Indian Journal of Dermatology
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Year : 2016  |  Volume : 61  |  Issue : 1  |  Page : 128
IgG4-related skin lesion with Mikulicz's disease

1 Department of Dermatology, Asahikawa Medical University, Midorigaokahigashi, Asahikawa, Japan
2 Department of Clinical Pathology, Asahikawa Medical University, Midorigaokahigashi, Asahikawa, Japan

Date of Web Publication15-Jan-2016

Correspondence Address:
Hidetoshi Takahashi
Department of Dermatology, Asahikawa Medical University, Midorigaokahigashi, Asahikawa
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.174194

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How to cite this article:
Takahashi H, Hori M, Miyokawa N, Iizuka H. IgG4-related skin lesion with Mikulicz's disease. Indian J Dermatol 2016;61:128

How to cite this URL:
Takahashi H, Hori M, Miyokawa N, Iizuka H. IgG4-related skin lesion with Mikulicz's disease. Indian J Dermatol [serial online] 2016 [cited 2019 Sep 22];61:128. Available from:


IgG4-related disease shows common pathologic features of increased serum IgG4 level and IgG4-positive plasma cell infiltration associated with fibrosis. [1],[2],[3] Various organs are involved including lacrimal glands, salivary glands, lung, liver, kidney, pancreas, retroperitoneum, breast, pituitary gland, and prostate. Mikulicz's disease is among the IgG4-related diseases, and is characterized by sclerosis/fibrosis, lymphocytic infiltration and atrophy of the lacrimal glands and salivary glands. [4]

A 32-year-old female was admitted to our hospital with pruritic erythematous papular skin eruption on the face and trunk of 3 weeks duration. Clinical examination showed the patient also had protrusion of bilateral eyes, and submandibular swelling of 12 months duration before the occurrence of the skin eruption [Figure 1]a and b. She had no history of allergies. Blood eosinophil count was 875/μl accounting for 10.2% of the total blood cell count. Serum IgG level was 1430 mg/dl with IgG4 426 mg/dl (29.8%). Computed tomography (CT) scans revealed swelling of bilateral lacrimal gland and parotid glands without lymphadenopathy. No abnormal finding was detected in lung, liver, or pancreas. Skin biopsy of the facial papular lesion revealed lymphocytic infiltration of vessels and adnexal structures in the dermis [Figure 1]c. Biopsy of salivary glands showed marked lymphocyte and plasma cell infiltration accompanied by eosinophils [Figure 1]d. Immunohistochemical study revealed numerous IgG4-positive cells. The average IgG4 positive cells in skin and salivary glands were 65% and 71% in 5 randomly-selected high-power fields, respectively [Figure 1]e and f. The patient was diagnosed as Mikulicz's disease showing features of IgG4-related disease. Systemic administration of 30 mg prednisolone resulted in rapid improvement of skin lesions. The swelling of lacrimal glands and parotid glands subsided and serum IgG4 levels returned to normal range. No recurrence of the abnormal findings was detected by 10 mg systemic prednisolone treatment.
Figure 1: Clinical feature and histopatological examination of the patients (a) Swelling of bilateral upper eyelids. (b) Erythematous papular skin lesions on the right cheek. H.E histopathological examination of skin lesion (c) salivary gland (d) fibrosis and infiltration of lymphocytes and plasma cells. Immunohistochemical examination of skin lesion (e) salivary gland (f) infiltration of IgG4 positive plasma cells

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IgG4 does not activate complement via the classical pathway because of its low affinity for C1q and Fc receptors, suggesting a low potential for immune activation. Thus, IgG4 is supposed to prevent large immune complex formation and shows inhibitory effect against allergic reactions. In the presence of abnormal expression of IgG4, however, various abonormalities are induced in the lacrimal glands, salivary glands, lung, liver, kidney, and in the skin.

Mikulicz's disease was previously assumed to be a subtype of Sjogren syndrome. However, because of increased serum levels of IgG4 and infiltration of IgG4-expressing cells in the lacrimal and salivary glands, Mikulicz's disease is now included in IgG-4 related disorders. [5] Only a few reports exist describing skin lesions associated with IgG4-related disease. [6] Mikulicz's disease accompanied with reactive perforating collagenosis, [7] and erythematous nodules [6] have been described. In this report, we described Mikulicz's disease with erythematous papular lesion on the face and trunk with IgG4-positive cells in the dermis. Because no definite criteria for the diagnosis of recently identified IgG4-related disease exist, only small number of cases has been described in the literature. Thus, we suppose that IgG4-related disease with erythematous papular skin eruption is more prevalent, which should be recognized in the dermatological field.

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.

   References Top

Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539-51.  Back to cited text no. 1
Guma M, Firestein GS. IgG4-related diseases. Best Pract Res Clin Rheumatol 2012;26:425-38.  Back to cited text no. 2
Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4-related disease (IgG4RD): General concept and details. Mod Rheumatol 2012;22:1-14.  Back to cited text no. 3
Yamamoto M, Takahashi H, Sugai S, Imai K. Clinical and pathological characteristics of Mikulicz's disease (IgG4-related plasmacytic exocrinopathy). Autoimmun Rev 2005;4:195-200.  Back to cited text no. 4
Schur PH. IgG subclasses. A historical perspective. Monogr Allergy 1988;23:1-11.  Back to cited text no. 5
Kakuchi Y, Yamada K, Suzuki Y, Ito N, Yagi K, Matsumura M, et al. IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis. Intern Med 2011;50:1465-9.  Back to cited text no. 6
Shiomi T, Yoshida Y, Horie Y, Yamamoto O. Acquired reactive perforating collagenosis with the histological features of IgG4-related sclerosing disease in a patient with Mikulicz's disease. Pathol Int 2009;59:326-31.  Back to cited text no. 7


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