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: sharon.wong@hotmail.co.uk

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.

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Wong et al.