Education
SELF-ASSESSMENT MINI-PROGRAM LAMAR S. OSMENT, M.D. From tine Department of Dermatology, Medical College of the University of Alabama, Birmingham, Alabama
A 55-year-old Negro man noticed the development of a velvety, slightly verrucous hyperpigmented change in his axillae, groin and neck for about a year. There was also accentuation of the skin markings and deepening of his skin color in the same areas. The lesions were neither itchy nor painful but he lost his appetite and 10 pounds of weight over the preceding 6 months before he sought medical help. On the patient's first visit to his physician, a biopsy was taken from the affected area. This showed mild hyperkeratosis and papillomatosis with acanthosis. The dermal papillae were thinned, and the dermis contained a scanty chronic inflammatory infiltrate, primarily perivascular. Most of the consultants thought that the work-up should be oriented to a particular group of diseases. 1. At this point in the management of the patient's condition, several laboratory and other diagnostic studies were planned. Which of the following disease groupings do you think would most appropriately be studied in this patient? A. B. C. D.
Avitaminosis Heavy metal intoxication Visceral malignancy Collagen disease
, '
450
An extensive investigation revealed an adenocarcinoma on the greater curvature of the stomach. Gastric resection was performed but hepatic metastases were found at the time of surgery. The fact that carcinoma was present in this case categorizes the cutaneous hypertrophic process as malignant acanthosis nigricans. 2. Which of the following types of acanthosis nigricans (AN) may be ascribed to simple obesity without malignancy? A. B. C. D.
Benign acanthosis nigricans Pseudoacanthosis nigricans Lipoacantbosis nigricans Flexural acanthosis nigricans
Appearance of AN may precede, coincide with or follow the clinical signs of malignancy (usually adenocarcinoma of the stomach). Other adenocarcinomas arising in organs located below the diaphragm may be present in the malignant form of AN. Malignancy when associated with AN may be found in the lungs, thyroid or other supradiaphragmatic organs. Lymphosarcoma has also been found in association. The malignancies are generally very active and invasive and few patients survive the underlying process. AN may occur as part of a congenital syndrome such as Bloom's syndrome, congenital lipodystrophy, insulin resistant diabetes, Rud's syndrome, chondrodystrophy and hepatolenticular degeneration. Endocrine problems, such as
No. 6
SELF-ASSESSMENT MINI-PROGRAM
Osment
451
pituitary tumors and Stein-Leventhal syndrome, may also stimulate the formation of AN. Avitaminosis was not considered a logical diagnostic possibility in this patient since the histopathological picture was more compatible with AN than with simple hyperkeratosis. Hyperpigmentation may be found in many avitaminoses. 3. Which of the following diseases would be least likely to cause darkening of the skin? A. B. C. D.
Vitamin A deficiency Pellagra Kwashiorkor Pantothenic acid deficiency
Fig. 1.
Answers and Explanations to Self-Assessment Mini-Program 1. The answer is C, internal malignancy. From the description of the histopathological changes seen in the biopsy specimen, the diagnosis would most likely be acanthosis nigricans. The epidermis in this condition is hyperplastic in all its layers and is associated with hyperpigmentation. 2. The answer is B, pseudo-acanthosis nigricans. The term "benign" acanthosis nigricans would be used to best describe those patients who were not obese and in whom no malignancy may be found. Answers C and D are not commonly used terms. 3. Pantothenic acid deficiency would not be expected to cause hyperpigmenta-
tion. On theoretical grounds it may, in fact, produce the opposite effect. To act as an enzyme promoting pigment formation copper must be linked chemically to a protein moeity. If pantothenic acid is not present, the linkage is inhibited. References 1. CurttA, H. O., Pigmentary changes of the skin associated witb internat disease. Postgrad. Med. 41:439, 1967. 2. Wheeler, C. E., Abele, D. C, and Briggaman, R. A., Skin manifestations of internal malignancy. Postgrad. Med. 41:494, 1967. 3. Bluefarb, S. M., and Caro, W. A., Pigmentation and internal disease: Part I. Modern Med. 37:83, 1969.
