Dermatology Online Journal

Coexistence of bullous pemphigoid, vitiligo, and thyroid disease: A multiple autoimmune syndrome?
Andrés Tirado-Sánchez MD, and Griselda Montes-de-Oca MD
Dermatology Online Journal 11 (2): 20

Department of Dermatology, General Hospital of Mexico, Mexico, DF. andrestiradosanchez@hotmail.com

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Abstract

Bullous pemphigoid is the most common autoimmune blistering skin diseases. The significance of the association of bullous pemphigoid with other autoimmune diseases is still unknown. There have been reports of an association with many autoimmune skin diseases. We report the simultaneous occurrence of bullous pemphigoid and thyroid disease in a 76-year-old patient who also suffered from vitiligo. It is possible that there is a common underlying pathogenic mechanism involved in the co-existence of these three diseases. The association adds to the documentation of bullous pemphigoid co-existing with other autoimmune disorders. This association is probably not fortuitous and suggests a pathogenic relationship.

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Introduction

Bullous pemphigoid is one of the most common autoimmune bullous diseases, although its frequency is less than pemphigus vulgaris [1].

The association of bullous pemphigoid with many other diseases has been mentioned before, but no common ethiopathogenic pathway has been demonstrated [2, 3, 4]. The most frequent associations are those with primary biliary cirrhosis [5, 6, 7], psoriasis [8, 9, 10], and an unusual condition termed multiple autoimmune syndrome, defined as the combination of al least three autoimmune diseases in the same patient [11]. With multiple autoimmune syndrome we observe a special frequency various dermatological autoimmune diseases, such as vitiligo, bullous pemphigoid, and pemphigus vulgaris [12].

Clinical synopsis

Figure 1
Tense blisters and depigmented lesions of the right hand

We report a 76-year-old man with hypo- and depigmented macules. This condition initially began in January 1987 and involved the chest and back. Shortly thereafter lesions developed on the scalp, perioral area, and soles. He was diagnosed as having vitiligo. Treatment was undertaken with 8-methoxypsoralen but there was poor response at 8-9 months (Fig. 1). In January 2004 the patient presented with disseminated, large, tense and pruritic blisters, exacerbated following sun exposure, diagnosed clinopathologically as bullous pemphigoid (Figs. 2 and 3).

Figure 2	Figure 3
Tense blister over an depigmented lesion on the left hand

Physical examination revealed that the skin was dry, cool, and mottled. His reflex relaxation was delayed. The thyroid gland was smooth and firm, and of normal size. Estimated T4 levels were 4 µg/dl, the T3 level was 113 ng/dl, TSH 9.1 µU/ml.

The patient was treated with daily prednisone (0.75 mg/kg) and Dapsone (100 mg), with adequate response of bullous pemphigoid. The hypothyroidism was treated with daily L-thyroxin (75 mg).

Discussion

Multiple autoimmune syndrome, proposed by Humbert and Dupond, is recognized with increasing frequency [11]. Multiple autoimmune diseases can be classified into three groups according to the prevalence of their associations with one another (Table 1). The pathogenesis of the association is not known yet; perhaps environmental triggers and genetic susceptibility are involved [13, 14].

Table 1. Classification of the multiple autoimmune syndrome

TYPE 1	TYPE 2	TYPE 3
thymoma	rheumatoid arthritis	ATD
myasthenia gravis	Sjögrens syndrome	pernicious anaemia
giant cell myocarditis	primary biliary cirrhosis	Addison disease
pemphigus vulgaris	pemphigus vulgaris	vitiligo
bullous pemphigoid	SLE	dermatitis herpetiformis
ATD = autoimmune thyroid disease SLE = systemic lupus erythematous

Several other associations have been proposed as a form of multiple autoimmune syndrome. The association of bullous pemphigoid, vitiligo [15] and autoimmune thyroid disease [16] is not reported and it shows that conditions do not have to be standardized in groups because of its variability.

We conclude the association we found is not typical of the multiple autoimmune syndrome as defined by Humbert and Dupond [11]. However, this case presents a new association that fulfills the requirements for the diagnosis of multiple autoimmune syndrome. The underlying mechanisms for this syndrome are not yet understood, but it may be more prevalent than currently recorded. The presence of one of these findings should prompt an evaluation for the others.

References

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