Cutaneous Manifestations of Systemic Diseases 428 C2 Notes Dr

Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb CUTANEOUS MANIFESTATIONS OF DIABETES MELLITUS: Specific manifestations: 1Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes 1. Diabetes dermopathy or “SHIN SPOTS”: Most common cutaneous manifestation of diabetes; M > F, males over age 50 years with long standing diabetes. They are: bilateral, symmetrical, atrophic red-brownish macules and patches, over the shins mainly but can occur at any sites, asymptomatic. There is no effective treatment. 2. Necrobiosis Lipoidica Diabeticorum (NLD): Patients classically present with single or multiple red-brown papules, which progress to sharply demarcated yellow-brown atrophic, telangiectatic erythematic plaques with a violaceous, irregular border. Usually it’s unilateral. Common sites include shins followed by ankles, calves, thighs and feet. Very atrophic plaque so any trauma will lead to ulceration, it occurs in about 35% of cases. Cutaneous anesthesia, hypohidrosis and partial alopecia can be found Pathology: Palisading granulomas containing degenerating collagen. The nonenzymatic glycosylation of dermal collagen and elastin will lead to degeneration of the collagen and atrophy (necrobiosis). 2Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Approximately 60% of NLD patients have diabetes and 20% have glucose intolerance. Conversely, up to 3% of diabetics have NLD, so if a patient has NLD its common that he is diabetic, but not every diabetic patient have NLD. (Important) Women are more affected than men. Treatment: Ulcer prevention (by avoiding trauma). No impact of tight glucose control on likelihood of developing NLD. There are multiple treatment options available and all of them reported to be effective: o Intralesional steroids o Systemic aspirin: 300mg/day and dipyridamole: 75 mg/day. o Antiplatelet. o Pentoxyfylline. o Preilesional heparin injection. 3. Acanthosis Nigricans: Hyperpigmentaion and thickening of the skin of the neck. It’s common to see it in any patient, not only in diabetic patients. Causes: Its not only present with diabetes, it can occur in other conditions like: o Obesity. o Insulin resistance. o Endocrinopathy: DM, acromegaly, Cushing syndrome, hypothyroidism, Addison disease and hyperandrogenic state as; PCOS, HAIRAN syndrome (hyperandrogen, insulin resistance, acanthosis nigricans). o Malignancy (esp. GIT, Lung and Breast CA). o Medications (nicotinic acid, niacinamide, testosterone, OCP and Glucocorticoid). Clinical picture: Hyperpigmented velvety plaques of the flexures; the neck, axial, antecubital fossa, popliteal fossa. Other sites include: The face, external genitalia, medial thighs, dorsal joints, lips and umbilicus can be involved in extensive cases. Pathogenesis involves: o Genetic sensitivity of the skin to hyperinsulinemia. o Aberrant keratinocyte and fibroblast proliferation stimulated by excess growth factor (e.g., Insulin like growth factor). 3Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Treatment: Treat the cause: Tight blood glucose control, treatment of underlying malignancy, weight control, and discontinuation of offending agent. 4. Diabetic Bullae OR Bullosis Diabeticorum: Non-inflammatory bullae. Rarest cutaneous complications of diabetes; M > F, long standing diabetics. Trauma and microangiopathy may play a role. Clinical picture: Rapid onset of painless tense blisters on the hands and feet. Pathology: Intraepidermal and/or subepidermal split without acantholysis. DIF is negative, because its not an inflammatory bullae. Treatment: Spontaneous healing in two to five weeks 5. Grannloma Annulare: Clinical picture: Asymptomatic red-purple dome shaped papules arranged in annular configuration. Association between granuloma annulare and diabetes is controversial. Usually when there is multiple granuloma annulare there is more risk of association with DM. Generalized form of GA is the most closely associated with DM, about 20%. It has a chronic and relapsing course. Treatment : ....Intralesional steroid. Systemic steroid. PUVA and many other options. Controlling blood sugar WILL NOT improve GA. 4Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes 6. Scleredema Diabeticorum: Occurs more commonly in type 2 diabetics, long-standing disease, and obese men (M>F). Clinical picture: Painless, symmetric woody “peau d’orange” indurations and thickening of the upper back and neck (Interscapular area), and usually it affect the shoulder motility. No specific treatment is available 7. Cutaneous Infections: Diabetic patients are predisposed to develop cutaneous infections due to poor microcirculation and low immunity: candidal Bacterial Fungal (Tinea pedis) OTHER MANIFESTATIONS OF DIABETES MELLITUS: Pruritus (Itching). Yellow Skin or Carotenosis.  