ARTICLE
Acantholysis between keratinocytes in the epidermis is the cardinal histological
sign found in pemphigus. This autoimmune induced phenomenon is responsible
for the transient bulla and the erosions commonly found in the disease.
If the group of the desmogleins is now well known to be the target antigen,
the mechanism of induction and of development of pemphigus is still speculative.
It has, however, been noted that various triggering factors may lead to
the appearance or reactivation of the disease. Exposition to X-rays or
ultraviolet radiation has been described to induce the disease, as has
development after burns [1-6]. A few reports have described the emergence
of lesions of pemphigus vulgaris (PV) on a surgical scar [1, 7-9]. We
describe here a case where reactivation of PV was defined by erosive and
crusted lesions occurring simultaneously in a recent and in an old scar
without involvement of any other part of the body, suggesting a Koebner's
phenomenon.
Case report
A 50 year-old patient was referred to our Department for evaluation
and treatment of painful and pruriginous erosive lesions on an erythematous
background, which appeared a few days earlier along two surgical scars
of the right knee. Two months before he underwent an arthroplasty of the
right knee and several years previously he had been operated on the external
part of the same knee. Two years before he had presented the same kind
of lesions on the head and back which led to the diagnosis of PV. At the
time of presentation, the patient was treated with low doses of prednisone
(2.5 mg every other day). On physical examination numerous erosive, scaly,
crusted and round lesions surrounded by a large erythema were found, covering
the recent longitudinal surgical scar on the anterior side of the right
knee (Fig. 1). A few lesions
were also present along the old scar of the external side of the right
knee. No other lesion was found on the skin or the oral mucosa. Microscopic
examination of the biopsy showed intraepidermal acantholysis where numerous
neutrophilic and eosinophilic granulocytes and a few acantholytic keratinocytes
were observed. In the dermis there was an inflammatory infiltrate composed
of lymphocytes and histiocytes with few eosinophils (Fig.
2). Direct immunofluorescence examination showed deposits of IgA,
IgG and C3 between keratinocytes. Indirect immunofluorescence on monkey
esophagus showed focal fluorescence at a dilution of 1/40 between keratinocytes.
An immunoblot was negative. These findings confirmed the suspected diagnosis
of a reactivation of pemphigus vulgaris and the patient was treated with
1 mg/kg prednisone and 150 mg azathioprine with rapid clinical regression
of his symptoms and lesions.
Discussion
Pemphigus is an autoimmune bullous disorder characterized by the appearance
of vesiculobullous and erosive lesions due to an acantholysis, which is
secondary to antibodies directed against epidermal antigens. The target
protein is now known to belong to the desmoglein group, which is located
in the extracellular portion of the desmosome. A few cases of PV occurring
simultaneously with the presence of other dermatoses such as psoriasis
or lupus erythematosus have been reported [10]. More interesting is the
case where a physical factor, leading to damage to the skin, was the triggering
event. Ultraviolet light, burns or radiotherapy have been successively
described in association with PV [2-6]. A similar case has also previously
been described four weeks after a cosmetic chemical peeling [11]. We report
the case of a PV, which was reactivated along two surgical scars. A few
cases have been described so far in the literature, following grafting,
breast surgery, chest drainage or rhinoplasty [1, 8, 9, 12, 13]. The interval
between the surgical procedure and the occurrence of pemphigus vulgaris
was between 2 months and 3 years. An interval as long as 40 years is even
reported for a surgical scar following the excision of a histiocytofibroma
[7]. To our knowledge this is the first reported case of PV reactivated
along both an old and a recent surgical scar simultaneously. This very
rare occurrence can be named a Koebner's phenomenon, which has been observed
in association with several dermatological diseases such as psoriasis,
lichen planus, erythema multiforme, lichen nitidus or vitiligo. The Koebner's
phenomenon is encountered when the typical features of a dermatosis are
observed on a part of the skin previously subject to friction or trauma.
The exact pathogenesis of the Koebner phenomenon remains unknown, particularly
in the case of PV. Immunological local alterations have been described
in such a situation and may contribute to the initiation of the disease.
It is likely that in our case the recent wound permitted the exposure
of pemphigus antigens, which then reacted with low levels of circulating
antibodies, which would not have caused the disease without the preexisting
trauma. This, however, does not fully explain why PV lesions appeared
along the ancient scar. Reactivation of latent antigens may however be
suspected in this case as the old scar was near the recent one.
Article accepted on 19/6/00
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