Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb Cutaneous Manifestations of systemic diseases Dr. Eman Almukhadeb CUTANEOUS MANIFESTATIONS OF DIABETES MELLITUS: Specific manifestations: 1 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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). 2 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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). 3 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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. 4 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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: Diabetic neuropathy (peripheral), Neuropathic Xanthoma can occur in DM, it is a ulcers. lipid disposition in the skin. 5 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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. 6 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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). 7 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb * 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) 8 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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. 9 Cutaneous Manifestations of systemic diseases 428 C2 Notes Dr. Eman Almukhadeb 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 428 C2 Notes Dr. Eman Almukhadeb Hyperlipoproteinemia: Type I o Familial lipoprotein lipase deficiency (AR) or apoprotein CII deficiency o Increased chylomicrons o Associated with hepatomegaly, pancreatitis Type IIa o Familial hypercholesterolemia, common hypercholesterolemia (AD) o Increased LDL Type IIb o Familial hypercholesterolemia (AD) o Increased LDL and VLDL Type III o Familial Dysbetalipoproteinemia (AR) o Increased IDL Type IV o Familial hypertriglyceridemia (AD) o Increased VLDL Type V o Familial type V hyperlipoproteinemia, familial