ARTICLE
Case 1
A 31-year-old Japanese male presented with a one year history of asymptomatic
erythema on the right cheek. The lesion first appeared on the right cheek
and, 6 months later, a second lesion developed below the right eye. There
was no history of trauma. He was a carpenter and had experienced frequent
sun exposure. Physical examination revealed two areas of erythema on the
right infraorbital area and the central part of the cheek. The erythema
was slightly elevated and scaled (Fig.
1).
Case 2
A 36-year-old Japanese housewife presented with a one month history
of an asymptomatic eruption on the left cheek. She had been anxious about
the lesion and had therefore covered the lesions with cosmetics every
day. There was no history of trauma or frequent sun exposure. Physical
examination revealed an area of erythema on the left cheek. The erythema
was well-demarcated, depressed and scaled (Fig.
2). Since trauma or contact dermatitis was suspected, we told
her not to use cosmetics. Over the six week follow up period, the center
became ulcerated.
Discoid lupus erythematosus
with an unusual clinical appearance mimicking sporotrichosis
Discoid lupus erythematosus (DLE) usually develops on sun-exposed areas
and appears as erythematous patches of various sizes with adherent scale,
follicular plug, telangiectasia and sometimes with scarring. In the clinical
setting, there have been some cases of DLE presenting with unusual clinical
appearances. In such cases, the definitive diagnosis is difficult without
histopathological examination. In the present two cases, our initial diagnosis
was sporotrichosis.
In both cases, the results of routine laboratory tests were within normal
limits. Antinuclear antibody was positive (1:40) with a homogeneous-speckled
pattern, and other antibodies (anti-SS-A, SS-B, Sm, nRNP, Scl-70 and ds-DNA
antibodies) were all negative in both cases. Photosensitivity was clinically
unremarkable. In case 2, the results of patch tests with the cosmetics
were all negative. Skin biopsy was performed in both cases. Histological
examination revealed ortho-hyperkeratosis with keratotic plugging and
liquefaction degeneration. In the dermis, there were dense mononuclear
cell infiltrates around perivascular and periadnexal areas (Fig.
3). Direct immunofluorescence tests demonstrated a granular deposition
of IgG at the basement membrane zone in both cases. These findings were
considered to be consistent with a diagnosis of DLE.
Discussion
The common clinical features of DLE are a well-mariginated, round or
oval erythematous plaque with adherent scale and telangiectasia on sun-exposed
areas. DLE, however, sometimes has an unusual presentation, such as acne
vulgaris [1] and Melkersson-Rosenthal Syndrome [2]. Recently, we reported
linear cutaneous lupus erythematosus following Blaschko's lines [3]. In
the present cases, the initial diagnosis was sporotrichosis and the diagnosis
of DLE was not established until histological examination was performed.
The commonest type of sporotrichosis is the localized lymphatic variety
which usually develops on sun-exposed areas. The clinical characteristics
are a well-mariginated, round or oval erythematous plaque with or without
pustules, adherent scales and small ulcers.
The common histopathological features include small granulomatous reaction
with epitheloid cells and multinucleated giant cells, and nonspecific
inflammatory infiltrate composed of neutrophils, lymphocytes, plasma cells
and histiocytes.
Uitto et al. [4] reported seven cases
of DLE with hyperkeratotic lesions resembling keratoacanthoma, verruca
or lichen planus. However, these patients had atypical lesions mainly
on the upper extremities along with typical DLE on the face or scalp.
The histopathological examination of the lesions of the upper extremities
showed a lichenoid tissue reaction (LTR). It was speculated that LTR might
play a key role in the development of atypical lesions. The most significant
histopathological change in DLE is liquefaction degeneration characterized
by vacuolar spaces beneath and between basal keratinocytes [5]. In the
active lesions, mononuclear infiltrates can be seen at the dermo-epidermal
junction along with the dermal change of dense mononuclear infiltrates
around perivascular and periadnexal areas. This dermal change is distinguished
from LTR, as LTR is characterized by a band-like lymphocytic infiltration
beneath the epidermis [6].
Reviewing the present cases, the unusual appearance might have been
induced by certain aggravating factors. It is well known that ultraviolet
light sometimes elicits or aggravates DLE [7, 8]. Some authors have stated
that ultraviolet light triggers the release or the translocation of sequestered
nuclear antigen such as anti-SS-A, SS-B or ds-DNA [9-11]. We were not
able to gain the patients' consent for photosensitivity testing. Patient
1 was an outdoor worker (carpenter), however, no extractable nuclear antibodies
including anti-SS-A antibody were revealed. Although we could find no
correlation between ultraviolet light and disease development in this
case, the patient had been free from DLE by using a sunscreen. On the
other hand, patient 2 was a housewife and did not have the habit of staying
outdoors for long periods of time. She had been so anxious about the lesion
that she had covered the lesion with cosmetics every day. She stated that
her lesion had become worse using cosmetics.
The negative results of patch testing on the uninvolved skin can not
exclude the possibility of primary irritant contact dermatitis. We speculate
that the cosmetics caused irritation by penetrating the inflamed epidermis.
CONCLUSION
Some aggravating factors may modify the clinical appearance of DLE, resulting
in an unusual presentation. Thus, when we encounter a patient with an
unusual lesion on the face, it is advisable to perform a skin biopsy to
ensure a definite diagnosis and thus the most effective treatment.
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