Diseases of Subcutaneous Fat. Endocrine Diseases

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<ul><li> 1. Diseases of Subcutaneous Fat. Endocrine Diseases. Ben Adams KCOM 5-11-05 </li> <li> 2. Diseases of Subcutaneous Fat <ul><li>Panniculitis </li></ul><ul><li>May appear similar due to depth </li></ul><ul><li>Two broad categories: </li></ul><ul><li><ul><li>Septal (classically E.N.) </li></ul></li></ul><ul><li><ul><li>Lobular (Vascular) </li></ul></li></ul></li> <li> 3. Erythema Nodosum <ul><li>Acute Erythema Nodosum </li></ul><ul><li><ul><li>Crops of bilateral, deep, tender, 1-10 cm diameter, red nodules, on pretibial area (or other). </li></ul></li></ul><ul><li><ul><li>Lesions resolve over several days or weeks, leaving a bruise like area. (erythema contusiforme) which resolves without scarring. </li></ul></li></ul><ul><li><ul><li>Fever, malaise, leg edema, arthritis and other systemic symptoms may be present </li></ul></li></ul><ul><li><ul><li>Associated states (reactive process) </li></ul></li></ul><ul><li><ul><li><ul><li>W&gt;&gt;M, TB, Streptococcal inf., Mycoplasma, Coccidioidomycosis, Yersinia, Salmonella, deep fungal infections, Sarcoidosis, drug (oral contraceptives). Pregnancy. Many others. </li></ul></li></ul></li></ul><ul><li><ul><li>Idiopathic 35-55% of cases </li></ul></li></ul></li> <li> 4. Erythema Nodosum </li> <li> 5. Erythema Nodosum </li> <li> 6. Findings suggestive of a systemic cause for Erythema Nodosum <ul><li>Synovitis </li></ul><ul><li>Diarrhea </li></ul><ul><li>Abnormal chest X-ray </li></ul><ul><li>Preceding upper respiratory tract infection </li></ul><ul><li>Elevated anti-Dnase B </li></ul><ul><li>Positive tuberculin skin test </li></ul></li> <li> 7. Erythema Nodosum <ul><li>Chronic Erythema Nodosum </li></ul><ul><li>Distinguishing features: </li></ul><ul><li><ul><li>Older women </li></ul></li></ul><ul><li><ul><li>Unilateral, Single lesion which spreads centrifugally to form annular plaques. Sometimes bilateral. </li></ul></li></ul><ul><li><ul><li>No systemic symptoms ( Possibly arthralgia ) </li></ul></li></ul><ul><li><ul><li>Less tender </li></ul></li></ul><ul><li><ul><li>No underlying disease </li></ul></li></ul><ul><li><ul><li>Prolonged course of months / years. </li></ul></li></ul><ul><li>Key to diagnosis: Pretibial, no systemic complaints. Biopsy seldom required. </li></ul><ul><li>DDX: erythema induratum (post. Calf), syphilitic gummas (unilateral). Subcutaneous fat necrosis assoc with pancreatits etc. systemic. </li></ul></li> <li> 8. Erythema Nodosum <ul><li>Histologic Features </li></ul><ul><li><ul><li>Septal infiltrate of neutrophils (early), other mononuclear cells. Histiocytes and Multinucleated Giant cells may predominate older lesions. Some foamy histiocytes may be seen. </li></ul></li></ul><ul><li><ul><li>Meischers radial granulomas: Aggregates of histiocytes around stellate clefts is characteristic not diagnostic. </li></ul></li></ul></li> <li> 9. Erythema Nodosum Septa are widened and edematous, and infiltrated by lymphocytes and neutrophils </li> <li> 10. Erythema Nodosum Miescher's microgranulomas within septa. </li> <li> 11. Erythema Nodosum <ul><li>Treatment: </li></ul><ul><li><ul><li>Treat underlying causes. </li></ul></li></ul><ul><li><ul><li>NSAIDS, bed rest / reduced activity </li></ul></li></ul><ul><li><ul><li>Potassium Iodide: Increase to control. </li></ul></li></ul><ul><li><ul><li><ul><li>Watch for hypothyroidism. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>Reports of EN triggered by SSKI </li></ul></li></ul></li></ul><ul><li><ul><li>Intralesional / systemic Steroids </li></ul></li></ul><ul><li><ul><li>Refractory cases: anti-malarials, colchicine. </li></ul></li></ul><ul><li><ul><li>Most cases resolve in 3-6 wks. </li></ul></li></ul></li> <li> 12. Nodular Vasculitis <ul><li>AKA: Erythema Induratum </li></ul><ul><li>Tender subcutaneous nodules of calves of middle aged, thick legged women. Bilateral, often ulcerate and recur over years. </li></ul><ul><li>Pathology: Arteritis, venulitis of septal vessels with substantial necrosis of lobular adipocytes resulting in suppuration and perforation. Granulomatous inflammation. </li></ul><ul><li>DDX: Erythema induratum. TB testing, PCR of affected tissue for mycobacterium. </li></ul><ul><li>Treatment: SSKI (50% effective). Other agents used in E.N. </li></ul></li> <li> 13. Nodular Vasculitis <ul><li>Nodular lesions on the lower leg with evidence of ulceration </li></ul></li> <li> 14. Sclerosing Panniculitis (Lipodermatosclerosis) <ul><li>Presentation: Development of woody induration of lower calves (esp. left) with appearance of inverted champagne bottle. Induration begins near ankles and slowly progresses proximally . Most common W &gt; 40 years. May be painful. </li></ul><ul><li>Pathogenesis: venous insufficiency with hypoxia of center of fat lobule. May not be apparent venous disease. </li></ul><ul><li>Histopathology: Necrosis of fat lobules with ghost cells (pale cells no nuclei). Foamy histiocytes, inflammatory cells and septal thickening with fat microcyst. </li></ul><ul><li>Biopsy should be avoided. Heals poorly. Venous eval. </li></ul><ul><li>TX - Treat venous insufficiency. </li></ul><ul><li><ul><li>Compression stockings (may be painful). </li></ul></li></ul><ul><li><ul><li>Stanozolol decreases perivascular fibrin, decreases pain in 3 wks and induration in 8 wks. </li></ul></li></ul></li> <li> 15. Sclerosing Panniculitis </li> <li> 16. Sclerosing Panniculitis <ul><li>Lipomembranous change, consisting of cystic formation with elaborate papillary configurations </li></ul></li> <li> 17. Physical Panniculitis <ul><li>Sclerema Neonatorium </li></ul><ul><li>Presentation: Gravely ill, premature neonates skin begins to harden on buttocks and LE and rapidly spreads to involve entire body except palms, soles and genitalia. </li></ul><ul><li><ul><li>Mobility affected by induration. </li></ul></li></ul><ul><li><ul><li>Rapidly fatal. </li></ul></li></ul><ul><li><ul><li>Child is very ill. </li></ul></li></ul><ul><li>Histopathology: Enlarged adipocytes filled with needle like clefts in radial array. </li></ul><ul><li>TX: treat underlying disease. </li></ul></li> <li> 18. Sclerema Neonatorium Needle-shaped clefts within lipocytes, in the absence of inflammation. </li> <li> 19. Subcutaneous Fat Necrosis of the Newborn. <ul><li>Presentation: Asymptomatic, firm, rubbery nodules on upper back, buttocks, cheeks and proximal extremities. May fuse into plaques which resolve over several months without scarring. </li></ul><ul><li><ul><li>Occurs during first 4 weeks of life. </li></ul></li></ul><ul><li><ul><li>Child is otherwise healthy </li></ul></li></ul><ul><li><ul><li>Associated with perinatal hypothermia, asphyxia and difficult labor and maternal drug use. </li></ul></li></ul><ul><li><ul><li>Rarely, hypercalcemia occurs during the episode. </li></ul></li></ul><ul><li>Histopathology: Lobular panniculitis with radial needle clefts in adipocytes. </li></ul><ul><li>Fine needle aspiration has been used to DX. </li></ul></li> <li> 20. Subcutaneous Fat Necrosis of the Newborn. <ul><li>Indurated plaques on the trunk </li></ul></li> <li> 21. Subcutaneous Fat Necrosis of the Newborn. Needle-shaped clefts in radial configuration are present within giant cells. </li> <li> 22. Cold Panniculitis <ul><li>Synonyms: Popsicle / equestrian panniculitis. </li></ul><ul><li>Presentation: Within several days of cold exposure, affected skin becomes mildly erythematous with nontender firm Sub-Q nodules. Face, thighs and scrotum. </li></ul><ul><li>Typical