SELF-ASSESSMENT MINI-PROGRAM LAMAR S. OSMENT, M.D. From tine Department of Dermatology, Medical College of the University of Alabama, Birmingham, Alabama
A 55-year-old Negro man noticed the development of a velvety, slightly verrucous hyperpigmented change in his axillae, groin and neck for about a year. There was also accentuation of the skin markings and deepening of his skin color in the same areas. The lesions were neither itchy nor painful but he lost his appetite and 10 pounds of weight over the preceding 6 months before he sought medical help. On the patient's first visit to his physician, a biopsy was taken from the affected area. This showed mild hyperkeratosis and papillomatosis with acanthosis. The dermal papillae were thinned, and the dermis contained a scanty chronic inflammatory infiltrate, primarily perivascular. Most of the consultants thought that the work-up should be oriented to a particular group of diseases. 1. At this point in the management of the patient's condition, several laboratory and other diagnostic studies were planned. Which of the following disease groupings do you think would most appropriately be studied in this patient? A. B. C. D.
Avitaminosis Heavy metal intoxication Visceral malignancy Collagen disease
, '
450
An extensive investigation revealed an adenocarcinoma on the greater curvature of the stomach. Gastric resection was performed but hepatic metastases were found at the time of surgery. The fact that carcinoma was present in this case categorizes the cutaneous hypertrophic process as malignant acanthosis nigricans. 2. Which of the following types of acanthosis nigricans (AN) may be ascribed to simple obesity without malignancy? A. B. C. D.
Benign acanthosis nigricans Pseudoacanthosis nigricans Lipoacantbosis nigricans Flexural acanthosis nigricans
Appearance of AN may precede, coincide with or follow the clinical signs of malignancy (usually adenocarcinoma of the stomach). Other adenocarcinomas arising in organs located below the diaphragm may be present in the malignant form of AN. Malignancy when associated with AN may be found in the lungs, thyroid or other supradiaphragmatic organs. Lymphosarcoma has also been found in association. The malignancies are generally very active and invasive and few patients survive the underlying process. AN may occur as part of a congenital syndrome such as Bloom's syndrome, congenital lipodystrophy, insulin resistant diabetes, Rud's syndrome, chondrodystrophy and hepatolenticular degeneration. Endocrine problems, such as
No. 6
SELF-ASSESSMENT MINI-PROGRAM
Osment
451
pituitary tumors and Stein-Leventhal syndrome, may also stimulate the formation of AN. Avitaminosis was not considered a logical diagnostic possibility in this patient since the histopathological picture was more compatible with AN than with simple hyperkeratosis. Hyperpigmentation may be found in many avitaminoses. 3. Which of the following diseases would be least likely to cause darkening of the skin? A. B. C. D.
Vitamin A deficiency Pellagra Kwashiorkor Pantothenic acid deficiency
Fig. 1.
Answers and Explanations to Self-Assessment Mini-Program 1. The answer is C, internal malignancy. From the description of the histopathological changes seen in the biopsy specimen, the diagnosis would most likely be acanthosis nigricans. The epidermis in this condition is hyperplastic in all its layers and is associated with hyperpigmentation. 2. The answer is B, pseudo-acanthosis nigricans. The term "benign" acanthosis nigricans would be used to best describe those patients who were not obese and in whom no malignancy may be found. Answers C and D are not commonly used terms. 3. Pantothenic acid deficiency would not be expected to cause hyperpigmenta-
tion. On theoretical grounds it may, in fact, produce the opposite effect. To act as an enzyme promoting pigment formation copper must be linked chemically to a protein moeity. If pantothenic acid is not present, the linkage is inhibited. References 1. CurttA, H. O., Pigmentary changes of the skin associated witb internat disease. Postgrad. Med. 41:439, 1967. 2. Wheeler, C. E., Abele, D. C, and Briggaman, R. A., Skin manifestations of internal malignancy. Postgrad. Med. 41:494, 1967. 3. Bluefarb, S. M., and Caro, W. A., Pigmentation and internal disease: Part I. Modern Med. 37:83, 1969.
