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.
Article accepted on 30/12/02REFERENCES
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