seasonal cutaneous sarcoidosis: a photo-induced variant
TRANSCRIPT
B R I E F C O M M U N I C A T I O N
Seasonal cutaneous sarcoidosis: a photo-induced variantSharon Wong1, Claire Pearce2, Dorota Markiewicz3 & Anshoo Sahota1
1Department of Dermatology, Whipps Cross University Hospital, London, UK, 2Barts and The London NHS Trust, London, UK, and3Histopathology Department, Whipps Cross University Hospital, London, UK
Key words:photo-induced; sarcoidosis; seasonal
Correspondence:Dr Sharon Wong, Department of Dermatology,
Whipps Cross University Hospital, Whipps Cross
Road, Leytonstone, London E11 1NR, UK.
e-mail: [email protected]
Accepted for publication:24 January 2011
Conflicts of interest:None declared.
Summary
Sarcoidosis is a multisystem granulomatous disease, with cutaneous involvement in up to
35% of cases. Owing to its heterogeneous clinical presentation, sarcoidosis is often referred
to as the ‘great imitator’ of dermatological disease. A rare variant of photosensitive cutaneous
sarcoidosis has been infrequently reported in the literature. We describe an unusual case of
recurrent, photo-distributed cutaneous sarcoidosis presenting only during the summer
months.
The seasonal nature of sarcoidosis is recognized in some
forms of clinical disease, such as sarcoidal arthritis and
Lofgren syndrome (1), in which disease flares have been noted
during the spring and summer months. We report an unusual
case of seasonal cutaneous sarcoidosis, in a patient presenting
with recurrent, photosensitive lesions.
Case report
A 58-year-old Filipino woman was diagnosed with micropapular
cutaneous sarcoidosis following a skin biopsy and positive Kveim
test 36 years previously. At the time of diagnosis, the sarcoidosis
was asymptomatic, confined to the skin and limited to a focal
area on her back, which resolved completely with potent topical
steroids. She re-presented 29 years later having noted a consistent
photo-induced eruption in the preceding 6 years. This typically
appeared as an asymptomatic, skin-coloured, micropapular
eruption across her upper chest and arms (Fig. 1) with similar
changes on her upper back and legs. Sparing of the hands, face
and skin creases was noted. The rash completely resolved during
the winter, and recurred in the same distribution every summer.
The flares were particularly florid on returning to the Philippines
during the warmer, dry season. She had also developed gritty,
sore eyes on several occasions and was diagnosed with uveitis
following an ophthalmology review. Past medical history
included thyrotoxicosis treated with carbimazole, which was
started shortly after her original diagnosis of sarcoidosis, and
atopic dermatitis, with known allergy to pollens. Differential
diagnoses considered at this time included polymorphic light
eruption (PLE), follicular eczema, drug eruption and photo-
induced sarcoidosis.
Investigations
Routine bloods including full blood count, urea and electrolytes,
liver function tests and bone profile were unremarkable. Serum
angiotensin-converting enzyme, lupus serology and chest
X-ray were also normal. A subsequent skin biopsy demonstrated
non-caseating granulomas with a sparse lymphocytic infiltrate
(Fig. 2). Special stains for microorganisms were negative, and
the clinico-pathological diagnosis was consistent with photo-
induced sarcoidosis.
Progress
Treatment with hydroxychloroquine and doxycycline as tried on
separate occasions, but discontinued after a few doses due to
nausea and the development of a rash, respectively. Methotrexate
was considered but due to the patients long and frequent visits to
the Philippines, safe prescribing and monitoring were not
possible. As the rash was asymptomatic and episodic, the patient
opted for conservative management with photo-protection only.
Discussion
Cutaneous manifestations of sarcoidosis may be classified as non-
specific, such as erythema nodosum, and specific, which include
r 2011 John Wiley & Sons A/S � Photodermatology, Photoimmunology & Photomedicine 27, 156–158156
papular eruptions, subcutaneous nodules, lupus pernio and
lichenoid lesions. Papular sarcoidosis is the commonest pattern
observed. It is particularly prevalent in African–American
women, and most frequently affects the facial skin. However, an
unusual variant of photosensitive cutaneous sarcoidosis has been
described and is becoming increasingly recognized as a rare, but
distinct entity (2,3).
