boards’ fodder

Dermatologic Manifestations of Underlying Endocrinopathy Sharon E. Jacob, M.D., Christopher J. Ballard, B.S., & Claudia Vogel, M.D.

Disorder Cutaneous Manifestations Systemic Manifestations Lab Abnormalities

Acromegaly Acanthosis nigricans; Acral growth; Broad nose; u GH, u IGF-1 Acrochordons (skin tags); Carpal tunnel syndrome; u Calcium in urine Coarsened face with Colonic polyps; Deep voice; Oral glucose tolerance test: accentuated creases; Galactorrhea; ; HTN; failure of GH production to v Cutis vertices gyrate; Hypogonadism; Joint , Doughy skin; ; Organomegaly; Pre-pubertal (does not affect gigantism; Thick lips; beard); ; Oily skin; Prognathism; Visual Δ; Thick and hard nails Widened teeth spaces

Addison’s Disease Auricular calcification; Hair may u ACTH, u K+ (Adrenocortical darken; Hyperpigmentation; v BP; Postural dizziness; v Na+, u Ca+2 insufficiency) Longitudinal pigmented bands in Salt craving Metabolic acidosis nails; Loss of body hair GI symptoms: Abdominal ; (especially axillae); Mucosal ; Constipation; Diarrhea; Eosinophilia pigmentation; Pigmented hand Nausea; Vomiting creases; Pigmented scars and Muscle nevi; Vitiligo Cushing’s Acanthosis nigricans; ; Abdominal pain; Amenorrhea; Urinary Free Cortisol > 3x Syndrome Broad purple striae; “Cigarette Impotence; Buffalo hump; normal, loss of normal diurnal paper” wrinkling dorsal hands Central ; Exophthalmos; rhythm in ACTH and Cortisol and elbows (Liddle sign — can Facial plethora; HTN; secretion. peel off with tape); Cutis Psychological changes; Moon Failure of Cortisol to v after marmorata; Easy bruising; facies; from Dexamethasone suppression Hirsuitism; Telangiectasia; kyphosis and osteoporosis; test. Thin dermis and epidermis; Weakness Glucose intolerance u Calcium in v Wound healing urine, u renal stones Cushing’s Disease Acne As above; u ACTH, v K+, u Free Cortisol (Pituitary excess Tumor mass effect: visual field Urinary free Cortisol suppression of ACTH) Hyperpigmentation loss & headache after high-dose Dexamethasone (Differentiate from ectopic ACTH secretion: no cortisol suppression after high dose Dexamethasone) Mellitus Acanthosis nigricans; ; ; ; FPG > 126 mg/dl Bullous diabeticorum; Retinopathy; Nephropathy; RPG > 200 mg/dl Type 1: Diabetic Dermopathy (pretibial neuropathy: foot drop; postural Autoimmune, lack pigmented patches); hypotesion; resting tachycardia; Glucose in urine if glucose level of secretion Foot Ulcers; Frequent fungal gastroparesis; erectile >180 mg/dl , especially Candida; dysfunction; Charcot’s Type 2: Furuncles; Nail bed arthorpathy; cardiovascular Abnormal A1C, abnormal lipids, Insulin resistance telangiectasia; Necrobiosis disease; candidiasis and hypertriglyceridemia, Obesity lipoidica diabeticorum; Pedal usnusual infections. microalbuminuria. Normal insulin petechial purpura; Rubeosis faciei; Scleredema adultorum; Tight, thick, waxy skin; Vitiligo; Xanthomas; Yellow skin and nails Graves’ Disease Acropachy (distal clubbing, ; Diarrhea; Goiter; Heat u Free T4, v TSH, u FTI, u T3 () swelling and periosteal intolerance; ; Positive antibodies thickening); Diffuse alopecia; Opthalmopathy; Pretibial Palmar erythema; Plummer’s nail myxedema; Tachycardia; Tremor; — onycholysis; Skin: Weight loss moist (u sweating) & warm (u cutaneous blood flow); Vitiligo Hashimoto’s v Body Hair; Dry, brittle, coarse, If Hypothyroid: Bradycardia; v Free T4, v Total T4, u TSH, Thyroiditis and slow growing hair; Fine Cold intolerance; Constipation; u Serum cholesterol wrinkling; Loss of outer 1/3 of Cretinism (in children): severe + TPO AB eyebrow (madarosis); Malar flush; mental retardation, impaired Myxedema (mucopolysaccha- growth; Menstrual irregularities; rides) in hands and periorbital; Protuberant abdomen; Nails: brittle, slow growing; Patchy Somnolence; , goiter, and diffuse ; Thin, cold, thyroid antibodies pale, dry skin irectionsin DResidency p. 6 • Winter 2006 boards’ fodder

