结合临床病史和特征性的检查结果通常使得黑棘皮病的诊断非常容易,而一个皮肤活检可能对非典型的病例很有帮助,也可以除外其他需要鉴别的诊断。
识别危险因素
与黑棘皮病非常相关的危险因素包括肥胖、高胰岛素血症、黑棘皮病或糖尿病的阳性家族史、某些遗传性综合征(尤其是那些与高胰岛素血症、肥胖或成纤维细胞生长因子缺乏相关的)以及恶性肿瘤病史。
病史
患者首先发现的症状是色素沉着,表现为受累皮肤看起来发黑或显得很脏。之后表皮会变厚,皮纹明显。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com这些变化通常自颈后开始出现,皮肤软垂疣(较小的良性带蒂肿物)在受损部位经常出现。[20]Houpt KR, Cruz PD. Acanthosis nigricans. In: Freedberg IM, Eisen AZ, Wolff K, et al, eds, Fitzpatrick's dermatology in general medicine, 6th ed. New York, NY; McGraw Hill: 2003: 1796-1801.
肥胖相关黑棘皮病可能伴有体重增加。恶性黑棘皮病常常突然、迅速地发展,还可能出现体重下降。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[9]Moore RL, Devere TS. Epidermal manifestations of internal malignancy. Dermatol Clin. 2008 Jan;26(1):17-29.http://www.ncbi.nlm.nih.gov/pubmed/18023768?tool=bestpractice.com[10]Curth HO. Cancer associated with acanthosis nigricans: a review of the literature and report of a case of acanthosis nigricans with cancer of the breast. Arch Surg. 1943 Dec;47(6):517-52.良性或单侧 AN 患者中可能有阳性家族史。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[5]Berk DR, Spector EB, Bayliss SJ. Familial acanthosis nigricans due to K650T FGFR3 mutation. Arch Dermatol. 2007 Sep;143(9):1153-6.http://www.ncbi.nlm.nih.gov/pubmed/17875876?tool=bestpractice.com[22]Torley D, Bellus GA, Munro CS. Genes, growth factors and acanthosis nigricans. Br J Dermatol. 2002 Dec;147(6):1096-101.http://www.ncbi.nlm.nih.gov/pubmed/12452857?tool=bestpractice.com疾病的发生很少与开始使用一种新的药物相关。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[20]Houpt KR, Cruz PD. Acanthosis nigricans. In: Freedberg IM, Eisen AZ, Wolff K, et al, eds, Fitzpatrick's dermatology in general medicine, 6th ed. New York, NY; McGraw Hill: 2003: 1796-1801.
体格检查
通常表现为在皱褶部位的黄色至深棕色的对称、色素增加的斑块。皮损好发于颈后、腋下、外阴、脐周、大腿内侧以及腹股沟等屈侧部位,通常表现为在皮肤表面出现的黄色至深棕色的对称、色素增加的斑块,[20]Houpt KR, Cruz PD. Acanthosis nigricans. In: Freedberg IM, Eisen AZ, Wolff K, et al, eds, Fitzpatrick's dermatology in general medicine, 6th ed. New York, NY; McGraw Hill: 2003: 1796-1801.
[Figure caption and citation for the preceding image starts]: 一位肥胖的白人女性患者,腋下有黑棘皮病累及由Melvin Chiu,MD提供 [Citation ends].
[Figure caption and citation for the preceding image starts]: 黑棘皮病累及一位肥胖黑人女性的后背及上臂由Miguel Gutierrez,MD提供 [Citation ends].可出现皮赘。患者可能超重或是肥胖。黏膜受累,和在掌跖部位出现角化过度时需要警惕出现恶性黑棘皮病的可能性。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[9]Moore RL, Devere TS. Epidermal manifestations of internal malignancy. Dermatol Clin. 2008 Jan;26(1):17-29.http://www.ncbi.nlm.nih.gov/pubmed/18023768?tool=bestpractice.com[10]Curth HO. Cancer associated with acanthosis nigricans: a review of the literature and report of a case of acanthosis nigricans with cancer of the breast. Arch Surg. 1943 Dec;47(6):517-52.[13]Ramirez-Amador V, Esquivel-Pedraza L, Caballero-Mendoza E, et al. Oral manifestations as a hallmark of malignant acanthosis nigricans. J Oral Pathol Med. 1999 Jul;28(6):278-81.http://www.ncbi.nlm.nih.gov/pubmed/10426202?tool=bestpractice.com广泛累及的皮损可能出现在良性(家族性)或恶性的黑棘皮病当中。本病也可分布在肢端的伸侧表面,但不累及间擦部位。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[11]Schwartz RA. Acral acanthosis nigricans (acral acanthotic anomaly). J Am Acad Dermatol. 2007 Feb;56(2):349-50.http://www.ncbi.nlm.nih.gov/pubmed/17224380?tool=bestpractice.com
恶性肿瘤筛查
如果没有其他已知的原因且迅速、广泛播散累及黏膜表面以及掌跖部位,进行腹部恶性肿瘤的筛查就十分必要(例如在彻底的临床检查后进行腹部CT扫描)。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[11]Schwartz RA. Acral acanthosis nigricans (acral acanthotic anomaly). J Am Acad Dermatol. 2007 Feb;56(2):349-50.http://www.ncbi.nlm.nih.gov/pubmed/17224380?tool=bestpractice.com恶性 AN 可能伴随突然出现的多发性脂溢性角化或全身性瘙痒。[9]Moore RL, Devere TS. Epidermal manifestations of internal malignancy. Dermatol Clin. 2008 Jan;26(1):17-29.http://www.ncbi.nlm.nih.gov/pubmed/18023768?tool=bestpractice.com这些患者通常体形消瘦而不是肥胖,一般年龄都在40岁以上。[2]Schwartz RA. Acanthosis nigricans. J Am Acad Dermatol. 1994 Jul;31(1):1-19.http://www.ncbi.nlm.nih.gov/pubmed/8021347?tool=bestpractice.com[9]Moore RL, Devere TS. Epidermal manifestations of internal malignancy. Dermatol Clin. 2008 Jan;26(1):17-29.http://www.ncbi.nlm.nih.gov/pubmed/18023768?tool=bestpractice.com[10]Curth HO. Cancer associated with acanthosis nigricans: a review of the literature and report of a case of acanthosis nigricans with cancer of the breast. Arch Surg. 1943 Dec;47(6):517-52.
高胰岛素血症的检测
如果患者体型肥胖,为了进行高胰岛素血症的筛查,一般需要进行空腹血糖和胰岛素检测(例如空腹血中胰岛素检测)。[19]Mukhtar Q, Cleverly G, Voorhees RE, et al. Prevalence of acanthosis nigricans and its association with hyperinsulinemia in New Mexico Adolescents. J Adolesc Health. 2001 May;28(5):372-6.http://www.ncbi.nlm.nih.gov/pubmed/11336866?tool=bestpractice.com[20]Houpt KR, Cruz PD. Acanthosis nigricans. In: Freedberg IM, Eisen AZ, Wolff K, et al, eds, Fitzpatrick's dermatology in general medicine, 6th ed. New York, NY; McGraw Hill: 2003: 1796-1801.
皮肤活检及组织学检查
大多数病例中皮肤活检并不是必需的,但是在一些不寻常的病例中或是鉴别诊断中需要除外一些其他疾病时是很有帮助的。