The preferential distribution of cutaneous sarcoidosis in
photo-exposed sites has been reported in only three cases in the
recent literature. In these cases, the patients developed a photo-
distributed, papular eruption, which became erythematous and
pruritic with sun exposure. The diagnosis of sarcoidosis was
supported histologically and all three patients responded well to
hydroxychloroquine and/or corticosteroids (2,3). In contrast to
the previous cases reported, our patients’ skin eruption was
completely asymptomatic.
Although phototesting was not performed in our patient, it is of
interest that the case described by Truchot et al. (3) had a normal
UVB MED and the UVB iterative phototest (20 MEDs) was negative.
Of the other differential diagnoses, PLE and eczema were considered
unlikely owing to the absence of pruritus, and with a latency period
of over 20 years, a drug rash secondary to carbimazole was also
considered unlikely. In addition, these differential diagnoses were
not supported histologically. The features in favour of sarcoidosis
included two histological specimens (36 years apart) with
granulomatous inflammation, including the most recent with
‘naked granulomas’.
Seasonal variation, as seen in our patient, is not confined to
cutaneous sarcoidosis. The same pattern of disease flare has also
been documented for sarcoidal arthritis and Lofgren syndrome (1),
an acute presentation of sarcoidosis with bi-hilar lymphadenopathy,
fever, erythema nodosum and arthritis. One study showed a peak
presentation of Lofgren syndrome 148% above average in May
compared with 72% below average in November and January (1).
The aetiopathogenesis of cutaneous sarcoidosis remains obscure.
It is thought that continued exposure of a genetically susceptible
individual to an antigenic stimulus through the epidermis,
produces a chronic immunological response and granulomatous
inflammation. Infectious agents and environmental factors such as
pollen have been suggested, but not proven as possible antigens.
While both pollen exposure and infection could account for
seasonality in sarcoidosis, neither would adequately explain the
photo-distribution of lesions observed in this variant of cutaneous
sarcoidosis. Exposure to ultraviolet radiation (UVR) appears to be
the most plausible causative factor. Although the pathomechanism
is not known, the observation of an increased number of dendritic
cells in the epidermis of sarcoidal lesions, compared with a reduced
number in solar-irradiated skin may be relevant (4). As dendritic
cells are the primary antigen-presenting cells in the skin, this
observation would explain the absence of sarcoidal lesions in areas
consistently exposed to UVR, such as the face and dorsum of the
hands, compared with areas that are only uncovered in warmer
weather. The sparing of frequently photo-exposed areas is
commonly seen in other photodermatoses such as PLE.
While this may account for the selective sparing of sarcoidosis in
certain sites, there is clearly a complex, conflicting and currently
unexplained interaction between UVR and cutaneous granulomatous
inflammation of the skin in general. Historically, phototherapy has
been, and still is used to treat a number of granulomatous disorders
such as lupus vulgaris and generalized granuloma annulare (GA),
respectively. Paradoxically, it has also been implicated as the cause of
actinic granuloma of O’Brien, an uncommon variant of GA, in
which solar-damaged elastic tissue is thought to act as a low-grade
antigen, triggering granuloma formation (5).
Fig. 2. Biopsy of the papules demonstrating well-formed, non-caseating
granulomas in the papillary dermis, composed of epithelioid histiocytes
and surrounded by a sparse rim of small lymphocytes (haematoxylin and
eosin, original magnification � 200).
Fig. 1. Skin-coloured micropapular lesions in a photo-sensitive
distribution over the neck, upper chest and upper arms. Note the sparing
of skin creases.
157r 2011 John Wiley & Sons A/S � Photodermatology, Photoimmunology & Photomedicine 27, 156–158
Seasonal cutaneous sarcoidosis
Our case of photosensitive cutaneous sarcoidosis is a rare,
but increasingly recognized, variant of a relatively common
dermatosis. The pathogenesis of photodermatoses in general
and photosensitive cutaneous sarcoidosis remains unknown.
References
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