Disorder Cutaneous Manifestations Systemic Manifestations Lab Abnormalities

Hyperparathyroidism Deposition of Calcium : 1°: u PTH, u Ca+2 in serum and Pruritus ; urine, v serum Phosphorus, Renal calculi u urinary Phosphorus 2°: u PTH, v serum Ca+2, u serum Phosphorus Hypoparathyroidism Hair: coarse, sparse Altered dentition: defective v PTH, v Ca+2, u Phosphorus Nails: opaque and brittle with enamel/ dental hypoplasia; EKG Changes: prolonged Q-T transverse ridges Chvostek’s sign — contraction intervals and T-wave changes Skin: dry, hyperkeratotic, scaly, of muscle by tapping along and puffy facial nerve (sign of tetany) Loss of body hair; Pale skin Amenorrhea; Impotence; v ACTH, v Cortisol, v LH and FSH (mucous membranes normal Infertility; v TSH, v GH, v IGF-1 color); Scalp hair is fine, dry, & If pituitary tumor: u Prolactint galactorrhea thin; v Sebaceous secretions and visual changes (diplopia, and sweating; Thin skint fine reading problems, field loss); Symptoms will change wrinkling around eyes and Lack of energy depending on the hormonal mouth deficiency

McCune-Albright Large irregular Coast of Maine- Hyperthyroidism; Polyostotic GNAS1 gene ; Syndrome like Café-au-lait macules fibrous dysplasia; u GH from pituitary (CALM) Precocious puberty u Serum Alkaline Phosphatase u Urinary Hydroxyproline MEN I Facial angiofibromas; Tumors or of: MEN1 gene 11q13 (defect menin) (Wermer Syndrome) Collagenomas, Confetti Pituitary; Parathyroid & u Calcium, u PTH macules; CALM; Lipomas Pancreas u Prolactintamenorrhea, Autosomal Dominant GastrinomatZ-E Syndrome galactorrhea Papillary CA Thyroid u GHt u ACTHtCushing’s syndrome

MEN IIa Lichen/ Macular amyloidosis Parathyroid (hyperplasia or 10q11.2 RET (point mutation) (Sipple Syndrome) interscapular tumor) u Calcitonin, u PTH, u Ca+2 Autosomal Dominant Thyroid: Medullary CA MEN IIb (III) Inverted lids (from thick corneal Pheochromocytoma 10q11.2 RET (missense mutation) (Wagenmann- nerve); Marfanoid habitus; Thyroid: Medullary CA u Calcitonin, u Catecholamines, Froebose) Multiple mucosal neuromas or VMA, and Metanephrines ganglioneuromas, CALM Autosomal Dominant Pheochromocytoma Flushing of face and forehead; u BP (10% malignant); u Catecholamines, VMA, and Redness and cyanosis of the Headaches; Palpitations; Metanephrines hands; Sympathetic Crisis; Tremor u Sweating Polycystic Ovarian Acanthosis Nigricans; Acne; Amenorrhea u LH, v FSH Syndrome Androgenic alopecia; Hirsutism; (or oligo-menorrhea) u Testosterone (Androgens) Nipple, perineum, axillae Obesity u DHEA, hyperinsulinemia Sharon E. Jacob, M.D., hyperpigmentation; Thickened Precocious puberty and coarse pubic & axillary hair Short stature is an Assistant Professor children Polyeystic ovaries of Clinical Dermatology, Director of the Contact

Abbreviations: Dermatitis Clinic and +2 Δ= change; ACTH: Adrenocorticotropic Hormone; BP: Blood Pressure; CA: Carcinoma; Ca : Calcium; CALM: Café-au-lait macules; DHEA: Dehydroepiandrosterone; EKG: Medical Student Electrocardiogram; FPG: Fasting Plasma Glucose; FSH: Follicle-Stimulating Hormone; FTI: Free Thyroxine Index; GH: ; GI: Gastrointestinal; HbA1c: Hemoglobin A1c; HTN: ; IGF-1: Insulin-like Growth Factor-1; K+: Potassium; LH: Luteinizing Hormone; MEN: Multiple Endocrine Neoplasia; Na+: Sodium; PTH: Parathyroid Hormone; Education, Department RPG: Random Plasma Glucose; TPO AB: Thyroperoxidase Antibodies; TSH: Thyroid-Stimulating Hormone; VMA: Vanillylmandelic Acid; Z-E: Zollinger-Ellison of Dermatology and References Cutaneous Surgery, 1. Jabbour SA. Cutaneous manifestations of endocrine disorders. Am J Clin Dermatol. 2003; 4(5): 315-331. 2. Bolognia JL, Jorizzo JL, Rapini RP. Dermatology. New York: Elsevier Limited, 2003. University of Miami, 3. Braunwald E, Fauci AS, Kasper DL et al., eds. Harrison’s Principles of Internal Medicine, 15th ed. New York: McGraw-Hill, 2001. School of Medicine, 4. Cecil RL, Goldman L (ed), Bennett JC (ed). Cecil Textbook of Medicine, 21st ed. Philadelphia: W. B. Saunders, 2000. 5. Freedberg IM, et al., eds. Fitzpatrick’s Dermatology in General Medicine, 5th ed. New York: McGraw-Hill, 1999. Miami, Fla. 6. Francis S. Greenspan, Gardner. Basic & Clinical , Sixth edition; San Francisco: Lange Medical books/McGraw-Hill, 2001

irectionsin Winter 2006 • p. 7 DResidency