LETTER TO EDITOR
Year: 2017 I Volume: 1 I Issue: 1 I Page: 30-32
Psoriasis with Bullous Pemphigoid: plausible association or chance co-incidence?
Sanjay Singh, Tanvi Dev, Firdaus Ali, Neetu Bhari, Kaushal K. Verma
1Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India
2Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
Dr. Kaushal K. Verma,
Department of Dermatology and Venereology,
All India Institute of medical Sciences,
New Delhi, India.
Key Words-Psoriasis, Bullous pemphigoid, Autoimmune disease
How to cite this article:
Singh S, Dev T, Ali F, Bhari N, Verma KK. Psoriasis with Bullous Pemphigoid: plausible association or chance co-incidence?. Indian Journal of Clinical Dermatology. 2017;1:30-32.
A 35-year-old male, known case of psoriasis for 25 years, presented with exacerbation of psoriasis since 1 month with body surface area of 20% involvement and PASI of 13.4. The patient had received various topical as well as oral therapies including oral psoralen with ultraviolet A (PUVA) therapy for psoriasis and was off treatment for 6 months. Four days prior to consultation, he started developing multiple, severely itchy, mildly erythematous urticarial plaques with occasional targetoid lesions in a generalized distribution. The lesions were predominantly present on the chest, upper back and acral areas, both on psoriatic plaques as well on unaffected skin. There was no mucosal involvement. In the next 2 days, clear fluid-filled tense vesicles and bullae developed on these lesions (Figure 1A-C).
|Figure 1A & 1B: Involvement of chest, right lower thigh and right upper leg in form of multiple clear fluid filled tense vesicles and bullae on psoriatic plaques as well on normal skin.
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|Figure 1C: Occasional targetoid lesions with central vesiculation and circumferential oedematous, dusky erythema over right ankle.
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Nikolsky sign was negative, while bulla spread sign was positive. A biopsy from the margin of a bulla was taken with clinical differentials of bullous pemphigoid (BP) and linear IgA disease. It revealed a subepidermal cleft with occasional eosinophils and neutrophils admixed with RBCs (Figure 2A). Direct immunofluorescence (DIF) from perilesional skin showed C3 and IgG deposition at dermo-epidermal junction. Indirect immunofluorescence (IIF) was done on salt split study of normal skin which showed linear deposition of IgG along the epidermal roof confirming the diagnosis of BP (Figure 2B). The patient was treated with methotrexate 15 mg/week, prednisolone 40mg/day and dapsone 100mg once daily. There was more than 80% improvement in both psoriasis and bullous pemphigoid lesions in the next 2 weeks following which prednisolone was rapidly tapered and stopped in 2 months while methotrexate and dapsone were continued. Four months later, methotrexate was stopped, however, dapsone was continued. There was no recurrence of bullous lesions after 5 months of follow-up.
|Figure 2A: Split at dermo-epidermal junction with occasional eosinophils and neutrophils admixed with RBCs (haematoxylin and eosin, 40X).
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|Figure 2B: Indirect immunofluorescence (IIF) done on salt split showed linear deposition of IgG along the epidermal roof.
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Bullous pemphigoid is an autoimmune bullous disease characterized by extremely pruritic, tense, clear as well as hemorrhagic fluid-filled bullae over the erythematous, urticarial, or non-inflammatory base with relative sparing of the mucous membranes. The typical histopathological finding in bullous pemphigoid is a subepidermal bulla with eosinophils. DIF shows linear deposition of C3 and IgG in most cases. IIF done on salt-split study of normal skin is diagnostic which shows linear deposition of IgG at the roof of the blister. Our patient had clinical as well as laboratory tests findings consistent with bullous pemphigoid.
Several autoimmune bullous disorders have been described in association with psoriasis, of which bullous pemphigoid (BP) is the most common 1. The inciting factor responsible for the development of BP in patients with psoriasis remains unknown. Though various hypothesis have been proposed, of which immunological damage at the basement membrane zone secondary to primary disease, damage induced by psoriasis treatment (anthralin, tar, ultraviolet B, PUVA), and common immunological mechanisms in both the diseases are the important ones 1,2. The concept of “epitope spreading” appears quite plausible in this process, whereby tissue damage from a primary inflammatory process leads to release and exposure of a ‘sequestered’ antigen in exciting a secondary autoimmune response 1. Our patient was a known case of psoriasis who received various drugs i.e. tar, PUVA in the past. Thus, immunological damage secondary to psoriasis or these therapies could possibly have contributed to the development of bullous pemphigoid in him. Recently, many biologics i.e. etanercept, efalizumab, ustekinumab and secukinumab have been attributed for development of BP in patients of psoriasis 3–5. We have summarized the recently reported cases of BP developing in psoriasis patients (Table 1) 3-13.
|Table 1: Bullous pemphigoid associated with psoriasis
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Various drugs, alone or in combination i.e. methotrexate, acitretin, azathioprine, dapsone, mycophenolate mofetil, etanercept,and rituximab have been used successfully to treat BP with psoriasis 1,14–16.
We report this case in view of the rarity of these two common dermatological disorders occurring in the same patient and a good response to a combination therapy of prednisolone, methotrexate, and dapsone.
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