Bullous eruption on sun exposed area : bullous pemphigoid ?

Michelle , Brahm U Pendit , Pristia

Department of Dermatology and Venerology, Gatot Soebroto Army Hospital

Abstract : A 53 year old woman came to our hospital with bullous eruption on sun
exposed area, predominantly on her face. Biopsy of the lesion was taken, which
revealed subepidermal blistering. Treatment with oral corticosteroids were
satisfactory, with no new blisters emerged. After the possibility of photodermatosis
and other bullous disease excluded, the diagnosis of bullous pemphigoid was made
by histological examination and thorough history taking.

Introduction : Bullous pemphigoid is the most common autoimmune blistering

disorder in the adult population. It is by its very nature a polymorphic disease. Several
atypical forms of bullous pemphigoid has been reported by several authors. All of
them have a classical histology and immunofluorescence of bullous pemphigoid.
Eruption of bullous pemphigoid in sun exposed area, predominantly the face is not
well documented, therefore the need to recognize this atypical variation of bullous
pemphigoid is important for dermatologist to avoid misdiagnosis.

Case : A 53 year old woman had noticed blistering on her face that became
increasingly severe over a period of 10 months and spread over the ears. The
condition was accompanied by itching. Examination of the skin revealed that only sun
exposed areas that are not covered by clothes were affected (the patient wears hijab
and clothes covering all body areas except her face). A sharply demarcated, minimally
crusted, erythematous plaque on both cheeks, nose, and earlobes with non inflamed
areas of hypopigmentation and scarring marking the sites of prior lesions that have
resolved were observed. The lesion is pruritic. Nikolski sign is negative. The patient
observed that exposure to sunlight is sometimes painful, but does not led to
deterioration. She didn’t experience any systemic manifestation such as malaise,
fever, myalgia, and arthralgia. A tissue biopsy was taken, and the result is a
subepidermal blister with neutrophils, and perivascular inflammatory cell infiltrate
with eosinophils. Results for her blood work is normal, except for a low TSH. She
was diagnosed with hyperthyroidisim 5 years ago and was treated with
propylthiouracyl and propanolol for 3 years, and had stopped the medication ever
since. She was not on other any medication after since. A 2 weeks initial treatment
with methylprednisolone was given, and the result is satisfactory. No new bullae
emerged, and pruritus is minimal.

Discussion : The skin lesions of our patient corresponded most likely to bullous
pemphigoid. The fact that blistering was strictly limited to sun-exposed areas,
especially the face, is unusual. As a differential diagnosis, a phototoxic or
photoallergic reaction was also considered. The result of the histological
examination, however, was not compatible with a phototoxic or photoallergic reaction
: there were no epidermal spongiosis, dermal edema and enlargement of endothelial
cells typical sunburn cells (vacuolated keratinocytes) were missing; and blister
formation was subepidermal instead of spongiotic intraepidermal blisters as might be
expected. Porphyria was also excluded by histological results.A normal routine blood
work, CRP, liver function, kidney function test shows no indication of internal
malignancy, thus no evidence for paraneoplastic bullous pemphigoid. The patient
experienced no systemic manifestation such as malaise, fever, myalgia, and arthralgia
therefor decreasing the possibility of cutaneous manifestation of systemic
autoimmune disease such as bullous lupus erythematosus. Based on the typical
histological changes-subepidermal blister with eosinophils and perivascular
inflammatory cell infiltrate with eosinophils, we established the diagnosis of bullous
pemphigoid. The strict limitation of the blisters to the face draws attention to the role
of light as the provocation factor. UV light has been recognized to be a physical factor
triggering bullous pemphigoid in few case reports. Treatment with oral corticosteroid
has led to a suppression of the disease.

Conclusion : The patient gave a good response with methylprednisolone 12mg-

12mg-0/24 hours, which was tapered gradually every two weeks. This case shows
adequate systemic corticosteroid administration with appropriate tapering down can
control the extensive course of bullous pemphigoid disease. The exact role of UV
light in precipitating bullous lesions remain to be clarified in future studies.

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