Dermatology 5th year, 3rd lecture (Dr. Faraedon Kaftan)

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The lecture has been given on Apr. 10th, 2011 by Dr. Faraedon Kaftan.

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Dermatologic Signs of Systemic Disease 5th year Lecture 8 By Dr. Faraedon Kaftan Consultant Dermatologist College of Medicine University of Sulaimani Nov. 2011

Dermatologic Signs of Systemic Diseases5th yearLecture By Dr. Faraedon KaftanConsultant DermatologistCollege of MedicineUniversity of Sulaimani April. 10th2011The skin is often a window to systemic disease.By recognizing cutaneous manifestations of systemic diseases, the internist can often determine the appropriate diagnosis and therapy or the need for referral to a dermatologist.1- Common cutaneous disorders (Seb D, Seb K, Urt,EM, V, EN)2- Blistering diseases (P, Pd, EBA)3- Internal malignancies (Cut. Metastases, Pag. Dis, AN, Amyloid)4- Cardiovascular disease (PXE, EDS)5- Pulmonary disease (Sarcoidosis)6- Rheumatic disease (Ps. A, LE, Sclerod., DM )7- Gastrointestinal disease (DH, ACE, LP )8- Renal disease (Nephrogenic fibrosing dermopathy) 9- Endocrine and metabolic disease (Porphyria and Diabetes Mellitus-Related Skin Conditions)1- Common cutaneous disorders Seborrheic dermatitis may be associated with systemic disease, such as 1-Parkinson's disease2- (HIV: Human immunodeficiency virus ) infection. 3- (CVA: cerebrovascular accident ) can develop unilateral seborrheic dermatitis on the scalp corresponding to the affected hemisphere, (The pathophysiology of this phenomenon is not completely understood.)

Seborrheic Keratoses

the most common benign cutaneous neoplasms, are warty, age-related hyperkeratotic papules and plaques that appear anywhere on the body, most commonly the trunk. (Sign of Leser-Trlat):Rarely, if seborrheic keratoses appear suddenly in great numbers indicate an underlying adenocarcinoma of the GI tractUrticaria or Hivesare pruritic, edematous, evanescent wheals that resolve within 24 hours.Acute urticaria typically lasts less than 6 weeks. is most often caused by: 1- Medication (commonly penicillin or other antibiotics, sulfa drugs, aspirin) 2- Food (shellfish, nuts, chocolate), 3- infection: less often

Erythema Multiforme (EM)Is a cutaneous hypersensitivity reaction characterized by Iris (Target) lesions on the face, hands and feet. is usually caused by: 1- Infection (herpes simplex virus or Mycoplasma pneumoniae) 2- and less commonly by drug sensitivity (sulfonamides, barbiturates, antibiotics).

Vitiligocommonly occurs in periorificial areas (mouth, orbits, vagina, anus) or at sites of trauma (hands, elbows, knees). Vitiligo is often associated with: 1- Autoimmune thyroid disease, 2- diabetes mellitus (Insulin-dependent) 3- Pernicious anemia, or 4- Addison's disease.

Erythema Nodosum (EN)Painful, erythematous nodules on the shins and occasionally elsewhere, in young women, between 20 and 40 years, lasting an average of 3 to 6 weeks.Causes of EN: 3 S + Tb1- Streptococcal pharyngitis is the most common . 2- Tuberculosis, 3- (GI) infections with Yersinia, Salmonella, or Shigella, and 4- Systemic fungal infections. 5- Less common causes: drug sensitivity (sulfonamides, salicylates, iodides, oral contraceptives or hormone replacement therapy), and a variety of systemic diseases, most often inflammatory bowel disease (Crohn's disease more than ulcerative colitis) and sarcoidosis.

2- Blistering diseasesPemphigus Vulgarispainful mucosal erosions and flaccid skin blisters (bullae) that become erosive. occurs in the 40th to 60th of life, Mortality is significant, even with treatment.

Bullous Pemphigoidcharacterized by large, tense subepidermal blisters, which are often pruritic. Mucosal disease is rare.in the elderly, 65 and 75 years of age.

Epidermolysis Bullosa Acquisita: EBAuncommon bullous disease characterized by skin fragility, milia (small cysts), scarring alopecia, and nail dystrophy. primarily on the hands, feet, elbows, and knees.EBA may be associated with other autoimmune diseases, most frequently inflammatory bowel disease.

3- Internal malignancies

Cutaneous MetastasesAny malignant neoplasm can metastasize to the skin. in men: Cutaneous metastases are from cancers of the lung, large intestine, and kidney.in women: Cutaneous metastases are from cancers of the breast and large intestine.Flesh-colored to violaceous nodules in close proximity to the primary neoplasm; Most common sites are the - Head (scalp), - Neck, and - Trunk.

Paget's Disease1- Mammary Paget's disease: Unilateral eczematous plaque of the nipple and areola. - is strongly associated with an underlying invasive Ca. of the breast

2- Extramammary Paget's disease: * persistent, eczematous plaque of the anogenital or axillary regions: * is often associated with an underlying - adnexal (apocrine gland) carcinoma or - cancer of the genitourinary tract or distal GIT.

