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?

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

Corresponding Author:
Dr. Kaushal K. Verma,
Professor,
Department of Dermatology and Venereology,
All India Institute of medical Sciences,
New Delhi, India.
Email: prokverma@hotmail.com
Phone: 011-26593454

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.

Sir,

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.

Click here to view
Figure 1C: Occasional targetoid lesions with central vesiculation and circumferential oedematous, dusky erythema over right ankle.

Click here to view

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).

Click here to view
Figure 2B: Indirect immunofluorescence (IIF) done on salt split showed linear deposition of IgG along the epidermal roof.

Click here to view

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

Click here to view

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.

References:

1. Rao R, Gupta A, Yunis F, Handettu S, Chandrashekar B. Coexistence of psoriasis with bullous pemphigoid. Indian Dermatol Online J 2012;3:119–21.

2. Koerber WA, Price NM, Watson W. Coexistent psoriasis and bullous pemphigoid: a report of six cases. Arch Dermatol 1978;114:1643–6.

3. Wilmer EN, Becker N, Chen A, Kroumpouzos G. Etanercept-induced generalization of chronic, localized, anogenital bullous pemphigoid in a psoriatic patient. JAAD Case Rep 2016;2:25–7.

4. Nakayama C, Fujita Y, Watanabe M, Shimizu H. Development of bullous pemphigoid during treatment of psoriatic onycho-pachydermo periostitis with ustekinumab. J Dermatol 2015;42:996–8.

5. Ho PH, Tsai TF. Development of bullous pemphigoid during secukinumab treatment for psoriasis. J Dermatol 2017 May 23.

6. Lesniewska A, Kalinska-Bienias A, Kowalewski C, Schwartz R, Wozniak K. Development of bullous pemphigoid in a patient with psoriasis and metabolic syndrome. Cutis 2016;98:E19–23.

7. Loget J, Plée J, Antonicelli F, Bernard P. A successful treatment with ustekinumab in a case of relapsing bullous pemphigoid associated with psoriasis. J Eur Acad Dermatol Venereol 2017;31:e228–30.

8. Okahashi K, Oiso N, Ishii N, Uchida S, Matsuda H, Hashimoto T, et al. Bullous pemphigoid associated with psoriasis: A possible example of an inverse intramolecular epitope-spreading phenomenon. J Dermatol 2015;42:758–9.

9. Caca-Biljanovska N, Arsovska-Bezhoska I, V’lckova-Laskoska M. PUVA-induced Bullous Pemphigoid in Psoriasis. Acta Dermatovenerol Croat 2016;24:214–7.

10. Garrido Colmenero C, Arias Santiago S, Blasco Morente G, Pérez López I, Aneiros Fernández J. Photoletter to the editor: Psoriatic erythroderma associated with bullous pemphigoid: clinical appearance and histopathology. J Dermatol Case Rep 2015;9:23–4.

11. Iskandarli M, Gerceker Turk B, Yaman B, Ozturk G. Pemphigoid Diseases as a Sign of Active Psoriasis: A Case Report and Brief Review. Dermatol Basel Switz 2015;231:319–21.

12. Onsun N, Sallahoglu K, Dizman D, Su Ö, Tosuner Z. Bullous pemphigoid during ustekinumab therapy in a psoriatic patient. Eur J Dermatol 2017;27:81–2.

13. Le Guern A, Alkeraye S, Vermersch-Langlin A, Coupe P, Vonarx M. Bullous pemphigoid during ustekinumab therapy. JAAD Case Rep 2015;1:359–60.

14. Gunay U, Gunduz K, Ermertcan AT, Kandiloglu AR. Coexistence of psoriasis and bullous pemphigoid: remission with low-dose methotrexate. Cutan Ocul Toxicol 2013;32:168–9.

15. Si X, Ge L, Xin H, Cao W, Sun X, Li W. Erythrodermic psoriasis with bullous pemphigoid: combination treatment with methotrexate and compound glycyrrhizin. Diagn Pathol 2014;9:102.

16. Wang TS, Tsai TF. Remission of bullous pemphigoid after rituximab treatment in a psoriasis patient on regular low-dose methotrexate. Acta Derm Venereol 2014;94:108–9.



Top