ARTICLE
A 61 year-old woman had noticed a bullous eruption at the site of a stoma
(Fig. 1). The stoma had
been undertaken 9 years earlier for postradiation colitis after the irradiation
of an adenocarcinoma of the cervix.
Bullae were found to be localized only around
the stoma and hindered the use of stomal devices. Allergic contact dermatitis
was suspected but epicutaneous tests were negative for ICDRG batteries,
antiseptics and stomal devices. A bethamethasone dipropionate treatment
was inefficient and new bullae appeared on the skin around the stoma and
also on buccal, specially on gingiva and palate (Fig.
2) and genital mucous membranes. Histological examination of a
bulla showed a subepidermal blister associated with a dermal infiltrate
of lymphocytes, neutrophils and rare eosinophilic cells. Direct immunofluorescence
performed on a perilesional skin biopsy demonstrated linear staining with
IgG and C3 at the dermo-epidermal junction. Indirect immunofluorescence
analysis of 1.0 mol/l sodium chloride split skin was negative and no circulating
antibodies were detected in the serum. Western blot analysis of an epidermal
extract (kind gift from Pr. P. Bernard, Limoges, France) demonstrated
reactivity with a 180 kD protein (Fig. 3).
The count of blood eosinophils was in the normal range (208/mm3).
The patient was treated with prednisone (1 mg/kg/d) for 2 months and the
dosage was progressively reduced over 3 years. The mucous bullae disappeared
but the peristomal lesions persisted. Tetracycline treatment (2 g/d for
2 months) and local corticotherapy (clobetasol dipropionate) led to significant
improvement and the patient could then use a stomal device.
Comment
We describe a case of peculiar autoimmune blistering disease occurring
only on the mucous membrane and around a stoma. The clinical presentation,
with lesions on the mucous membrane and the presence of a unique 180 kD
antigen, was compatible with the cicatricial pemphigoid diagnosis despite
the lack of cicatricial scarring. Unfortunately, direct immunoelectronmicroscopy
was not performed in our patient leaving us unable to categorize definitively
this sub epidermal blistering disease.
Pemphigoid has rarely been reported on peri-stomal
skin [1-3]. We have only found three cases in the literature (Table
I). In two cases [1, 3], the pemphigoid was a localized type.
In the third case [2], the onset of generalized pemphigoid was after an
eczematous eruption arising around a colostomy. The colostomy was performed
for an adenocarcinoma of the sigmoid, cancer of the rectum, and post-radiation
colitis, following irradiation of an adenocarcinoma of the cervix. The
period between the stoma operation and bullous eruption varied from a
few months to 9 years. In these three cases, bullous pemphigoid was diagnosed
but neither immunofluorescence on NaCl split skin nor Western blot analysis
were performed (Table II).
In our patient, systemic corticosteroid treatment was efficient on the
mucous membrane lesions but not on the peristomal lesions. Local corticotherapy
associated with tetracycline has been proposed for the treatment of classic
bullous pemphigoid [4] and good clinical results were obtained with this
regimen in the case of Van de Maele [3]. Localized pemphigoid arising
on an area of cutaneous injury are sometimes described, suggesting a Koebner
phenomenon. Pemphigoid is described on amputation stumps [5], scars [6],
after UV radiation [7], and more frequently after irradiation therapy
[8]. Cicatricial pemphigoid has been also described after radiotherapy
[9]. Post-radiotherapy pemphigoid could be suspected in our case, but
the lesions were not strictly limited to the irradiated area.
The pathogeny of the disease is unclear. In
some patients, the trauma could lead to a predisposition to develop blisters
through modification of the basement membrane zone antigens which permit
antigen presentation, followed by subsequent auto-immunisation [10].
The occurrence of an autoimmune blistering skin disease on peristomal
skin should be recognized and differentiated from contact dermatitis by
systematic epicutaneous tests and direct immunofluorescence study. Characterization
of target antigen could enhance classification of this peculiar form of
autoimmune bullous disease, and perhaps lead to a better understanding
of the lesional mechanisms of lesions occurring on atypical sites.
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