Acanthosis Nigricans (ANs)smooth, velvet-like, hyperkeratotic plaques in intertriginous areas (e.g., groin, axillae, neck).Type I (Malignant AN): is associated with malignancy. May be adenocarcinoma of the GI tract (60% gastric) has a sudden onset and more extensive distribution, including the face, palms, and trunk. Type III AN is associated with obesity and insulin resistance and is the most commonly occurring type.AN can develop following the use of drugs: systemic Cs, nicotinic acid, diethylstilbestrol, and isoniazid (INH).

AmyloidosisPurpuric and Ecchymotic Papules on the eyelids and extremities due to amyloid infiltration in the vessels. may be a sign of multiple myeloma.

4- Cardiovascular diseasePseudoxanthoma Elasticum: PXEBrittle (Broken)and calcified elastic fibers. Yellow papules over redundant skin folds on the neck, abdomen, and groin, giving the skin the appearance of plucked chicken skin. Angioid streaks in Bruch's membrane of the eye.Associated signs of PXE : 1- Hypertension (HTN) 2- Peripheral vascular and coronary artery disease 3- Retinal and GI hemorrhage4- Stroke.

Ehlers-Danlos Syndrome: EDSabnormalities in collagen biosynthesis, which can affect multiorgansJoint hyperextensibility, Hypermobility (Hyperelasticity) Skin and Vessel fragility, and fish-mouth scars. * (7-11) types of EDS identified associated with: mitral valve prolapse, blue sclerae, vascular aneurysm, aortic dissection, hernias, angina, GI bleeding (perforation), and peripheral vascular disease. * Patients with vascular (type IV) EDS are prone to arterial rupture and have the highest mortality.

EDS

5- Pulmonary diseaseSarcoidosisis a multisystem, granulomatous disease of the lungs, bones, CNS, lymph nodes, eyes, and skin. more in women and in African Americans.

Skin disease, affecting 25-35% of patients, includes (Lupus pernio): Red to purple indurated plaques of the nose midfacial papules annular plaques or nodules on the trunk and extremities. predilection for scars. Erythema nodosum is the most common manifestation.

6- Rheumatic diseasePsoriatic Arthritis: (PsA)Affects 5-10% of patients with psoriasis. Asymmetric fusiform swelling of the distal interphalangeal joints (sausage digits) in 70%.

Lupus Erythematosus: DLE, SLE & SCLE(DLE) usually localized to the head or neck, is characterized by atrophic, scarring plaques on sun-exposed areas. 5% of patients develop SLE.

SLEcutaneous manifestations include Malar erythema (The butterfly rash): is the most common expression of SLE.PhotosensitivityOral ulcersDiscoid plaques Bullae Purpura Calcinosis cutis alopecia.

systemic or generalized SclerodermaAre of 2 forms:1- CREST syndrome: (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). - has a better prognosis

2- Progressive systemic sclerosis.

DermatomyositisSymmetric proximal muscle weakness (myositis); photosensitivity; Heliotrope: periorbital edema with a violaceous hue. - Gottron's papules: papules and plaques on the hands, elbows, and knees and Telangiectatic plaques with atrophy and hypopigmentation (poikiloderma) on the face, neck, trunk, and extremities; Nail abnormalities (periungual telangiectases and cuticular hypertrophy).In adults: Dermatomyositis has a strong association with neoplasm of the breast, GIT, or lung.

7- Gastrointestinal diseaseDermatitis Herpetiformis (DH)Acrodermatitis Enteropathica (ACE)Necrolytic Migratory Erythema (glucagonoma syndrome)Lichen planus (LP)Hereditary Hemorrhagic Telangiectasia (HHT)Peutz-Jeghers SyndromePyoderma Gangrenosum

Dermatitis Herpetiformis (DH)is a chronic, intensely pruritic blistering disease Most patients have an asymptomatic gluten-sensitive enteropathy less commonly thyroid disease.

Acrodermatitis Enteropathica (ACE)Is an inherited or acquired condition due to zinc deficiency. Acral and periorificial: pustules, bullae and scaling.Most patients have diarrhea.Treatment is zinc supplementation.

Necrolytic Migratory Erythema(glucagonoma syndrome)is rare, characterized by erythematous, scaly plaques on Acral, Periorificial or Intertriginous areas + hyperglycemia, diarrhea, weight loss, and atrophic glossitis.Is in association with an islet cell tumor of the pancreas. Treatment is rmoval of the tumor.

Lichen planusviolaceous, flat, polygonal papules on the flexor aspects of the wrists, trunk, medial thighs, genitalia, and oral mucosa. May occur with: - Primary biliary cirrhosis - Hepatitis B virus immunization or - Gold therapy - In hepatitis C patients: Oral erosive lichen planus.

Hereditary Hemorrhagic Telangiectasiais an autosomal dominant disorder characterized by numerous telangiectases on the skin and oral mucosa. Recurrent epistaxis is the most common (85 - 90%) presenting manifestation. Telangiectases can involve the lungs, liver, brain, eyes, and GIT hemorrhage can occur at any site. Pulmonary arteriovenous fistulae and central nervous system angiomas