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Generalized Acanthosis Nigricans Related to Type B Insulin Resistance Syndrome: a Case Report

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Generalized Acanthosis Nigricans Related to Type B Insulin Resistance Syndrome: a Case Report Generalized Acanthosis Nigricans Related to Type B Insulin Resistance Syndrome: A Case Report Ki-Heon Jeong, MD; Seung-Joon Oh, MD, PhD; Suk Chon, MD, PhD; Mu-Hyoung Lee, MD, PhD Acanthosis nigricans (AN) is a cutaneous marker generalized AN and type B insulin resistance syn- for many underlying states such as endocrine drome with hypoglycemia. abnormality, obesity, certain drugs, and malig- nancy. Generalized AN is a rare condition and Case Report is commonly seen in adults with an underlying A 36-year-old man presented with progressive dark- malignancy. The type B insulin resistance syn- ening of the skin and coarsening of features over drome, a rare autoimmune disorder, is caused 6 months before he visited a clinic. The patient also by the autoantibodies to the insulin receptor. reported intermittent sweating, anxiety, and dizzi- Patients typically present with hyperglycemia but ness at dawn. Hyperpigmentation and thickening of also may present with hypoglycemia. We report the skin on the periocular and perioral areas, trunk, a rare case of a 36-year-oldCUTIS man with general- and extremities were observed on physical examina- ized AN and type B insulin resistance syndrome tion (Figure 1). Thicker, tan, velvety hyperkeratotic with hypoglycemia. plaques were prominent on the neck and axillae Cutis. 2010;86:299-302. (Figure 2) as well as the groin. The mucous mem- branes, palms, and soles were not affected. Histopathologic examination showed hyperkera- canthosis nigricans (AN) is a condition that is tosis, mild acanthosis, prominent papillomatosis, and Doclinically characterized byNot dark, coarse, thick- slight hyperpigmentationCopy of the basal layer (Figure 3). Aened skin with a velvety texture and is most A slight perivascular infiltrate of mostly lymphocytes prominent on the neck and axillae. There is much was present in the papillary dermis. speculation about the etiology of AN and a multi- Results of laboratory investigations, including tude of systemic diseases have been associated with complete blood cell counts and urinalysis, were AN. Tissue resistance to insulin has been thought to within reference range. Serum chemistry results were play an important role in the pathogenesis of AN. normal, except for the blood glucose level, which Type B insulin resistance syndrome is an unusual was 54 mg/dL (reference range, 70–110 mg/dL). To autoimmune disorder characterized by the produc- rule out diabetes mellitus, we performed the oral tion of antibodies against the insulin receptor. It glucose tolerance test, which did not indicate the dis- typically presents with severe hyperglycemia but ease. On the fasting provocation test, hypoglycemia also may cause episodes of hypoglycemia. We pre- developed at 3 hours with blood glucose and insulin sent an uncommon case of a 36-year-old man with levels at 24 mg/dL and 19.8 μIU/mL (reference range, 2.0–20 μIU/mL), respectively. We also performed abdominal computed tomography to rule out insu- linoma, but no abdominal mass was evident. Other All from the College of Medicine, Kyunghee University, Seoul, Korea. laboratory data included antinuclear antibodies, Drs. Jeong and Lee are from the Department of Dermatology and anti– double-stranded DNA antibodies, anticardio- Drs. Oh and Chon are from the Department of Internal Medicine. lipin antibodies, antiphospholipid antibodies, lupus The authors report no conflict of interest. Correspondence: Mu-Hyoung Lee, MD, PhD, Department of anticoagulant, and rheumatoid factor, which were Dermatology, College of Medicine, Kyunghee University, 1 Hoeki-Dong, all negative. Thyroid function studies revealed no Dongdaemun-Ku, Seoul 130-702 Korea ([email protected]). abnormalities and thyroid autoantibody was within WWW.CUTIS.COM VOLUME 86, DECEMBER 2010 299 Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Generalized Acanthosis Nigricans A B Figure 1. Acanthosis nigricans involving the periocular and perioral regions (A) as well as the trunk (B). CUTIS Do NotA Copy B Figure 2. Thick, tan, velvety hyperkeratotic plaques on the neck (A) and axillae (B). reference range. Insulin autoantibody was 9.2% (ref- Comment erence range, 0%–7%) and insulin receptor autoanti- Acanthosis nigricans is characterized by velvety to body was positive. verrucous, hyperkeratotic, hyperpigmented plaques The patient was diagnosed with generalized with grayish brown coloration. The lesions are sym- AN and type B insulin resistance syndrome. His AN metrically distributed and affect only flexural areas, was