Severe bullous pemphigoid with excoriation disorder
- 1 Rowan University School of Osteopathic Medicine, Stratford, New Jersey, USA
- 2 Hackensack Meridian Ocean University Medical Center Pathology Department, Brick, New Jersey, USA
- Correspondence to Ms Kaitlin McGowan; mcgowa47@rowan.edu
Abstract
Bullous pemphigoid is the most common autoimmune blistering skin disease. Pathogenesis involves autoantibodies that attack the basement membrane, resulting in blisters and intense pruritus. We present a case of bullous pemphigoid with concurrent excoriation disorder in a woman in her 50s. The suspected diagnosis of bullous pemphigoid was confirmed through direct immunofluorescence testing on a specimen obtained via punch biopsy, then treated with vancomycin and steroids. In addition, cross tapering from duloxetine to fluoxetine was used to treat the patient’s excoriation disorder. The concurrent dermatological and psychiatric components, as well as the severity, made this case unique.
Background
Bullous pemphigoid is the most common autoimmune blistering disease, most frequently affecting elderly individuals in the eighth decade of life.1 Classic symptoms include blisters overlying urticarial plaques on the torso and extremities. Aetiology includes an abnormal T cell response that triggers production of IgG and IgE autoantibodies, which attack the hemidesmosomes of the basement membrane.2 The condition can result in intense pruritus that begins during the prodromal period.3 The case presented here describes a middle-aged woman with full body skin involvement, sparing only the face and feet, who concurrently met the Diagnostic and Statistical Manual for Mental Disorders Fifth Edition (DSM-5) criteria for excoriation (skin-picking) disorder. Excoriation disorder is related to obsessive-compulsive disorder (OCD) and is characterised by recurrent skin picking that results in lesions, repeated attempts to stop or decrease the picking, and resultant mental distress or impairment in functioning.4 5 Sufferers often feel shameful, anxious or depressed about their condition.6 No exact counts of epidemiological prevalence exist, but 3.1% of survey respondents from a convenience sample of 10 000 participants reported a history of excoriation disorder.4 According to the DSM-5, an estimated 75% of patients are female, and the disorder may be precipitated or exacerbated by an underlying dermatological condition.5 Grant and Chamberlain also note that mental health comorbidities are not uncommon—roughly 25% of individuals with a history of the disorder also report having a panic disorder, OCD, attention deficit hyperactivity disorder or post-traumatic stress disorder.4 To our knowledge, no cases of classic bullous pemphigoid with concurrent excoriation disorder of this severity or in a patient of this age have been reported in the literature.
Case presentation
A woman in her 50s presented to the emergency room for evaluation of a diffuse rash present on all four extremities, trunk, abdomen and back but sparing the upper feet and face. The patient stated that the current rash had been present for the past 4 months but that she had experienced a recurring rash intermittently for the past year. She was unable to associate the rash with a change in medication or illness. She endorsed diffuse itching and a burning pain over the affected areas. The patient described the discomfort from her skin condition as a ‘burning hurt’. At times, the pain from her skin was so severe that she could barely walk. She admitted to often feeling an uncontrollable urge to pick at her skin in response to feelings of anxiety. She denied fever, chills, weakness or recent insect bites. She had a history of type 2 diabetes, polycystic ovarian syndrome, and bipolar disorder type II but had no previous history of autoimmune disease. She also had no family history of autoimmune disease.
Her primary care provider had been managing her care for eczema using triamcinolone cream two times per day as needed. She also had a history of excoriation disorder that had been controlled with pimozide, which was discontinued after she developed tardive dyskinesia. Venlafaxine and mood stabilisers in the past had proven unsuccessful in managing her excoriation disorder. The patient had started duloxetine 60 mg daily for anxiety and depression 1 month prior, which was several months after onset of the rash.
Physical examination showed a severe rash of the lower face, neck, chest, trunk and extremities with an erythematous base accompanied by moderate diffuse excoriation and severe dryness (figures 1–4). No lesions were present on the mucosa of the oropharynx or nares. The rash consisted of both scattered clear bullae filled with serosanguinous fluid and scabs in stages of healing that ranged from crusted to actively weeping (figure 3). The bullae were tense and firm and did not slough off when pressure was applied. None of the closed lesions were tender. During the examination, the patient was observed repeatedly rubbing her hands over her excoriated arms.
Patient with bullous pemphigoid and excoriation disorder. The extremities show diffuse rash with erythematous base, scabs in various stages of healing and scattered clear bullae.
Diffuse rash on the posterior torso of a woman with bullous pemphigoid and excoriation disorder.
A 2 cm closed bullous lesion containing clear serosanguinous fluid seen on the right medial lower extremity posterior to the medial malleolus of a patient with bullous pemphigoid and excoriation disorder.
Diffuse scabs on the anterior neck region of patient with bullous pemphigoid and excoriation disorder.
Investigations
Two punch biopsies were taken, one 0.4 cm in diameter from the left thigh and one 0.5 cm in diameter from the right thigh. Histology showed subepidermal bullous dermatosis with mixed inflammatory infiltrate of predominantly eosinophils (figures 5 and 6). Direct immunofluorescence of samples demonstrated linear deposition of IgG and C3 along the basement membrane (figure 7). A leukaemia panel of the peripheral blood indicated no diagnostic immunophenotypical abnormalities detected by flow cytometry. The histological features coupled with immunofluorescent findings are consistent with the diagnosis of bullous pemphigoid.
Subepidermal bullous dermatosis with mixed inflammatory infiltrate in the blister cavity (10 × magnification).
The mixed inflammatory infiltrate in the subepidermal blister cavity consists predominantly of eosinophils (40 × magnification).
