Abstract
Cushing syndrome due to primary adrenocortical nodular dysplasia was diagnosed in two patients, aged 3 years 9 months and 9.5 years. Subsequently, adrenalectomy was performed and followed by steroid replacement. In both cases, the adrenals were normal or only slightly enlarged and showed adrenocortical nodular dysplasia histologically. Small lymphocytic infiltrates consisting of T-cells and class II MHC positive macrophages were present in adrenal specimens of both the patients. Samples of protein A sepharose purified serum immunoglobulins from both children stimulated adrenocortical DNA synthesis and cortisol production in cultured guinea-pig adrenal segments in vitro in a dose dependent fashion. Adrenal stimulating immunoglobulins were also demonstrated in serum specimens of both patients' mothers. However, none of them had overt signs of adrenal disease. Our data support the view that autoimmune mechanisms may be involved in primary adrenocortical nodular dysplasia.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Abbreviations
- ACTH:
-
adrenocorticotropic hormone
- CS:
-
Cushing syndrome
- IgGs:
-
immunoglobulins of class G
- PAND:
-
primary adrenocortical nodular dysplasia
References
Andrada JA, Murray FT, Andrada EC, Ezrin C (1979) Cushing's syndrome and autoimmunity. Arch Pathol Lab Med 103:244–246
Arce B, Licea M, Hung S, Padron R (1978) Familial Cushing's syndrome. Acta Endocrinol (Copenh) 87:139–147
Bohm N, Lippmann-Grob B, Petrykowski Wv (1983) Familial Cushing's syndrome due to pigmented multinodular adrenocortical dysplasia. Acta Endocrinol (Copenh) 102:428–435
Danoff A, Jormark S, Lorber D, Fleischer N (1987) Adrenocortical micronodular dysplasia, cardiac myxomas, lentigines, and spindle cell tumors. Report of a kindred. Arch Intern Med 147: 443–448
De Gennes, Garnier H, Calmette M, Malinsky M, Bertrand C (1970) Etude clinique, biologique et histologique d'un cas exemplaire de polymicroadenomatose corticosurrenale. Ann Endocrinol (Paris) 31:1022–1038
Donaldson DC, Grant DB, O'Hare MJ, Shackleton CHL (1981) Familial congenital Cushing's syndrome due to bilateral nodular adrenal hyperplasia. Clin Endocrinol 14:519–526
Goldblatt E, Snaith AH (1958) A case of Cushing's syndrome in an infant. Arch Dis Child 33:540–542
Hasleton PS, Ali HH, Anfield C, Beardwell CG, Shalet S (1982) Micronodular adrenal disease: a light and electron microscopic study. J Clin Pathol 35:1078–1085
Houwen RHJ, Drop SLS, Hazebroek FWJ, Kate FWJ ten (1983) Pituitary-dependent Cushing disease and primary adrenocortical nodular dysplasia in childhood. Eur J Pediatr 141:101–108
Kaplowitz PB, Carpenter R, Newsome HH, Downs RW (1986) Cushing's syndrome resulting from primary pigmented nodular adrenocortical disease. Am J Dis Child 140:1072–1075
Klevit HD, Campbell RA, Blair HR, Bongiovanni AM (1966) Cushing's syndrome with nodular adrenal hyperplasia in infancy. J Pediatr 68:912–920
Krabbe S, Nielsen Damkjær M (1986) Trilostane in the treatment of primary adrenocortical micronodular dysplasia (PAMD). Pediatr Res 20:1203
Larsen JL, Cathey WJ, Odell WD (1986) Primary adrenocortical nodular dysplasia, a distinct subtype of Cushing's syndrome. Case report and review of the literature. Am J Med 80:976–984
Marova EI, Bronstein ME, Kazeev KN, Yuryeva NP (1980) Microadenomatosis of the adrenal cortex and hypercorticism in pubertal age. Sov Med 3:15–20
McArthur RG, Bahn RC, Hayles AB (1982) Primary adrenocortical nodular dysplasia as a cause of Cushing's syndrome in infants and children. Mayo Clin Proc 57:58–63
Ralfkiaer E, Wantzin GL, Mason DY, Hou-Jensen K, Stein H, Thomsen K (1985) Phenotypic characterization of lymphocyte subsets in mycosis fungoides. Comparison with large plaque parapsoriasis and benign chronic dermatoses. Am J Clin Pathol 84: 610–619
Schweizer-Cagianut M, Froesch ER, Hedinger C (1980) Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia). Acta Endocrinol (Copenh) 94:529–534
Shenoy BV, Carpenter PC, Carney JA (1984) Bilateral primary pigmented nodular adrenocortical disease. Rare cause of the Cushing's syndrome. Am J Surg Pathol 8:335–344
Van Berkhout FT, Groughs RJM, Kater L, Schuurman HJ, Meyling FJHG, Kooyman CD, Gaag RD van der, Jolink D, Drexhage HA (1986) Familial Cushing's syndrome due to nodular adrenocortical dysplasia. A putative receptor-antibody disease? Clin Endocrinol 24:299–310
Wegienka LC, Wuepper KD, Komarmy LE, Forsham PH (1966) Cushing's syndrome with adrenal medullary insufficiency and adrenal auto-antibodies. Lancet I:741–743
Wulffraat NM, Drexhage HA, Wiersinga WM, Gaag RD van der, Jeucken P, Berghout A, Rees LH (1987) Adrenal stimulating immunoglobulins in Cushing's syndrome and eucorticoid adrenal dysplasia. In: Wulffraat NM (1988) Adrenal autoimmunity. Immunoglobulins affecting adrenal growth and steroidogenesis. Thesis, Amsterdam University, Amsterdam, pp 61–80
Wulffraat NM, Drexhage HA, Wiersinga WM, Gaag RD van der, Jeucken P (1988) Immunoglobulins of patients with Cushing's syndrome due to pigmented adrenocortical micronodular dysplasia stimulate in vitro steroidogenesis. J Clin Endocrinol Metab 66: 301–307
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Carstensen, H., Krabbe, S., Wulffraat, N.M. et al. Autoimmune involvement in Cushing syndrome due to primary adrenocortical nodular dysplasia. Eur J Pediatr 149, 84–87 (1989). https://doi.org/10.1007/BF01995852
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01995852