Download Purplish Papules and Plaques with Blisters on Lower Lip

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Resident Forum
Purplish Papules and Plaques with Blisters on Lower Lip
Yu-Ting Huang1 Woan-Ruoh Lee2 Chung-Hong Hu1
CASE REPORT
A 47-year-old woman came to our dermatology clinic for longstanding erosive and crusting vesicles on her lower lip. The erosions began three years earlier and responded poorly to topical steroids.
Dermatologic examination revealed rupturing vesicles arising from violaceous polygonal plaques over
her lower lip with erosions, blood clots and crust formations. The lesions bled easily while we pulled
the lower lip down for recording photos (Fig.1a). Other parts of the oral mucosa were intact and the
skin had no similar lesions.
A skin biopsy taken from the edge of the vesicle and the underlying plaque over the lower lip
showed a subepidermal blister, vacuolization of the basal cell layer, and Civatte bodies. (Fig. 2 and Fig. 3)
Fig.1
(a) Ruptured blisters leaving erosions and crusts formations (black arrow) and
overlying purplish polygonal plaques (whitearrow) on the lower lip.
(b) 2 weeks after topical tacrolimus therapy, the erosions healed. Only vesicles
(black arrows) and underlying purplish plaques (white arrow) were noted.
(c) 1 year after topical tacrolimus treatment, residual tiny vesicles (black arrow)
locating on unapparent whitish scar over central lower lip.
(d). The residual smaller papules (white arrow) on right lower lip after 1 year
topical tacrolimus treatment.
Fig. 2
Fig. 3
The dermoepidermal junction separates and forms subepidermal blister. Lichenoid interface reaction and melanin
incontinence (black arrow) are noted. (H & E, x40)
Vacuolization of the basal cell layer. Civatte bodies in lower
epidermis and papillary dermis. Band-like lymphocyte infiltrates along dermoepidermal junction. (H & E, x200)
From the Department of Dermatology, Taipei Medical University- Taipei Municipal Wan-Fang Hospital,1 and Taipei Medical
University Hospital2
Accepted for publication: March 03, 2006
Reprint requests: Chung-Hong Hu, Department of Dermatology, Taipei Medical University- Taipei Municipal Wan-Fang Hospital,
No.111, Sec. 3, Xing-Long Rd, Taipei 116, Taiwan
TEL: 886-2-29307930 ext. 2901-2902
Dermatol Sinica, June 2006
224
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DIAGNOSIS
Bullous Lichen Planus on Lower Lip
DISCUSSION
Lichen planus (LP) is a chronic mucocutaneous disease of unknown causes that is relative
commonly seen in the dermatological clinics.1
LP lesions often affect flexions, lower limbs,
genitalia and oral mucosa.1 By comparison, the
incidence of bullous lichen planus (BLP) is
much lower.1, 2 Reviewing the literature, the cases of BLP that have been reported were most
often involving lower limbs and trunk.1 BLP on
oral mucosa is rarely seen.
The oral BLP are commonly seen on the
buccal mucosa and less commonly on gingival
and inner aspect of the lips.2 The bullae are generally short-lived and leave ulcerative lesions on
rupturing.2 The lacking of underlying polygonal
violaceous plaques of lichen planus delimits
pemphigus, cicatricial pemphigoid and other
mimicking disease from BLP. More then clinical appearances pathology findings also offer
striking values for diagnosis.
The histopathologic hallmarks of BLP are
subepidermal blisters along with typical findings of LP.3 It should be distinguished from other subepidermal bullous disorders such as bullous pemphigoid (BP) and lichen planus pemphigoides (LPP), an entity of co-existing of LP
and BP.3 BP typically contains eosinophils, neutrophils and predominant lymphocytes; relative
normal epidermis and absence of the dense
band-like infiltration along dermo-epidermal
junction.3 LPP possesses histopathologic features of BP and LP.3 Immunopathology provides
additional clues. Direct immunofluorescence
(DIF) tests show linear deposition of C3 or
immunoglobulins along basement membrane
zone in BP and LPP, but usually not in BLP.3
The causes of BLP are not well known.
Immunologic processes, exogenous chemical
substances, and physical agents are all considered
to play roles in the pathogenesis of BLP. These
events are supposed to trigger the destruction of
the basal keratinocytes through host immune
responses to the wide range of various antigens.4
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The Langerhans cells presumably act as
antigen presenting cells which process the antigens and present antigens to the lymphocytes
which in turn destroy keratinocytes causing
cytolysis.4 It then follows that theņsubepidermal
cleftsŇ
(Max-Joseph spaces) results from the
cytolysis of increasing numbers of cells along
the basal layer of the epidermis.4 With further
extremely destruction course, the clinically rarely
seen blisters of BLP eventually developed.4
The treatment modalities of OLP include
topical corticosteroids, topical treinoin, topical
cyclosporine, intralesional corticosteroids, systemic corticosteroids, retinoids, antimalarials,
griseofulvin, dapsone, oral PUVA, and surgical
techniques.5 Recently, topical tacrolimus has
been reported showing promising effects for
OLP.5 Our case is the first reported case of oral
BLP that has been effectively treated with topical
tacrolimus in Taiwan. (Fig. 1b-d) Tacrolimus
inhibits calcineurin and thus interferes with the
synthesis of cytokines such as IL-2, IL-3, IL-4,
IL-12, TNF and INF-ə
.5 Tacrolimus also inhibits
5
T-cell proliferation, and we suppose this may
prevent further destruction of keratinocytes by
infiltrated lymphocytes in BLP lesions because
of the recruitment of cytotoxic lymphocytes is
disrupted.
REFERENCES
1. Daoud MS, Pittelkow MR: Lichen planus. In:
Freedberg IM, Eisen AZ, Wolff K, et al., eds.
Dermatology in General Medicine. 6th ed. New
York: McGraw-Hill, 463-477, 2003.
2. Unsal B, Gultenkin SE, Bal E, et al.: Bullous oral
lichen planus: report of two cases. Chin Med J
116: 1594-1595, 2003.
3. Gawkrodger DJ, Stavropoulos PG, Mclaren KM,
et al.: Bullous lichen planus and lichen planus
pemphigoides---clinicopathological comparisons.
Clin Exp Dermatol 14: 150-153, 1989.
4. Smoller BR, Glusac EJ: Immunofluorescent analysis of the basement membrane zone in lichen
planus suggests destruction of the lamina lucida in
bullous lesions. J Cutan Pathol 21: 123-128, 1994.
5. Rozycki TW, Rogers III RS, Pittelkow MR, et al.:
Topical tacrolimus in the treatment of symptomatic oral lichen planus: a series of 13 patients. J
Am Acad Dermatol 46: 27-34, 2002.
Dermatol Sinica, Sep 2006