Direct immunofluorescence shows linear deposition of IgG (primarily IgG4) and C3 along the basement membrane.
Treatment
The patient was initially treated with 125 mg of intravenous methylprednisolone while in the emergency room, and was subsequently treated with a course of vancomycin 15 mg/kg intravenously every 12 hours during her hospital stay after she was found to have a superinfection with methicillin-resistant Staphylococcus aureus. She was also cross-tapered from duloxetine to fluoxetine 40 mg daily to treat her excoriation disorder. At discharge, she was prescribed an oral prednisone taper starting at 60 mg daily and decreasing by 10 mg every 5 days until complete. She was also referred for outpatient psychotherapy.
Outcome and follow-up
The subject of this case was followed for 3 months after she was discharged from the hospital. She experienced significant improvement in skin symptoms while on the oral prednisone taper before she was lost to follow-up.
Discussion
Bullous pemphigoid is an autoimmune blistering disorder in which autoantibodies are directed against the hemidesmosomal antigens BP180 and BP230. As seen in this case, classic symptoms include severe pruritus and tense blisters with erosions and crusts.6 For the extensive form of bullous pemphigoid, first-line therapy includes a regimen of oral prednisone at a dosage of 0.5–0.75 mg/kg/day. Alternatively, some studies have shown that potent topical steroids appear to have similar efficacy to systemic steroids. If corticosteroid therapy alone fails, the use of immunosuppressive drugs such as azathioprine, mycophenolate mofetil, methotrexate, chlorambucil or cyclophosphamide can be considered as second-line therapy. For patients with mucosal involvement, dapsone can be used as an additional treatment agent. In patients with treatment resistance, intravenous immunoglobulin, plasmapheresis or rituximab can be used.7
Treatment for excoriation disorder involves psychotherapy, such as cognitive-behaviour therapy or habit reversal therapy. Pharmacotherapy options include selective serotonin reuptake inhibitors and lamotrigine.8 Excoriation disorder is related to OCD, for which fluoxetine is known to be especially efficacious in treating.9 Hence, the decision was made to cross taper this patient from duloxetine to fluoxetine for excoriation disorder management.
There is emerging, yet still controversial, evidence suggesting links between bullous pemphigoid and neuropsychiatric conditions. A nationwide study in Finland found an increased incidence of bullous pemphigoid in patients with a variety of psychiatric histories, including those with major depressive disorder like the patient in this case.10 Another European study also concluded that, among other conditions, individuals with unipolar or bipolar depression were at elevated risk for bullous pemphigoid.11 Conversely, however, a Singaporean study found no statistically significant correlation between psychiatric conditions and bullous pemphigoid.12 It is worth noting that each of these studies found an elevated risk for bullous pemphigoid in patients with dementia. No studies were found that specifically discussed OCD or excoriation disorder in bullous pemphigoid patients. This may be a consideration for future research.
A literature search revealed another case study in which a woman with unexplained skin lesions was misdiagnosed with excoriation disorder, despite adamantly denying skin-picking behaviour, before a thorough dermatological workup with biopsies revealed bullous pemphigoid.13 In the case of our patient, the remarkable appearance of the excoriations was nearly enough to camouflage the bullae on a cursory glance. However, close interdisciplinary cooperation and a complete examination of the patient that included her psychiatric history ultimately led to confirmed diagnoses of both bullous pemphigoid and excoriation disorder.
In this case, results of direct immunofluorescence that showed linear deposition of IgG and C3 along the basement membrane were used to diagnose the patient (figure 7). Other methods, including serological detection of autoantibodies by indirect immunofluorescence on human salt-split skin as well as ELISA using recombinant fragments of BP180 and BP230, can also be used to diagnose the condition.6 If direct immunofluorescence and serological tests return negative results for bullous pemphigoid, differential diagnoses can include drug reaction, erythema multiforme, atopic eczema, scabies, prurigo simplex subacuta, eosinophilic dermatitis or cutaneous T cell lymphoma.6
This report presented a case of bullous pemphigoid with concurrent skin excoriation disorder in a woman in her 50s. This particular case is notable for its concurrent severe dermatological and psychiatric components. When treating patients with bullous pemphigoid, physicians need to be aware of a potential link between the condition and neuropsychiatric conditions in order to ensure they address any concurrent or underlying mental health issues.
Patient’s perspective
I do not like to go anywhere where my skin will show and I have to wear long sleeves, even in the summer. It gets old when people keep asking what happened to your skin. If my skin heals, I want to go everywhere. The first place I would like to go is my local swimming pool. Sometimes my skin hurts so bad I can barely walk. It keeps opening up and bleeding.
Learning points
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Bullous pemphigoid can be diagnosed via direct immunofluorescence, salt-split skin analysis or ELISA.
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Research suggests there is a potential link between bullous pemphigoid and psychiatric disorders.
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Bullous pemphigoid and skin excoriation disorder can exist concurrently.
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Skin excoriation disorder (which is related to obsessive-compulsive disorder) can be managed pharmacologically with fluoxetine along with psychological intervention.
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First-line pharmacological management of bullous pemphigoid is systemic corticosteroids. Second-line pharmacological management includes immunosuppressive medications.
Ethics statements
Patient consent for publication
Acknowledgments
The authors thank the library staff at the Rowan University School of Osteopathic Medicine, as well as the pathology department at Ocean University Medical Center for their assistance. The authors also thank Harvard Vanguard Medical Associates for providing an expert dermatopathology consultation.
Footnotes
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Contributors KM and SP conceived of the presented case report. NV handled pathology specimens and pathology interpretation. KM and SP wrote all of the manuscript with the exception of the pathology section written by NV. As the fully practising physician involved in the case, NV helped supervise the project.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
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