Eruptive Xanthomas: Xanthoma can occur in DM, it is a lipid disposition in the skin. Diabetic neuropathy (peripheral), Neuropathic ulcers. 5Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes CUTANEOUS MANIFESTATIONS OF THYROID DISEASE: (Hyperhidrosis) ** Thyroid acropachy is a cutaneous condition characterized by digital clubbing and swelling of digits and toes. NON-SPECIFIC MANIFESTATIONS OF HYPERTHYROIDISM: Skin: o Warm, and moist. o Localized or generalized hypertrichosis. o Palmar erythema. o Flushing of head/neck, trunk. Hair: o Soft/fine/straight. o Diffuse reversible alopecia (Telogen effluvium). Nails: o Faster rate of growth. o Onycholysis. o Plummer nails: concave deformity with distal onycholysis. Pigmentation: o Focal or generalized hyperpigmentation. 6Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes o Vitiligo. GRAVES Disease: Autoimmune Hyperthyroidism. 1. Thyroid Dermopathy (Pretibial Myxedema): Bilaterally symmetric, non-pitting yellowish-brown to red waxy papules, nodules and plaques on the shins Occur in Graves’s disease. The clinical findings are due to an increase in hyaluronic acid in dermis. Treatment regimens include high potency topical steroids & intralesional steroid. Remember that Pretibial Myxedema that occurs with GRAVES Disease (Autoimmune HYPERthyroidism) is different from the Myxedema that occurs with HYPOthyroidism. 2. Exophthalmoses. 3. Thyroid acrophachy. NON-SPECIFIC MANIFESTATIONS OF HYPOTHYROIDISM: Skin: o Cool, dry, pale. o Xerosis (is an abnormal dryness of the skin or mucus membranes). o Hypohidrosis. o Yellowish hue secondary to carotenemia. o Generalized myxedema: swollen waxy appearance. o Swollen lips, broad nose, macroglossia. o Purpura secondary to impaired wound healing. Hair: o Dry, brittle, coarse. o Increase in percentage of telogen hairs. o Diffuse alopecia. o Loss of lateral third of eyebrow (madarosis). 7Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Thickening of the palm and sole. *Ichthyosis: refers to a relatively uncommon group of skin disorders characterized by the presence of excessive amounts of dry surface scales. CUTANEOUS MANIFESTATIONS OF ADDISON’S DISEASE (HYPOCORTICISM): ADDISON’S DISEASE is a chronic endocrine disorder in which the adrenal glands do not produce sufficient steroid hormones. Clinical picture: Generalized hyperpigmentation that is more prominent in light exposed areas, scars, genitalia, palmar and finger creases, and under the nails. The pigmentation characteristically affects the mucous membranes. It is because of the increased amount of ACTH, which have an action similar to melanocyte-stimulating-hormones (MSH), so it will stimulate melanocytes that will cause pigmentations. (Important) 8Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Loss of pubic and axillary hair in females; androgen-dependent areas (because the androgens hormones are secreted from the adrenal gland). Improvement of acne (The same mechanism as above). Cushing syndrome: Endogenous or exogenous. Deposition of fat over the clavicles and back of the neck” Buffalo hump”. rounded erythematosus face with telangiectasia “Moon face” Trunkal obesity with slender wasting limbs. Striae distensae . Hirsutism, acneform rash, androgenetic alopecia. (Hyperandrogenism). Addisonian-like pigmentation. Easy bruising of the skin on simple trauma. 9Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Buffalo Hump. Striae Distensae. Buffalo Hump. Striae Distensae. , because steroids will cause immunosupression. , monomorphic, no-comedons. Acromegalcy: Skin is oily (because of large sebaceous glands) and wet due to hyperhidrosis with wet hands on hand shaking. The lower lip is thickened, protruded with wide spaced teeth. Large and furrowed tongue. Increased skin pigmentation. Hirsutism. Acanthosis nigricans. 