ARTICLE
In December 1998, a 51-year-old woman was admitted to our department
with a six-month history of skin lesions. Numerous erythematous-cyanematous
plaques were symmetrically diffused on the arms, legs and trunk; the skin
appeared atrophic, with surface irregularities and there was a remarkable
induration of subcutaneous tissues, causing impaired mobility (Fig.
1A and B).
All laboratory data were within normal ranges. Chest X-ray demonstrated
a moderate pulmonary fibrosis, confirmed by CT scan, with no reduction
of functionality. Involvement of other internal organs was excluded.
Histology showed a moderate atrophy of the epidermis, with an hyperpigmentation
of the basal layer. The collagen bundles in the papillary and reticular
dermis were thickened and closely packed. There was a moderate-to-severe
inflammatory infiltrate, with lymphocytes, histiocytes and a moderate
number of eosinophils, predominantly around the blood vessels. The blood
vessels showed a fibrotic wall and a narrowed lumen (Fig.
2). Direct immunofluorescence (DIF) demonstrated a positive staining
for IgM in the papillary dermis.
Inflammatory disseminated
morphea profunda
The diagnosis, made on the basis of clinical and histopathological data,
was that of disseminated morphea profunda.
Prednisolone treatment at 1 mg/kg daily was established, with a subjective
improvement of symptoms and a partial disappearance of the cutaneous lesions
after 3 months' therapy (Fig.
3A and B).
Comments
The term "scleroderma" is applied to a spectrum of disorders characterised
by thickening of the skin and subcutaneous tissue; two different clinical
categories can usually be identified: systemic sclerosis, in which visceral
lesions are present, and localized scleroderma, or morphea, in which lesions
are limited to the skin. The revised classification of morphea, based
on clinical morphological findings [1], subdivides it into five groups:
plaque, generalised, bullous, linear and deep, or subcutaneous.
Histologically speaking, all subtypes show homogenisation of the collagen
bundles, while the distinguishing factors are the distribution of the
lesions and the depth of the skin involvement [2, 4].
The deep morphea syndromes are characterised by the involvement of the
deep dermis, subcutaneous tissue, fascia or superficial muscle [5, 6],
whereas eosinophilic fasciitis, first described by Shulman in 1974 [7],
is usually described as a distinctive syndrome, characterised by eosinophilic
infiltration of the muscular fascia and by serious systemic manifestation
in nearly all reported cases [8].
In our case, histological diagnosis was that
of deep morphea; in fact, the histology demonstrates an involvement of
the subcutaneous tissues, with a deposite of pale and homogeneous collagen
in the papillary and reticular dermis without involvement of fascia or
muscle. An inflammatory infiltrate, predominantly constituted of lymphocytes
and histiocytes, with only a moderate number of eosinophils, was distributed
around the blood vessels, which showed a fibrotic wall.
No peripheral blood eosinophilia, hypergammaglobulinemia, or antinuclear
antibodies [9] were demonstrated in our case. These findings are rarely
present in patients with localized forms of scleroderma [2, 9, 10], whereas
they are usually described in eosinophilic fasciitis. Moreover, in our
case there was no evidence of antibodies against Borrelia burgdorferi.
Although evidence is still controversial, infection with Borrelia burgdoferi
may be involved in the pathogenesis of localized scleroderma, even if
individual susceptibility may play a role in determining the magnitude
of the inflammatory reaction and subsequent fibrosis.
In contrast to systemic sclerosis, internal
organ involvement is rare in localized scleroderma, even if has been documented
[2]. In our case, chest X-ray examination demonstrated a moderate pulmonary
fibrosis, confirmed by CT scan, with no reduction of functionality, whereas
involvement of other internal organs was excluded. Literature reports
evidence of pulmonary, muscle, esophageal and renal involvement [2, 11]
as well as a cardiac involvement similar to that seen in patients with
systemic sclerosis and eosinophilic fasciitis [12]. Visceral involvement
is usually associated with extensive skin involvement and has been occasionally
reported to evolve into systemic sclerosis, suggesting an overlap of the
two disorders [13].
Prednisolone treatment led to a subjective and objective improvement;
there was a reduction in the inflammatory skin changes and in the induration
of the lesion surface and an improvement in the joint mobility. These
changes are probably due to a reduction of the superficial and deep inflammatory
component which cannot be confirmed as the patient refused consent for
a further skin biopsy. Even if a number of patients with a poor response
to steroid therapy have been described [14, 15], in our case, we feel
the presence of a severe inflammation [16] justified the beneficial effects
of corticosteroids.
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