ARTICLE
Bullous pemphigoid (BP) is a subepidermal blistering
disease which is characterized by the presence of tissue-bound and circulating
autoantibodies against the basement membrane zone. There are several clinical
variants in BP, and Brunsting-Perry type of BP is one of the localized
types of BP [1]. We report a case presenting a unique distribution of
vesiculobullous lesions on the face and neck.
Case report
A 33-year-old woman developed pruritic skin lesions with small tense
blisters on the face and neck (Fig.
1). The vesiculobullous lesions were observed most frequently
on the perioral area, whereas other areas, such as the chest, axillae
and inguinal regions were rarely involved. There was no apparent ocular
and oral mucosal involvement. Serum IgG level was 1,730 mg/dl (normal,
870 to 1,700 mg/dl). Serum levels of total protein, IgA, IgM, IgE, C3,
and C4 were within normal limits. White blood cell count was normal, and
peripheral blood eosinophilia was not observed. Histopathology of a skin
biopsy revealed a subepidermal blister with dermal infiltrates of neutrophils
and eosinophils. Direct immunofluorescence demonstrated a clear linear
deposition of IgG and IgA (Fig.
2a, b), as well as a faint C3 deposition, along the basement membrane
zone. At the split areas, these reactivities were seen predominantly at
the dermal side (Fig. 2b).
Indirect immunofluorescence using sections of both normal skin and 1M
NaCl split skin showed no circulating antibodies of either IgG or IgA.
Immunoblotting using normal human epidermal extracts, dermal extracts,
and recombinant protein of BP180 NC16a domain did not show any positive
results. Treatment with minocycline combined with niacinamide, which was
reported as a potent treatment for BP [2], was initiated. Although the
blisters were dramatically reduced within 1 week, additional oral prednisolone
20 mg/day was needed to prevent new blisters. During the following ten
months, fresh vesiculobullous eruptions developed twice when the patient
was exposed to strong sunlight, and caught a cold.
Discussion
Classical cicatricial pemphigoid which is also named benign mucosal
pemphigoid is characterized by involvement of conjunctiva and oral mucosa
resulting in atrophic scar formation [3]. In 1957, Brunsting and Perry
described a unique form of cicatricial pemphigoid which was characterized
by pruritic vesiculobullous lesions located predominantly on the face
and neck with atrophic scar [1]. There-after, MacVicar and Graham proposed
the term "localized chronic pemphigoid" for the similar cases in which
the skin lesions were mostly located on the head and neck, and mucous
membranes could be affected occasionally [4]. Histopathological and immunological
findings in those cases were consistent with BP. The differences of clinical
features between the two diseases are summarized in table
I.
The present case showed similar findings to these variants of BP. The
distribution of blisters was the most unique clinical feature in this
case, and was compatible with that of Brunsting-Perry type of localized
BP. Although the Brunsting-Perry type of localized BP has been described
as a subtype of cicatricial pemphigoid because of atrophic scar formation,
absence or rareness of mucosal involvement was a characteristic feature
in previously reported cases [1, 5, 6], which also concorded with our
case. However, our case did not develop any apparent atrophic scars, and
was relatively younger than the previously reported cases. Because positive
reactivity was not obtained by indirect immunofluorescence and immunoblotting,
the antigenic molecules could not be identified. Although the immune reactivity
was observed at the dermal side of the direct immunofluorescence assay,
an immunoblotting assay with normal human dermal extracts showed negative
reactivity. Meanwhile, clear deposition of IgA along the basement membrane
zone, which was as strong as that of IgG, was a distinctive finding in
the present case. Because similar staining patterns have been reported
sporadically in cases of localized BP [7] and Brunsting-Perry type of
localized BP [8], involvement of IgA in the BP spectrum needs to be elucidated,
as discussed recently [9]. The reason why the present case developed the
skin lesions predominantly on the face and neck needs to be clarified.
Because the vesiculobullous lesions occurred recurrently after exposure
to strong summer sunlight, photodamage, as well as trauma and pressure,
may play a role in the pathogenesis.
This case, a relatively young woman without
atrophic scar formation, could be a variant of Brunsting-Perry type of
localized BP or localized epidermolysis bullosa acquisita (EBA). Because
of the variety of clinical features, immunofluorescence patterns, and
electron microscopic findings, the localized type of BP seems to be a
heterogeneous disease, and localized EBA could be a clinical feature of
BP [10, 11]. Although autoantigens in most autoimmune bullous diseases
have recently been identified, differences of molecular pathogenesis among
variants of BP and EBA have not been clarified yet. The precise mechanism
which determines the unique clinical phenotype should be elucidated in
further investigation.
Article accepted on 22/5/01
CONCLUSION
REFERENCES
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cases with chronic, scarring, herpetiform plaques about the head and neck.
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