ARTICLE
A 48-year-old woman was initially seen in 1996 (at the age of 45 years)
with a photosensitive erythema and polyarthritis. At that time she had
an antinuclear antibody titer of 1:80, with a speckled pattern and antibody
to dsDNA was positive (365 U/ml). Subsequently systemic lupus erythematosus
was diagnosed and she was treated for several years with oral prednisolone
40 mg/day and azathioprine 150 mg/day.
In 1999, the patient was seen because of multiple cutaneous nodules
that had appeared during the previous six weeks. She was still taking
10 mg prednisolone every day. On clinical examination numerous nodular
lesions, round or oval in shape and about 1 cm in diameter, with a color
ranging from pink-red to brown, with sharp margins were noted on the legs.
The surface of these nodules was smooth and their consistency was firm
(Fig. 1). In total the patient presented about 20 recently erupted
nodules. She did not complain of any symptoms. Histological examination
revealed fibrohistiocytic cells between thickened collagen bundles with
acanthosis of the overlying epidermis (Fig. 2). On immunohistochemistry
the spindle cells were negative for CD34, CD1a, CD68, S-100, muscle-specific
actin and HMB-45.
Multiple disseminated dermatofibromas in a woman with systemic lupus
erythematosus
Dermatofibromas (DF) are benign dermal proliferations that appear as
nodules, usually localized on the limbs, especially the legs of middle-aged
women. Although cases of solitary DF or occasionally a few DF are common,
multiple DF are rather infrequently observed. Baraf and Shapiro suggested
that the description "multiple dermatofibromas" be reserved for cases
of more than fifteen lesions [1, 2]. Niemi found that only 29 (8%) of
379 patients with DF possessed more than two lesions [3].
Multiple disseminated dermatofibromas (MDDF) may occur in patients with
various conditions such as diabetes mellitus [4], obesity [5], hypercholesterolemia
[5], hypertension [5], hydronephrosis [4], pregnancy [6], neoplastic diseases
[7] or following organ transplantation [8]. However, MDDF are more frequently
associated with immune-mediated disease under immunosuppressive therapy,
such as myasthenia gravis [9], atopic dermatitis [10], ulcerative colitis
[11], pemphigus vulgaris [11], Sjögren syndrome [12] and SLE, which
represents the most commonly associated disease [11-19]. Moreover, some
cases of MDDF have been recently observed in patients with HIV infection
[20, 21].
The etiology of DF is unclear. Whether it represents a reactive or a
neoplastic process is a subject of debate. It has been suggested that
DF may result from insect bites [20]. Evans, et al. [22] reported
that 87% of 30 patients with DF recalled having been bitten by insects
at some time before the development of their lesions. Recently it was
proposed that DF represents an abortive immunoreactive process mediated
by dermal dendritic cells [23]. According to this hypothesis, the development
of MDDF in immunodeficiency states could be facilitated by the inhibition
of down-regulatory T cells. Alternatively, DF could develop as a response
to a putative pathogen that could not be cleared by a suppressed immune
system [21].
Although MDDF sometimes occur in individuals with normal immune function,
they usually appear in immunocompromised patients. If MDDF are present,
the status of the patient with regard to autoimmune diseases or immunodeficiencies
due to either HIV infection or the intake of immunosuppressive drugs should
be considered. *
Article accepted on 2/11/00
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