Dermatologica Sinica

: 2021  |  Volume : 39  |  Issue : 1  |  Page : 61--62

Erythrodermic bullous pemphigoid

Mai Endo, Yukina Watanabe, Miyuki Yamamoto, Shohei Igari, Nobuyuki Kikuchi, Toshiyuki Yamamoto 
 Department of Dermatology, Fukushima Medical University, Fukushima, Japan

Correspondence Address:
Dr. Mai Endo
Department of Dermatology, Fukushima Medical University, Hikarigaoka 1, Fukushima 960-1295

How to cite this article:
Endo M, Watanabe Y, Yamamoto M, Igari S, Kikuchi N, Yamamoto T. Erythrodermic bullous pemphigoid.Dermatol Sin 2021;39:61-62

How to cite this URL:
Endo M, Watanabe Y, Yamamoto M, Igari S, Kikuchi N, Yamamoto T. Erythrodermic bullous pemphigoid. Dermatol Sin [serial online] 2021 [cited 2021 May 7 ];39:61-62
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Full Text

Dear Editor,

Erythroderma results from various inflammatory diseases including uncontrolled eczema, atopic dermatitis, psoriasis, drug allergy, cutaneous T-cell lymphoma, and internal malignancy; however, bullous pemphigoid (BP) rarely exhibits erythrodermic condition. Herein, we describe a case of BP presenting with erythroderma.

An 83-year-old female visited a nearby hospital, complaining of itchy erythema and bullae on the trunk and extremities, which appeared 1 week previously. She was diagnosed as having BP on histopathological and serological examination. Oral prednisolone (40 mg/day) was administered but was ineffective, and thus she was referred to our hospital. No new drugs were administered within these few years. Physical examination showed tense blisters, erosions, and crusts within the coalesced erythemas involving the trunk, extremities, and face [Figure 1]a. Mucous membrane was not involved. More than 90% of the body surface area was covered with erythema. Laboratory tests showed an increased number of eosinophils (10%) in the peripheral blood, and serum titer of anti-BP180 NC16A IgG antibody measured by enzyme-linked immunosorbent assay was >10,000 U/mL (cutoff: <8.9). Serum IgE level was elevated (317 U/mL, normal <170). Kidney and liver functions were normal. A biopsy from one of the blisters revealed subepidermal blister formation with prominent eosinophil infiltration in the upper dermis [Figure 1]b. Direct immunofluorescence (DIF) showed linear deposition of C3 at the basement membrane zone [Figure 1]c. On admission, she was treated with methylprednisolone pulse therapy (1000 mg/day for consecutive 3 days) followed by oral prednisolone (40 mg/day), which led to the improvement of the skin lesions. However, during tapering of prednisolone, the skin lesions relapsed. A second course of methylprednisolone pulse therapy and intravascular immunoglobulin (IVIG) therapy, followed by oral prednisolone and azathioprine, resulted in improvement of the lesions.{Figure 1}

Erythroderma is a generalized erythema, affecting over 90% of the whole body, which can be idiopathic or secondary. BP presenting with erythroderma is rare, and only several cases of erythrodermic BP have been reported to date.[1],[2],[3],[4],[5],[6],[7] There are several types of erythrodermic BP, such that (1) bullous lesions appear along with erythroderma, (2) bullous lesions are absent and only erythroderma is present, but BP is diagnosed by positive linear deposition of IgG or C3 at the basement membrane by DIF (nonbullous BP), and (3) bullous lesions appear after the initiation of treatment of erythroderma. In cases of erythroderma without any blistering, it is difficult to make a diagnosis of BP and thus the diagnosis is often delayed. Previous reports have demonstrated some cases of erythrodermic BP, which were resistant to prednisolone and required azathioprine.[3] Our case responded to prednisolone but had a relapse and required a second course of steroid pulse therapy, IVIG, and immunosuppressive drugs.

Erythroderma and BP share the same pathogenesis, that is, Th2-shifted cytokine balance such as interleukin (IL)-4, IL-5, and IL-6 and elevated IgE levels. Th2-dominant conditions in BP may lead to erythrodermic condition. Hayakawa-Asai et al. reported a case which developed papulo-erythroderma after improvement of BP,[8] supporting a shared mechanism of Th2-dominant cytokine balance in both conditions. The present case exhibited an erythrodermic appearance at the initial visit to our department. Some potential genetic, environmental, or hormonal factors, as well as a delay in the treatment of BP, may have mainly caused erythroderma in the present case, but further study is necessary to clarify the etiology of erythrodermic condition in BP.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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