Letter to the editor
Eur J Dermatol 2010; 20 (4): 547
Georgia Dermatopathology Associates,
1534 North Decatur Rd. NE; Suite 206,
Atlanta, GA 30307-1000, USA
Multiple autoimmune syndrome in a Japanese
patient with pemphigus foliaceus
<[email protected]>
doi: 10.1684/ejd.2010.0974
e would like to comment on the article by
Megumi Furuichi et al., Multiple autoimmune
syndrome in a Japanese patient with pemphigus foliaceus published in the last issue of the European
Journal of Dermatology [1].
In multiple autoimmune syndrome, patients often have at
least one dermatological condition, usually vitiligo or alopecia areata [2]. Researchers note that in many cases, the
presence of one autoimmune disorder helps lead to the
discovery of other autoimmune conditions [3, 4]. The
causes of multiple autoimmune syndrome seem to point
to familial or genetic, infectious, immunological and psychological factors. About 25 percent of patients with autoimmune diseases have a tendency to develop additional
autoimmune disorders. Multiple autoimmune syndrome
can be classified into three groups that correspond with
the prevalence of their being associated with one another.
In patients with two autoimmune diseases, this classification is helpful when signs of a third disorder emerge.
1) Type 1 MAS includes myasthenia gravis thymoma
(tumor of the thymus gland), polymyositis (inflammatory
muscle disease), and giant cell myocarditis (inflammatory
heart muscle disease).
2) Type 2 MAS includes Sjogren’s syndrome, rheumatoid
arthritis, primary biliary cirrhosis, scleroderma, and autoimmune thyroid disease (Hashimoto’s thyroiditis, atrophic
thyroiditis, Graves’ disease).
3) Type 3 MAS groups together autoimmune thyroid disease, myasthenia gravis and/or thymoma, Sjogren’s syndrome, pernicious anemia, idiopathic thrombocytopenic
purpura (ITP), Addison’s disease, insulin dependent diabetes, vitiligo, autoimmune hemolytic anemia (AIHA),
systemic lupus erythematosus (SLE), and dermatitis herpetiformis. For this group, the immune system marker
HLA-B8 and/or DR3 or DR5 seems to be an important
factor [5, 6].
Other conditions found in various combinations in MAS
are: i) Pemphigus and autoimmune thyroid disease in type
1 MAS; ii) Chronic active hepatitis, SLE, pemphigus, bullous pemphigoid, AIHA, ITP, alopecia areata and Addison’s disease in type 2 MAS; iii) Acquired primary hypogonadism, hypophysitis, rheumatoid arthritis, primary
biliary cirrhosis, relapsing polychondritis, multiple sclerosis, chronic active hepatitis, ulcerative colitis, and scleroderma in type 3 MAS.
EJD, vol. 20, n° 4, July-August 2010
The case report of Furiuchi et al. shows that a Japanese
female presented with clinical features of three autoimmune diseases, i.e., primary biliary cirrhosis (PBC),
Hashimoto’s thyroiditis (HT), and pemphigus foliaceus
(PF) [3, 4]. The authors nicely present their conclusion,
i.e., that the patient case fulfills the criteria for a multiple
autoimmune syndrome (MAS) [3, 4]. The present case
demonstrates a new clinical disease association that fulfills the MAS requirements. The authors follow elegant
reasoning from the general to the specific parts of their
deduction, i.e., that the association of two or more autoimmune diseases may be related to a more complex
immunogenetic mechanism. Further, the authors offer an
additional, subtle reasoning, presenting the possibility that
pemphigus foliaceus, given its well documented multifactorial pathophysiology, may often play a silent or overt
clinical role in multiple autoimmune syndrome patients.
Acknowledgements. Funding sources: Georgia Dermatopathology Associates (MSH), Atlanta, Georgia, USA.
Conflict of interest: none.
1. Furuichi M, Makino T, Hara H, Matsui K, Shimizu T. Multiple autoimmune syndrome in a Japanese patient with pemphigus foliaceus.
Eur J Dermatol 2010; 20: 396-7.
2. Wilkinson SM, Smith AG, Davis MJ, Hollowood K, Dawes PT.
Rheumatoid arthritis: an association with pemphigus folliaceus. Acta
Derm Venereol 1992; 72: 289-91.
3. Hambert P, Dupond JL. Multiple autoimmune syndromes. Ann
Med7 Interne 1988; 139 : 159-68.
4. Humbert P, Dupond JL, Vuitton D, Agache A. Dermatological autoimmune diseases and the multiple autoimmune syndromes. Acta Derm
Venereol 1989 (Suppl. 148): 1-8.
5. Mohan M, Ramesh T. Multiple Autoimmune Syndrome. Indian Journal of Dermatology, Venereology and Leprology 2003; 69: 298-9.
6. Tesavibul N. Multiple Autoimmune Diseases. The Ocular Immunology and Uveitis Foundation, accessed December 24, 2000.

Multiple autoimmune diseas the japanese