with MF and localized only to the head and neck region. Gibson et al. [14] described a relationship between old age and MF-associated FM, reporting the absence of MF in the cases confined to the head and neck region, in the group younger than forty years of age.

Several histopathological criteria can be evaluated to find specific features evoking a lympho-proliferative disease: atypia of the infiltrating cells, density of the infiltrate, presence of a band-like lymphocytic infiltrate, percentage of eosinophils and plasma cells and degree of mucin deposition. The presence of eosinophils has been controversially considered by some authors as a negative prognostic sign [15], and by others as a positive one [16, 17]. In our two patients we found an abundant eosinophilic population. However Gibson et al. [14] concluded that no single histopathologic feature can predict which patient with FM will have a benign course or not, and multiple histopathological criteria with repeated biopsy specimens are required for diagnosis, in addition to follow-up of these patients. In the review by Cerroni et al., no histopathologic clue was found which differentiates the two FM types [6]. Genotypic analysis may be helpful in defining the presence of a clonal population, but there is no direct correlation between monoclonality and disease evolution [6].

There is no standard therapeutic approach for FM. Several therapies have been used including interferon, psoralen in association with UVA, dapsone and indomethacin [9]. Oral tetracyclines associated with topical treatments for acne and oral isotretinoin have been reported as partially effective in FM presenting as an acneiform eruption [9]. In our two patients, tendency to a spontaneous resolution was observed and specific therapy for acne was ineffective.

Our two patients confirm the favourable prognosis of the single localization of FM to the head and neck region. All the cases described in the literature presenting as acneiform eruptions of the head and neck show a good prognosis, irrespective of age of onset and of the presence of a monoclonal population. However, the limited number of reported cases does not allow us to draw conclusions regarding the exact nosology and prognosis of this variant. A larger number of patients and longer follow-ups are needed to better understand this unusual variant.

CONCLUSION

We thank Mrs. Paula Franke for her formal linguistic revision of the manuscript.

REFERENCES

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