|Year : 2019 | Volume
| Issue : 2 | Page : 145-147
Malar blistering: A diagnostic dilemma
Kanathur Shilpa, B Leelavathy, DV Lakshmi, Divya Gorur
Department of Dermatology, BMCRI, Bangalore, Karnataka, India
|Date of Web Publication||15-Jul-2019|
No 53, OPD B Block, BMCRI, Bangalore, Karnataka
Source of Support: None, Conflict of Interest: None
Bullous pemphigoid (BP) is an autoimmune blistering disorder affecting elderly people. It presents as tense blisters on urticarial base and is associated with intense pruritus. It commonly involves lower abdomen, inner or anterior thighs, and flexor forearms. Localized BP limited to certain parts of the body has also been described in literature. Both classical and localized BP is diagnosed based on histopathology and immunofluorescence. Corticosteroids remain the main stay of treatment. Here, we describe a case of BP presenting as malar blistering closely mimicking bullous systemic lupus erythematosus.
Keywords: Bullous pemphigoid, localized, vesicular variant
|How to cite this article:|
Shilpa K, Leelavathy B, Lakshmi D V, Gorur D. Malar blistering: A diagnostic dilemma. Clin Dermatol Rev 2019;3:145-7
| Introduction|| |
Bullous pemphigoid (BP) is an autoimmune subepidermal dermatosis that generally affects people older than 70 years, with no ethnic, racial, or sexual predilection. Classic lesions include tense blisters on erythematous or urticarial base. Its varied manifestations include localized, nodular, vegetating, erosive, erythrodermic, juvenile, and drug induced. A few cases of BP manifesting in young females have been described in literature. Here, we report a case of vesicular BP localized to face in a 23-year-old Indian patient.
| Case Report|| |
A 23-year-old female patient presented to the dermatology department with a history of severe itching and water filled lesions limited to face and neck since 1½ month. Pruritus was intense, present throughout the day, unrelated to sun exposure and was not relieved by taking antihistamines (over the counter). Within 1–2 days, water-filled lesions developed at the site of scratching. They persisted for 2–3 days before rupturing, leaving raw areas which healed with hypo- and hyper-pigmentation.
There was no history of photosensitivity, fever, joint pain, oral ulcer, or any other symptoms suggestive of lupus erythematosus. There was no history of intake of any drug before the onset of lesions.
Examination revealed multiple tense vesicles distributed predominantly over bilateral malar area [Figure 1]. Crusting, hyper- and hypo-pigmented macules were seen at the site of healed lesions. There was no scarring or milia formation. Ocular, oral cavity and genital regions were normal. Nikolsky's sign was negative. Tzanck smear was negative for acantholytic cells and showed few neutrophils. BP, linear Ig A disease, and bullous systemic lupus erythematosus (BSLE) were considered as differential diagnoses. Histopathology revealed subepidermal blister with neutrophilic infiltrate over the papillary dermis with dermal edema [Figure 2]. Direct immunofluorescence (DIF) showed linear deposits of C3 along dermoepidermal junction, with negative Ig G, IgM, IgA, and C1q deposits [Figure 3]. Based on the immunofluorescence findings, complete blood count, liver function test, renal function test, urine routine, and ANA titers were done to rule out BSLE, but all were within normal limits except for mild elevation in ESR (22 mm/h). The patient did not fulfill the diagnostic criteria for BSLE. A diagnosis of localized BP (LBP) was made based on clinical feature, histopathology, and immunofluorescence findings. The patient was started on prednisolone 40 mg with tapering dose along with dapsone 100 mg and topical medications with sunscreens and mild topical corticosteroids (betamethasone dipropionate 0.05% cream for 2 weeks). There was an improvement in skin lesions in the 1st week. Later, the patient was lost to follow-up.
|Figure 1: Multiple vesicles with crusting and hypopigmentation on both cheeks|
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|Figure 3: Direct immunofluorescence showing linear deposits of immune reactants at dermoepidermal junction|
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| Discussion|| |
Autoimmune blistering skin diseases are a heterogeneous group of conditions characterized clinically by the formation of vesicle, bullae, and erosions on the skin and mucous membranes. Depending on the level of split, they are classified as intraepidermal and subepidermal blistering disorders. Subepidermal blistering disorders include BP, pemphigoid gestationis, lichen planus pemphigoides, linear IgA bullous disease (LABD), cicatricial pemphigoid, anti-p200, anti-p105, and anti-p450 pemphigoid, epidermolysis bullosa acquisita (EBA), and BSLEs.
Autoimmune bullous diseases may overlap in morphology and immunopathology; however, factors such as age of onset, course of the disease, absence of scarring, and extent of mucosal involvement are important in differentiating the diagnosis. DIF and salt-split indirect immunofluorescence are useful in distinguishing BP from other subepidermal diseases, namely linear IgA disease, mucous membrane pemphigoid, and EBA.
BP primarily affects elderly individuals in the fifth to seventh decade of life, with average age of onset being 65 years. BP in childhood has been reported from various countries including India. The pathogenesis of BP is characterized by tissue-bound and circulating IgG autoantibodies against two components of the hemidesmosome of stratified epithelia, BP 230 kD, and BP 180 kD.
The characteristic skin lesion is a large tense blister arising on erythematous, urticarial base, or on normal skin. Intense pruritus is frequently present. These lesions commonly involve lower abdomen, inner or anterior thighs, and flexor forearms, although they may occur anywhere. LBP is characterized by chronic intermittent eruptions affecting only a restricted area of the body. Although it accounts for 16%–29% of all cases of BP, the true incidence may be greater as it is often misdiagnosed and is highly responsive to topical steroids. LBP has got similar clinical, histopathological, and immunofluorescence features to generalized BP. Localized BP involving the pretibial, oral or vulvar region, breast, axillae, and groin has also been described. Clinical variants in which lesions are mainly vesicular (vesicular pemphigoid) or localized to head and neck have been described.
Histopathology from lesional skin demonstrates a subepidermal blister. The inflammatory infiltrate is typically polymorphous, with an eosinophilic predominance. Predominant neutrophils of subepidermal blisters in bullous pemphigoid are a rarity. Lara Andrachuk in his case report has suggested that BP should be considered in the differential diagnosis of neutrophil-rich subepidermal bullous disease with dermatitis herpetiformis and LABD., Tzanck smear shows only inflammatory cells.
DIF studies on normal-appearing perilesional skin demonstrate deposits of IgG antibodies in 90%–95% of cases and C3 in 100% of cases at the basement membrane zone. IgG deposits are rarely present in the absence of C3, but the presence of IgA, IgM, and IgE has also been described. In about 5%–10% of the cases, C3 deposits are present in the absence of detectable IgG. Isolated C3 deposits also occurs in HG, cicatricial pemphigoid, and extremely uncommon in EBA and BSLE. This pattern of immune reactants is not specific to BP and may be seen in cicatricial pemphigoid and EBA. BP can be differentiated from these conditions by the salt-split technique. DIF on salt-split skin reveals IgG on the blister roof (epidermal side of split skin) in BP.
The goal of therapy is to decrease blister formation, promote healing of blisters and erosions, and achieve the minimal dose necessary to control the disease process. Localized BP often can be treated successfully with topical steroids alone. The extensive disease needs to be treated with systemic anti-inflammatory and immunosuppressive agents, oral corticosteroids being the mainstay of treatment.
| Conclusion|| |
BP presenting in third decade with the predominantly vesicular variant limited to face with histopathology showing neutrophilic infiltrate and immunofluorescence showing isolated C3 linear deposits is a rare entity and has to be differentiated from bullous SLE which is a close mimic.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]