10 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Hyperlipoproteinemia: Type I oooFamilial lipoprotein lipase deficiency (AR) or apoprotein CII deficiency Increased chylomicrons Associated with hepatomegaly, pancreatitis Type IIa Type IIb Type III Type IV Type V ooFamilial hypercholesterolemia, common hypercholesterolemia (AD) Increased LDL ooFamilial hypercholesterolemia (AD) Increased LDL and VLDL ooFamilial Dysbetalipoproteinemia (AR) Increased IDL ooFamilial hypertriglyceridemia (AD) Increased VLDL ooFamilial type V hyperlipoproteinemia, familial lipoprotein lipase deficiency (AD) Increased chylomicrons and VLDL Xanthomatosis: 6 Clinical Types: 1. Tuberous Xanthoma: oFlat or elevated, rounded, grouped, yellowish-orange nodules over joints (particularly elbows and knees) ooTypes II, III, and IV. Biliary cirrhosis. Tuberous xanthomas of the knee. Note the yellowish hue. 2. Tendinous Xanthoma: oPapules or nodules over tendons (extensor tendons on dorsum of hands, feet, and achilles). Types II, III. oTendinous xanthomas of the fingers in a patient with homozygous familial hypercholesterolemia. 11 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Tendinous xanthoma: Linear swelling of the Achilles area representing a tendinous xanthoma in a patient with dysbetalipoproteinemia. 3. Eruptive Xanthoma: Very Important. oSmall multiple yellow/orange/red papules appearing in crops over entire body → buttocks, flexor surfaces, arms, thighs, knees, oral mucosa and may koebnerize. Associated with markedly elevated or abrupt increase in triglycerides (elevated chylomicrons) oooTypes l ,lll , lV , and V It can occur in patients who have Diabetes, obesity, pancreatitis, chronic renal failure, hypothyroidism, estrogen therapy, corticosteroids, isotretinoin, acitretin oEruptive xanthomas. Note the yellowish hue 4. Planar Xanthoma: oFlat macules or slightly elevated plaques, yellow/tan color Associated with biliary cirrhosis, biliary atresia, myeloma, monoclonal gammopathy, lymphoma. Characteristically around eyelids, neck, trunk, shoulders, or axillae Types ll,lll oooPlane xanthoma in a patient with a monoclonal IgG gammopathy 12 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes 5. Palmar Xanthoma: ooNodules and irregular plaques on palms and flexural surfaces of fingers Type III Plane xanthomas of the palmar creases in a patient with dysbetalipoprotenemia (arrows). 6. Xanthelasma: ooooMost common type of xanthoma Eyelids Usually present without any other disease, but can occur in types II and III. Common among women with hepatic or biliary disorders, also seen in myxedema, diabetes. oBest treated with surgical excision. Xanthelasma palpebrarum with typical yellowish hue. Courtesy of Yale Residents Slide Collection. 13 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes CUTANEOUS MANIFESTATIONS OF Inflammatory Bowel Disease (IBD): o Cutaneous and oral ulcerations. <ul style="display: flex;"><li style="flex:1">Association </li><li style="flex:1">Cutaneous Findings </li></ul><ul style="display: flex;"><li style="flex:1">Fissures and Fistulas </li><li style="flex:1">CD > UC </li><li style="flex:1">Commonly involves perineum </li></ul>associated with edema and inflammation <ul style="display: flex;"><li style="flex:1">Oral Crohn’s </li><li style="flex:1">CD </li></ul>CD Edema, cobblestone, ulcerations, nodules <ul style="display: flex;"><li style="flex:1">Metastatic Crohn’s </li><li style="flex:1">Nodules, plaques, ulcerations; </li></ul>commonly on extremities or intertrigenous regions mimics Erythema Nodosum. <ul style="display: flex;"><li style="flex:1">Erythema nodosum </li><li style="flex:1">UC>CD </li><li style="flex:1">Tender red nodules on anterior lower </li></ul>legs; precedes or occurs simultaneous with IBD flare 14 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Pyoderma Gangrenosum UC>CD (PG) Papules, pustules, hemorrhagic blisters → enlarge, ulcerate with dusky undermined edges; exacerbated by trauma; frequently on legs <ul style="display: flex;"><li style="flex:1">Pyoderma Vegetans </li><li style="flex:1">UC </li><li style="flex:1">Vegetating plaques, vesiculopustules of </li></ul>intertrigenous areas; heal with hyperpigmentation; when process involves mucosa =Pyostomatits vegetans <ul style="display: flex;"><li style="flex:1">Chronic Apthous Ulcers </li><li style="flex:1">UC>CD </li><li style="flex:1">Identical to common aphthous </li></ul>ulcers; develop with IBD flares Metastatic Crohn’s disease: The pathology in the skin is exactly the same as in the intestinal lesion. It can present with ulceration or plaque or vesicle nodules. It can occur anywhere in the skin but mainly over androgynous area. Erythema nodosum: Inflammation of SC fat. Clinical picture: Erythematous, tender nodules on anterior shins; also seen on thighs, lateral aspects of lower legs, arms, and face, bilateral, symmetrical. Often accompanied by fever, chills, malaise, and leukocytosis. 