Vascular cognitive impairment in Pemphigus vulgaris: a case report

Siqueira- Neto, José Ibiapina; Sales, Paulo Marcelo Gondim; Sobreira, Emmanuelle Silva Tavares; Limeira, Aline Miranda; Araujo, Heline Bessa; Santos, Júnia Vieira dos; Castro, José Daniel Vieira de

doi:10.1590/S1980-57642012DN06030014

ABSTRACT

Pemphigus vulgaris is a systemic auto-immune medical condition that mainly manifests with changes in skin and vasculopathy. This is a case report of a 69-year-old male with confirmed histopathologic diagnosis of Pemphigus vulgaris presenting ulterior Cognitive Impairment, mostly in executive function. The patient was treated using steroids, immunomodulatory therapy, fluoxetine and galantamine. Neuropsychological testing and magnetic resonance (MRI) were performed. This is the first report of correlational cognitive impairment with Pemphigus vulgaris in the literature. Physicians should be aware of vascular causes for cognitive impairment in patients presenting auto-immune conditions.

Key words:
Pemphigus vulgaris; cognitive vascular impairment; neuropsychological testing; auto-immune diseases; dementia

RESUMO

Phemphigus vulgaris é uma condição médica sistêmica autoimune que principalmente se manifesta com alterações de pele e vasculopatia. Este é um caso de um homem de 69 anos com diagnóstico histopatológico de Phemphigus vulgaris apresentando posterior comprometimento cognitivo, predominante em funções executivas. O paciente foi tratado com esteroides, terapia imunomoduladora, fluoxetina e galantamina. Avaliação neuropsicológica e ressonância magnética foram realizados. Este é o primeiro relato correlacionando comprometimento cognitivo a pênfigo vulgar na literatura. Os clínicos devem estar cientes das causas vasculares para comprometimento cognitivo em pacientes se apresentando com condições autoimunes.

Palavras-chave:
Phemphigus vulgaris; comprometimento cognitivo vascular; avaliação neuropsicológica; doenças autoimunes; demência

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REFERENCES

Aggarwal NT, DeCarli C. Vascular Dementia. Sem Neurol 2007;27:66-77.
Leys D, Hénon H, Mackowiak-Cordoliani M-A, Pasquier F. Poststroke dementia. Lancet Neurol 2005;4:752-759.
Román GC, Erkinjuntti T, Wallin A, Pantoni L, Chui HC. Subcortical ischaemic vascular dementia. Lancet Neurol 2002;1:426-436.
Román GC, Sachdev P, Royall DR, et al.Vascular cognitive disorder: a new diagnostic category updating vascular cognitive impairment and vascular dementia. J Neurol Sci 2004;226:81-7.
Peter Berlit. Neuropsychiatric disease in collagenvascular diseases and vasculitis. J Neurol 2007;254(Suppl 2):II87-9. Erratum in: J Neurol 2008;255:309-310.
Román GC, Tatemichi TK, Erkinjuntti T et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology 1993;43:250-260.
Venugopal SS, Murrell DF. Diagnosis and clinical features of pemphigus vulgaris Immunol Allergy Clin North Am 2012;32:233-43, v-vi.
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29:432-436.
Malik M, Ahmed AR. Dual diagnosis of Pemphigus vulgaris and connective tissue disease. J Am Acad Dermatol 2006;55:699-704.
Jindal T, Meena M, Kumar A, Khaitan BK. Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. Pediatr Int 2011; 53:1108-1109.
Grando SA. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity 2012;45:7-35.
Sansaricq F, Stein SL, Petronic-Rosic V. Autoimmune bullous diseases in childhood. Clin Dermatol 2012;30:114-127.
Khaled A, Taazayet SB, Ben Alaya N, et al. The course and prognosis of pemphigus in 47 Tunisian patients. J Eur Acad Dermatol Venereol 2011 Dec 7. doi:10.1111/j.1468-3083.2011.04362.x.
» https://doi.org/10.1111/j.1468-3083.2011.04362.x

Publication Dates

Publication in this collection
Jul-Sep 2012

History

Received
10 June 2012
Accepted
20 Aug 2012

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Aggarwal NT, DeCarli C. Vascular Dementia. Sem Neurol 2007;27:66-77.

[2] Leys D, Hénon H, Mackowiak-Cordoliani M-A, Pasquier F. Poststroke dementia. Lancet Neurol 2005;4:752-759.

[3] Román GC, Erkinjuntti T, Wallin A, Pantoni L, Chui HC. Subcortical ischaemic vascular dementia. Lancet Neurol 2002;1:426-436.

[4] Román GC, Sachdev P, Royall DR, et al.Vascular cognitive disorder: a new diagnostic category updating vascular cognitive impairment and vascular dementia. J Neurol Sci 2004;226:81-7.

[5] Peter Berlit. Neuropsychiatric disease in collagenvascular diseases and vasculitis. J Neurol 2007;254(Suppl 2):II87-9. Erratum in: J Neurol 2008;255:309-310.

[6] Román GC, Tatemichi TK, Erkinjuntti T et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology 1993;43:250-260.

[7] Venugopal SS, Murrell DF. Diagnosis and clinical features of pemphigus vulgaris Immunol Allergy Clin North Am 2012;32:233-43, v-vi.

[8] Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29:432-436.

[9] Malik M, Ahmed AR. Dual diagnosis of Pemphigus vulgaris and connective tissue disease. J Am Acad Dermatol 2006;55:699-704.

[10] Jindal T, Meena M, Kumar A, Khaitan BK. Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. Pediatr Int 2011; 53:1108-1109.

[11] Grando SA. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity 2012;45:7-35.

[12] Sansaricq F, Stein SL, Petronic-Rosic V. Autoimmune bullous diseases in childhood. Clin Dermatol 2012;30:114-127.

[13] Khaled A, Taazayet SB, Ben Alaya N, et al. The course and prognosis of pemphigus in 47 Tunisian patients. J Eur Acad Dermatol Venereol 2011 Dec 7. doi:10.1111/j.1468-3083.2011.04362.x.
» https://doi.org/10.1111/j.1468-3083.2011.04362.x

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