ARTICLE
Lichen sclerosus et atrophicus (LSA) was first reported by Hallopeau
[1] in 1889. As noted in the extensive review by Meffert et al.
[2], the characteristic clinical feature of LSA is white polygonal papules
which coalesce to form a discrete plaque. The histological features consist
of hyperkeratosis with keratotic plugs of the orifices of the hair follicles
and dermal appendages, vacuolar degeneration of the basal layer and edema
or sclerosis of the papillary dermis. Such changes in the basal layer
eventually result in the loss of melanin and bulla formation. Lesions
are most often observed on women's genitals followed by the neck and shoulders
[2]. Less common presentations include infraorbital lesions, scarring
alopecia and palm and/or sole lesions [2]. LSA usually shows a solitary
form, and extensive lesions have rarely been reported [3, 4]. We report
a case of LSA with unilateral linear lesions on the right extremities
and a whitish, parchment-like plaque on the right abdomen.
Case report
Our patient was a healthy 23-year-old woman, who had started to develop
asymptomatic, whitish skin lesions from her right abdomen to the right
leg at the age of 18 years. During the previous year, similar lesions
had appeared on her right axillary area and upper arm and the right hip.
The patient noted that the skin lesions were initially only erythematous
but later became increasingly thinner, parchment-like and finally whitish.
Past medical history and family history were not remarkable.
Physical examination revealed linear skin lesions
on her right extremities and patchy lesions on the right abdomen. The
eruption on the right axilla was an atrophic hypo- or hyper-pigmented
large macule margined by parchment-like leukoderma. Patches of leukoderma
up to 5 mm in size also extended to the flexsor aspect of the right arm.
Abdominal skin lesions showed multiple, similar small whitish macules,
which coalesced to form large parchment-like leukoderma with an irregular
configuration (Fig. 1a).
Similar patches of leukoderma were linearly-arranged on the right lower
extremity which extended from the right hip to the lower leg in an "S-shaped"
pattern (Fig. 1b).
Histopathological examinations of the abdominal and thigh eruptions
(Fig. 2) revealed mild
hyperkeratosis with plugs, atrophy of the epidermis with hydropic degeneration
of basal cells and edema of the upper dermis. The dermal papillae were
flattened and the connective tissue was poorly stained with hematoxylin-eosin
stain. A small number of lymphocytes were observed in the upper dermis.
Laboratory examinations revealed elevated serum levels of ASAT (44 IU/L),
ALAT (71 IU/L), gamma-globulin (20.2%) and IgG (1,940 mg/dl). High levels
of rheumatoid factor (158.1 IU/ml) and uncertain positive antinuclear
antibody were observed. However, anti-DNA antibody, anti-SS-A antibody,
anti-SS-B antibody, anti-RNP antibody and anti-Scl-70 antibody were negative.
Discussion
The diagnosis of LSA in our case was established by the following characteristic
histological features; atrophy of the epidermis with plugs and hydrophic
degeneration of basal cells, and the clinical features of LSA; atrophic
whitish macules. The clinical features of our case were the unilateral
and linear distribution of the lesions, which involved half the body;
right arm, abdomen and lower extremities. Such a unilateral distribution
has been reported in linear scleroderma, a variant of localized scleroderma,
but not in LSA. However Izumi et al. [5] reported a case of LSA
presenting as a linear lesion on the left arm and Libow and Coots [6]
recently reported a case having the lesion on left lower side of the trunk.
It is not considered to be difficult to differentiate these disorders.
The histopathology of old lesions of LSA may resemble localized scleroderma
[7], while the histological changes in localized scleroderma have been
reported to be completely different from those of developing LSA [8].
In addition, there has been occasional coexistence of LSA and scleroderma.
Wallace [9] found that 13 out of 380 patients with LSA had morphea, 6
of which were of the generalized type. Uitto et al. [10] reported
ten patients who exhibited clinical features of coexisting morphea and
LSA, 7 of which had the same lesions with clinical and histological evidence
of the two conditions. Connelly et al. [11] also noted a female
patient with coexistent LSA and generalized morphea. Otherwise, coexistent
lesions may be considered coincidental [12]. These previous reports have
discussed the relationship between macular types of scleroderma and LSA,
and suggested that the disorders represent either end of a spectrum or
occur as similar disease processes. Izumi et al. [5] and Libow
and Coots [6] reported a case of the linear type of LSA. Their cases and
our case indicated that LSA should be differentiated from the linear type
as well as macular type of localized scleroderma.
The mechanisms of the unilateral linear arrangement
in our case remains unknown. A variety of skin diseases are well known
to occur in linear streaks including lichen striatus, lichen planus and
porokeratosis. The distribution of some lesions may be related to the
distribution of blood vessels, lymphatics or peripheral nerves. The Koebner
phenomenon is also important in the localized appearance in some linear
skin diseases such as verruca plana juvenilis and psoriasis. The phenomenon
also contributes to LSA [2]. Genital and extragenital operations, trauma,
friction and solar radiation are known stimuli of the isomorphic phenomenon.
However, considering the distribution pattern and no previous smimuli
on her right extremities, none of these satisfactorily explain the unilateral
linear localization in our case. Many acquired as well as inherited or
nevoid skin disorders, such as lichen striatus and incontinentia pigmenti
show the linear distribution of the lesions along Blaschko's lines [13].
Our case exhibited an "S-shaped" distribution on her extremities which
appeared to follow Blaschko's lines. Libow and Coots [6] recently reported
a case of linear LSA with such a distribution on the left lower trunk.
Linear scleroderma was also listed in acquired skin diseases that follow
Blaschko's lines [13] although Bolognia et al. [14] disputed the
idea. As the unilateral linear form of LSA is very rare, the question
as to whether such a case of LSA is related to Blaschko's lines may be
answered by further study.
REFERENCES
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lesions and extensive involvement. Arch Dermatol Syphilol 1947;
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sclerosus et atrophicus. Br J Dermatol 1975; 93: 215-7.
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J Dermatol (Tokyo) 1995; 22: 279-82.
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J Am Acad Dermatol 1998: 831-3.
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10. Uitto J, Santa-Cruz DJ, Bauer EA, Eisen AZ. Morphea and lichen sclerosus
et atrophicus. J Am Acad Dermatol 1980; 3: 271-9.
11. Connelly MG, Winkelmann RK. Coexistence of lichen sclerosus, morphea,
and lichen planus. J Am Acad Dermatol 1985; 12: 844-51.
12. Rahbari H. Histochemical differentiation of localized morphea-scleroderma
and lichen sclerosus et atrophicus. J Cutan Pathol 1989; 16: 342-7.
13. Jackson R. Blaschko's lines: a review and reconsideration of observations
on the cause of certain unusual linear conditions of the skin. Br J
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14. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad
Dermatol 1994; 31: 157-90
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