15 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes 70% have associated arthropathy, mainly with the knee joints and the ankle joints. Occurs at any age, but most prevalent between 20 and 30 years of age, adolescence and young adult. Results from immunologic reaction triggered by drugs, benign and malignant systemic illness, bacterial, viral, and fungal infections Causes: The most common cause is post-streptococcal infection. (Important) SHOUT BCG: Sarcoid, Sulfa drug, Strep. Histoplasmosis. Oral contraceptives (The most common drug), pregnancy Ulcerative colitis TB Bechet’s Crohn’s GI infections (Yersinia, salmonella) Work-up: o Hx (exclude drugs; sulfa antibiotic and OCP, hx of infection & GI symptoms) o CBC, diff. o ESR o Throat swab (for streptococcal infection, remember it’s the most common cause). o ASO titre; Anti-Streptolysin O (for streptococcal infection too, because it is increased in it). o CXR (for TB). o PPD. o Stool for occult blood. Hisotology: Septal panniculitis without vasculitis. (panniculitis is inflammation of subcutaneous adipose tissue) Treatment: self limiting disease, treat the underlying cause. o Spontaneous resolution usually occurs within three to six weeks without scarring. o NSAIDs such as indomethacin or naproxen. o Rarely do we give systemic steroids; it is effective in severe cases and can be dangerous if infection is etiology. o Potassium iodide 400 to 900 mg daily 16 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Pyoderma Gangrenosum (PG): This is the classical type, the ulcerative type. It present with ulcers, distinct rolled edges and show satellite violaceous papules that break down and fuse with central ulcer. It has violaceous and 1.5-5% of patients with IBD develop PG. Associated with leukemia, myeloma, monoclonaL gammopathy (IgA), polycythemia, chronic active hepatitis, HCV, HIV, SLE & pregnancy Associated with PAPA syndrome → Pyogenic Arthritis, Pyoderma gangrenosum, severe cystic Acne. undermined borders. (Important) May be associated with arthritis. Four Types: o Ulcerative. o Pustular. o Bullous. (Presents with dermatological malignancy; leukemia, myeloma). o Vegetative. (The most mild form) Peristomal Pyoderma Gangrenosum. Histology: Massive dermal edema with epidermal neutrophilic abscesses. Work-up: o CBC diff. o Bone marrow (for hematological malignancy). o GI evaluation. Treatment: o Treat underlying cause. o Potent topical steroids or Intralesional (IL) steroids. o Topical tacrolimus, it is an immunomodulator. o Systemic steroids. o Sulfapyridine, sulfasalzine, and dapsone. o Cyclosporine is the next choice, with 85% of reported cases responding dramatically. o Infliximab. o Other agents: thalidomide, SSKI, azathioprine, cyclophosphamide, chlorambucil. 17 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes CUTANEOUS MANIFESTATIONS OF LIVER DISEASE: Pruritus: generalized itching especially in the presence of biliary obstruction or jaundice. (the itching is because of the bilirubin). Jaundice. Spider naevi: small telangeictatic blood vessels especially on the face and upper chest. Palmar erythema. Diffuse hyperpigmentation. Thinning of the hair and sometime loss of sexual hair in the axillae and pubic areas. Gynaecomastia. (because of hormonal imbalance) Prone to Porphyria cutana tarda. Xanthoma. 18 Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb 428 C2 Notes Hemochromatosis: is a disorder that results in too much iron being absorbed from the gastrointestinal tract, which leads to excessive deposition of iron in the tissues; skin, pancreas, heart. Deposition of iron in the skin will lead to hyperpigmentation. Diffuse bronzing of the skin in hemochromatosis. CUTANEOUS MANIFESTATIONS OF RENAL DISEASE: End stage renal disease (ESRD) and Dialysis: 1. Pruritus: the most common cutaneous manifestation of ESRD. (You have to do Urea and Electrolytes Test and LFT. urea is the cause of pruritus). 2. Half and half (Lindsay’s) nails result from edema of the nail bed and capillary network and give the proximal half of the nail an opaque white appearance, and the distal half will be erythematous. 3. Metastatic Calcification:

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