treated with 40 mg of oral prednisolone daily including the neck, axillae, groin, and antecubital to inhibit production of the autoantibodies and and popliteal areas.1 On occasion, the eruption 40 mg of oral isotretinoin daily. Serial decrements of may become almost generalized.2 Generalized AN the dosage of prednisolone were administered over does not represent a specific type of AN, but it can 14 months and then discontinued because there was be seen as a variant or rare manifestation of certain no further recurrence of hypoglycemic symptoms. types of AN. It is commonly seen in adults with an The isotretinoin dosage was maintained for underlying malignancy.3 8 months and then discontinued. For 3 years after Type B insulin resistance syndrome, a rare autoim- treatment, hypoglycemia did not recur and the mune disorder, is caused by the autoantibodies to the most recent random blood glucose levels were in insulin receptor.4 It has been predominantly seen in the 80 to 150 mg/dL range. Insulin autoantibody older black women and frequently is associated with was normalized to 6.9%, while the insulin receptor an autoimmune disease, particularly systemic lupus autoantibody was positive. The skin showed partial erythematosus.5 Patients typically present with hyper- improvement at 16 months of treatment (Figure 4). glycemia but also may present with hypoglycemia if 300 CUTIS® WWW.CUTIS.COM Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Generalized Acanthosis Nigricans Figure 3. Hyperkeratosis, mild acantho- sis, prominent papillomatosis, and slight hyperpigmentation of the basal layer (H&E, original magnification ϫ100). CUTIS Do Not Copy Figure 4. Acanthosis nigricans partially regressed after 16 months of treatment. the responsible immunoglobulins stimulate rather insulin resistance syndrome also can be associated than inhibit the signal transducing activity of the with generalized AN. insulin receptor.6 Acanthosis nigricans is a common Although the mechanism of AN occurrence feature of type B insulin resistance, and in patients remains to be elucidated, it has been suggested that with type B insulin resistance, there is a unique peri- hyperinsulinemia causes AN by exerting a toxic ocular distribution of AN that is not seen in other effect. Insulin has been demonstrated to cross the forms of insulin resistance.5 dermoepidermal junction to reach keratinocytes. At Considering the widespread distribution of our low concentrations, it preferentially binds to insu- patient’s lesions as well as the presence of periocu- lin receptors; at high concentrations (as in severe lar AN, he was diagnosed with generalized AN and insulin resistance), insulin substantially binds to type B insulin resistance syndrome. Therefore, type B insulinlike growth factor receptors on keratinocytes WWW.CUTIS.COM VOLUME 86, DECEMBER 2010 301 Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Generalized Acanthosis Nigricans or fibroblasts.7 Binding of insulin to insulinlike growth 5. Arioglu E, Andewelt A, Diabo C, et al. Clinical course factor receptors stimulates the proliferation of kerati- of the syndrome of autoantibodies to the insulin receptor nocytes and fibroblasts, which in turn leads to AN.8,9 (type B insulin resistance): a 28-year perspective. Medicine Fareau et al10 described a patient with regression (Baltimore). 2002;81:87-100. of AN associated with the disappearance of circulat- 6. Gorden P, Collier E, Roach P. Autoimmune mechanisms ing anti–insulin receptor autoantibodies in the set- of insulin resistance and hypoglycemia. In: Moller DE, ed. ting of type B insulin resistance. The patient showed Insulin Resistance. London, England: John Wiley; 1993:123-142. improvement of AN and hypoglycemic symptoms 7. Verrando P, Ortonne JP. Insulin binding properties of nor- after treatment with prednisolone and isotretinoin.10 mal and transformed human epidermal cultured keratino- Retinoids such as isotretinoin have diverse biologic cytes. J Invest Dermatol. 1985;85:328-332. effects. They affect cell growth, differentiation, and 8. Hermanns-Lê T, Scheen A, Piérard GE. Acanthosis nigri- morphogenesis, and alter cell cohesiveness.11 Oral cans associated with insulin resistance: pathophysiology or topical retinoids have been used in the treatment and management. Am J Clin Dermatol. 2004;5:199-203. of AN with varying success.12,13 The mechanism of 9. Cruz PD Jr, Hud JA Jr. Excess insulin binding to insulin-like action of isotretinoin, though not studied directly in growth factor receptors: proposed mechanism for acantho- AN, is probably normalization of epithelial growth sis nigricans. J Invest Dermatol. 1992;98(suppl 6):82S-85S. and differentiation.14 We suggest that this case con- 10. Fareau GG, Maldonado M, Oral E, et
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