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CORRESPONDENCE
Year : 2020  |  Volume : 65  |  Issue : 4  |  Page : 320-322
A report of multiple autoimmune syndrome: Pemphigus vulgaris associated with several immune-related diseases after thymectomy


1 Department of Dermatology, Peking Union Medical College, Peking Union Medical College Hospital, Dongcheng District, Beijing; Department of Dermatology, Graduate School of Hebei North University, Hebei, China
2 Department of Dermatology, Peking Union Medical College, Peking Union Medical College Hospital, Dongcheng District, Beijing, China

Date of Web Publication11-Jun-2020

Correspondence Address:
Ya-Gang Zuo
Department of Dermatology, Peking Union Medical College, Peking Union Medical College Hospital, Dongcheng District, Beijing
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_89_19

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How to cite this article:
Ge XL, Li SZ, Wang W, Zuo YG. A report of multiple autoimmune syndrome: Pemphigus vulgaris associated with several immune-related diseases after thymectomy. Indian J Dermatol 2020;65:320-2

How to cite this URL:
Ge XL, Li SZ, Wang W, Zuo YG. A report of multiple autoimmune syndrome: Pemphigus vulgaris associated with several immune-related diseases after thymectomy. Indian J Dermatol [serial online] 2020 [cited 2020 Jul 3];65:320-2. Available from: http://www.e-ijd.org/text.asp?2020/65/4/320/286411




Sir,

The co-occurrence of three or more autoimmune disorders in an individual is called multiple autoimmune syndrome (MAS).[1] According to the combination of diseases, it is divided into three types.[2] Currently, its pathogenesis is still unclear, and environmental factors and genetic factors are related to the occurrence of this disease.[3],[4] Patients with a single autoimmune disease have a 25% risk of developing other autoimmune diseases.[5] The coexistence of several diseases in an individual is rare. Here, we report a patient with thymoma who suffered from myasthenia gravis, vitiligo, pure red cell aplasia, systemic lupus erythematosus, lichen planus, alopecia areata, and pemphigus vulgaris after thymectomy. This is a novel combination of MAS. The presence of thymoma should alert the physician to the possible presence of MAS.

A 64-year-old female presented with an 8-year history of erythema and blisters on the scalp, face, trunk, and upper limbs. The patient had thymectomy 22 years ago, and she had been diagnosed with vitiligo, myasthenia gravis, pure red cell aplasia for 15 years, and systemic lupus erythematosus for nine years. She had been treated with oral prednisolone for both systemic lupus erythematosus and the blistering disorder and the blisters disappeared. During the process of prednisolone tapering, the blisters recurred. Her physical symptoms and past medical conditions were stable when the blistering disease recurred.

On physical examination, the hair on the scalp was almost completely lost [Figure 1]. Scattered erosive erythema with crusting and flaccid blisters were seen on the scalp, trunk, and upper limbs [Figure 2], with a positive Nikolsky's sign. A number of irregular depigmented patches could be seen on her chest and the face [Figure 3]. The depigmented patches, when exposed to ultraviolet (UV) light, glowed blue [Figure 4]. Irregular longitudinal grooving and ridging of the nail plate, thinning of the nail plate, and shedding of the nail plate with atrophy of the nail bed were also present [Figure 5]. The oral cavity, vulva, and other mucous membranes were not involved. The muscle strength was normal.
Figure 1: The hair on her scalp was almost completely lost

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Figure 2: Scattered erosive erythema and flaccid blisters with crust on the fore chest

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Figure 3: Depigmented patches on the face

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Figure 4: Blue glowing of the depigmented patches when exposed to ultraviolet light

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Figure 5: Irregular longitudinal grooving, ridging and thinning of the nail plate and shedding of the nail plate with atrophy of the nail bed

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Laboratory findings showed the erythrocyte sedimentation rate of 41 mm/h (0–20), lupus anticoagulant of 1.62 (≤1.2), antinuclear antibody (H-type) of 1:640 (<1:40), antidouble-stranded DNA antibody of 263 IU/ml (<100 IU/ml), antidesmoglein 1> 150 U/ml (<20 U/ml), and 91 U/ml (<20) of anti-desmoglein 3. No abnormalities in other blood parameters and urine, liver, and renal functions were detected. A skin biopsy from one of the blisters on her chest showed intraepidermal blister formation and acantholytic keratinocytes [Figure 6].
Figure 6: Microscopic examination showed intraepidermal blisters formation and acantholytic keratinocytes (H and E, x100)

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Based on these results, the patient was diagnosed as MAS—pemphigus vulgaris, myasthenia gravis, vitiligo, pure red cell aplasia, systemic lupus erythematosus, lichen planus and alopecia areata. The patient underwent treatment with methylprednisolone (24 mg/day). After treatment for 1 month, her skin lesions were resolved [Figure 7]. At the time of reporting, she was treated with 8 mg/day methylprednisolone, and the antidesmoglein 1 and 3 antibodies had dropped to 131 U/ml and 72 U/ml, respectively. Her condition was stable.
Figure 7: The blisters on the forechest disappeared after treatment

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This research effort was supported by grants from the Beijing Natural Science Foundation (7192166) and National Natural Science Foundation of China (81972944).

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Humbert P, Dupont JL. Multiple autoimmune syndromes. Ann Med Interne (Paris) 1988;139:159-68.  Back to cited text no. 1
    
2.
Harpreet S, Deepak J, Kiran B. Multiple autoimmune syndrome with celiac disease. Rheumatology 2016;6:326-9.  Back to cited text no. 2
    
3.
Johar AS, Anaya JM, Andrews D, Patel HR, Field M, Goodnow C, et al. Candidate gene discovery in autoimmunity by using extreme phenotypes, next generation sequencing and whole exome capture. Autoimmunity Rev 2015;14:204-9.  Back to cited text no. 3
    
4.
Selmi C, Crotti C, Meroni PL. Less traveled roads in clinical immunology and allergy: Drug reactions and the environmental influence. Clin Rev Allergy Immunol 2013;45:1-5.  Back to cited text no. 4
    
5.
Mohan MP, Ramesh TC. Multiple autoimmune syndrome. Indian J Dermatol Venereol Leprol 2003;69:298-9.  Back to cited text no. 5
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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