ARTICLE
Sarcoidosis is a systemic disease of which the cause and pathogenesis
are still unknown. The sites most commonly involved are the skin, lymph
nodes, lungs, eyes, and heart. The frequency of skin involvement of sarcoidosis
is considered to be 17-30% of all cases [1], but the prevalence of a particular
type of cutaneous lesion varies among races as well as individual cases.
Cutaneous involvement is divided into specific and nonspecific categories
[2, 3]. Specific lesions include nodules, plaques, lupus pernio, subcutaneous
nodules, and other rare manifestations. Lichenoid papules are one of the
rare types of cutaneous sarcoidosis, and are characterized by pinhead-sized
yellowish lesions closely grouped in round or oval clusters with slight
scaling [4].
We report here a case of the lichenoid type of cutaneous sarcoidosis
with a unique clinical course; the follicular cutaneous lesions were first
distributed all over the body, spontaneously regressed, and then relapsed,
especially in the intertriginous areas of the trunk, with occurrence of
uveitis.
Case report
A 62-year-old Japanese woman was seen at Kansai Medical University Hospital
with a two-month history of asymptomatic miliary papules all over the
body. She did not complain of any systemic symptoms such as fever, arthralgia,
or fatigue. There was no family history of sarcoidosis. Physical examination
revealed miliary follicular papules on the trunk and extremities (Fig.
1). They did not coalesce to form plaques. No eruptions were present
on the palms or soles. The histopathological findings showed circumscribed
granulomas of epithelioid cells in the perifollicular areas (Fig.
2). An admixture of lymphocytes was present at the margins of
the granulomas. Caseation necrosis was not seen and microorganisms were
not identified by PAS and Ziehl-Neelsen stainings. The results of polarization
microscopy were negative. Cultures of skin biopsy material were negative
for acid-fast bacilli and deep fungi. There were no abnormalities of the
lymph nodes or nerve cords. An intradermal skin test to purified protein
derivative (PPD) was negative. Complete blood cell count, hemoglobin,
and hematocrit were within normal limits. The serum chemistry was unremarkable
with the exception of mild elevation of lactate dehydrogenase and glutamic
oxaloacetic transaminase. Serum angiotensin converting enzyme level and
calcium level were normal at 10.6 IU/ml (normal, 8.3-21.4) and 4.7 mEq/l
(4.0-5.0), respectively. Chest X-ray film, computed tomographic (CT) scan,
and Ga scintigraphy showed no other organ involvement. Electrocardiogram,
pulmonary function and arterial blood gas levels were unremarkable. At
this time, a diagnosis of systemic sarcoidosis was not established. Then
the cutaneous lesions regressed without any treatment within 1 year. Two
years later, clouded vision of the right eye appeared, and uveitis due
to ocular sarcoidosis was diagnosed in the ophthalmological department
of our hospital. The micropapular eruptions relapsed when the ocular symptoms
appeared. The skin lesions were distributed in the intertriginous areas
of trunk such as the submammary areas and in fatty folds of the abdomen
(Fig. 3A, B) and extremities.
A skin biopsy specimen confirmed the recurrence of perifollicular sarcoidal
granulomas. Ga scintigraphy demonstrated accumulation in the right ophthalmological
region. The patient was treated with twice-daily applications of fluocinonide
ointment, and the skin lesions gradually resolved. The ophthalmological
involvement has not progressed with the use of prednisone eye drops. We
are now investigating the possibility of late appearance of other systemic
involvement.
Discussion
The lichenoid type of skin lesions is a rare manifestation of sarcoidosis,
and is estimated to constitute 1-2% of all cases of skin sarcoidosis [5].
In a review of the literature with English-language abstracts, we found
only 3 cases of sarcoidosis reported as lichenoid-type [6-8]. The lichenoid
type of cutaneous sarcoidosis consists of miliary papules distributed
on generalized or localized areas. Histologically, the lesions involve
small epithelioid cell granulomas present in the upper dermis. In some
cases, the granulomas are localized in the perifollicular areas.
The present case has two unique clinical features. First is the recurrence
of lichenoid-type skin lesions. The spontaneous resolution of lichenoid
lesions of cutaneous sarcoidosis has been reported [7, 8]. However, there
have been no reported cases involving recurrence of such lesions. Also
relapsed cases of any type of cutaneous sarcoidosis are considered to
be rare. Few cases of recurrent cutaneous sarcoidosis have been reported
in the English and Japanese literature. Kishimoto et al. [9] reported
a case of sarcoidosis recurring twice while the patient was pregnant.
Gogstetter and Goldsmith [10] described a case of recurrent nodular cutaneous
sarcoidosis in an immunodeficient patient. The present case did not have
such a trigger or other systemic disorders. Yokogawa et al. [11]
and Roegel et al. [12] also reported relapsed cases of cutaneous
lesions of sarcodosis without any complications.
Second is the distribution of skin lesions. Eruptions in our case relapsed
in the intertriginous areas of the trunk. The reason why the eruptions
appeared in these areas is unknown. One possibility may be an association
of the Koebner phenomenon with physical factors such as friction and pressure
of skin in the body folds. Tanabe et al. [13] described cutaneous
sarcoidal lesions occurring by the Koebner phenomenon. Alternatively,
an occlusive condition may enhance the absorption of unknown pathogens
or a causative agent through the hair follicles. An association of hair
follicles and sarcoidosis has been suggested by the reported cases of
follicular sarcoidal lesions. Gange et al. [14] reported two cases
involving large numbers of small papules arising in an eruptive fashion
that showed histopathologically sarcoidal granulomas closely related to
the pilosebaceous follicles of our case. Georgouras [15] described childhood
cases of sarcoidosis with micropapular eruptions in the perioral areas
and suggested that certain ingredients of bubble gum may be absorbed by
follicles and hence produce a perifollicular reaction of a granulomatous
nature. With regard to pathogens related to pilosebaceous follicles, propionibacterium
acnes is suspected to be a causative agent of sarcoidosis in Japan
[16].
Serum level of angiotensin converting enzyme has been used as an important
laboratory test in sarcoidosis since a 1975 report by Lieberman [17].
However, Bunting et al. [18] reported that increased serum ACE
level is not specific for sarcoidosis because the sensitivity and specificity
were 77% and 93%, respectively. In our case, even when the skin lesions
were generalized on the body, the serum ACE level was normal, indicating
that the level is not associated with the disease activity. The volume
of sarcoidal granulomas in the lichenoid type of sarcoidosis may be too
small to produce a high enough level of ACE to be detected as an elevated
serum level.
Article accepted on 03/2/00
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