patients: ( Hx of cold exposure ). </li></ul><ul><li><ul><li>Infants and young children. Black &gt; Whites. </li></ul></li></ul><ul><li><ul><li>Scrotal disease: prepubertal, 9-14 yr old male who is over weight. Bilateral, painful, no transillumination. </li></ul></li></ul><ul><li>Resolves in several days to weeks without TX. </li></ul></li> <li> 23. Cold Panniculitis Erythematous, violaceous plaques on the thighs in a case of equestrian cold panniculitis. </li> <li> 24. <ul><li>Post Steroid Panniculits </li></ul><ul><li>Firm sub-Q nodules form within one month of rapid withdraw from high dose systemic steroids. </li></ul><ul><li>Predominantly children. </li></ul><ul><li>Most cases resolve. May have to restart steroids and wean more slowly. </li></ul><ul><li>Traumatic Panniculitis </li></ul><ul><li>Trauma, often not recalled, induces a firm mildly tender lipoma like sub-Q mass. </li></ul><ul><li>Heals with fibrosis of septa. </li></ul></li> <li> 25. <ul><li>Factitial Panniculitis </li></ul><ul><li>Factitial injection of foreign substances into the skin. Medical personal are common patients. </li></ul><ul><li>DX: </li></ul><ul><li><ul><li>Careful HX. Fits no other pattern. </li></ul></li></ul><ul><li><ul><li>Healing injection site, biopsy revealing foreign material, polarized light micro, Mass spectroscopy in difficult cases is suspicion is high. </li></ul></li></ul></li> <li> 26. Sclerosing Lipogranuloma <ul><li>Granulomatous and fibrotic reaction from intentional, often cosmetic, injection of mineral oils, silicon or guayacol (Mexico). </li></ul><ul><li>Presentation: Up to ten years after implantation, skin becomes erythematous, hyperpigmented and indurated with a lumpy quality of sub-q. Due to migration of material, features may extend beyond the implantation site. </li></ul><ul><li>66% autoimmune finding (+) ANA, Raynauds, Sjogrens ect. 10% connective tissue disease usually scleroderma. </li></ul><ul><li>Histologically: Swiss cheese appearance of panniculus. Histiocytes with ingested material. Fibrosis. </li></ul></li> <li> 27. Sclerosing Lipogranuloma 2 nd to Cosmetic Paraffin injections </li> <li> 28. Sclerosing Lipogranuloma Oil granuloma . Numerous vacuolated spaces, in this case due to grease gun injury. </li> <li> 29. Pancreatic Panniculitis <ul><li>Fat necrosis 2 nd to Pancreatitis or pancreatic carcinoma. May be first sign of malignancy (40%). Digestive enzymes cause damage. </li></ul><ul><li>Presentation: Tender or painless, erythematous Sub-q nodules 1-5 cm. In diameter. Lower leg is affected 90%. Lesions typically number M, onset 20-30 years.) </li></ul><ul><li><ul><li>Thyroid Acropachy ( 1% of graves patients) </li></ul></li></ul><ul><li><ul><li><ul><li>Digital clubbing, diaphyseal proliferation in acral and distal bones. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>Frequently accompanies exophthalmos and pretib. myxedema </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>May be seen in euthyroid and hypothyroid pt on occasion. Acromegally, Pul. osteoperiostitis. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>Radiographic findings are pathognomonic. </li></ul></li></ul></li></ul></li> <li> 67. Thyroid Acropachy </li> <li> 68. Hyperthyroidism <ul><li>Pretibial Myxedema </li></ul><ul><li><ul><li>4% of graves patients. Also, during Hashimotos thyroiditis and primary hypothyroidism. </li></ul></li></ul><ul><li><ul><li>Bilateral, localized accumulations of glycosaminoglycans assoc with thyroid stimulating antibodies. </li></ul></li></ul><ul><li><ul><li>Exophthalamous and acropachy assoc. </li></ul></li></ul><ul><li><ul><li>TX: </li></ul></li></ul><ul><li><ul><li><ul><li>intralesional steroids, clobetasol under occlusion. (not systemic) </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>IVIG </li></ul></li></ul></li></ul><ul><li>Vitiligo 7 % assoc with graves. </li></ul></li> <li> 69. Hyperthyroidism </li> <li> 70. Hypo -parathyroidism <ul><li>Cutaneous: </li></ul><ul><li><ul><li>Poor dentition if during development. </li></ul></li></ul><ul><li><ul><li>Dry scaly skin. </li></ul></li></ul><ul><li><ul><li>Scanty hair. Absence of axillary and pubic hair. </li></ul></li></ul><ul><li><ul><li>Brittle malformed nails with onycholysis. </li></ul></li></ul><ul><li><ul><li>15 % of idiopathic cases develop candidisis. </li></ul></li></ul><ul><li><ul><li>APECED syndrome: </li></ul></li></ul><ul><li><ul><li><ul><li>Autoimmune PolyEndocrinopathy, Candidasis, External Dystrophy - syndrome. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>Hypoparathroidism most common endo. Dysfunction with this syndrome. </li></ul></li></ul></li></ul></li> <li> 71. Pseudo - hypoparathyroidism <ul><li>Autosomal dominate or X-linked. </li></ul><ul><li>End organ unresponsiveness to PTH . </li></ul><ul><li>Elevated PTH and phosphorus. Low calcium. </li></ul><ul><li>Clinical findings: </li></ul><ul><li><ul><li>Short stature, obesity, round face, prominent forehead, low nasal bridge, attached ear lobes, short neck, short wide nails, delayed dentition, mental deficiency, blue sclera and cataracts. Sub-Q calcifications </li></ul></li></ul><ul><li><ul><li>Shortened long bones 2 nd to premature epiphyseal closure. </li></ul></li></ul><ul><li><ul><li><ul><li>Short stubby toes with metacarpophalangeal dimpling (Albrights sign). </li></ul></li></ul></li></ul><ul><li><ul><li>Albrights hereditary osteodystrophy </li></ul></li></ul><ul><li><ul><li><ul><li>Includes Pseudo-hypoparathyroidism and Pseudo-pseudo PH. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>Defect in G protein pathway leads to resistance to agents acting through adenylate cyclase pathway. </li></ul></li></ul></li></ul></li> <li> 72. Albrights Hereditary Osteodystrophy Features of Albright Hereditary Osteodystrophy (AHO). [A] Young woman with short stature (~ 3rd centile), disproportionate shortening of the limbs , generalized obesity, and round, flattened face. [B] Radiograph of the hand showing the shortened 4th and 5th metacarpals. [C] Fist with the characteristic ' dimples ' over the 3rd, 4th, and 5th digits replacing the knuckles formed by the distal head of normally sized metacarpal bones (Archibald sign). [D] Brachydactyly of the hand, with the short 4th and 5th digits, the greatly foreshortened terminal 1st digit, and very short, wide thumbnail (potter's thumb). (Reproduced from Levine, 2000, with permission). </li> <li> 73. Hyper -Parathyroidism <ul><li>Multiple endocrine neoplasia (MEN-I) </li></ul><ul><li><ul><li>Tumors of Parathyroid, pancreas, pituitary, thyroid and adrenal glands. </li></ul></li></ul><ul><li><ul><li>Most common abnormality is hypercalcemia from the tumors of the parathyroids. </li></ul></li></ul><ul><li><ul><li>Autosomal dominate. Presents in 4 th decade. </li></ul></li></ul><ul><li><ul><li>Assoc. with multiple angiofibromas, collaganomas, caf au lait macules, lipomas, confetti like hypopigmentation and gingival macules. </li></ul></li></ul><ul><li><ul><li>Tumors arise 2 nd to abnormal tummor suppresor genes </li></ul></li></ul></li> <li> 74. Hyper -Parathyroidism and MEN-I Angiofibromas Collagenomas Gingival papules </li> <li> 75. Acanthosis Nigricans <ul><li>Hyperpigmentation and papillary hypertrophy in a symmetrical distribution. </li></ul><ul><li>Any area of the body may be involved including conjunctiva, lips and buccal mucosa. Palms may show hyperkeratosis. </li></ul><ul><li>Type I : Malignancy associated. </li></ul><ul><li><ul><li>Preceeds(18%), accompanies(60%), follow(22%) malignancy </li></ul></li></ul><ul><li><ul><li>Adenocarcinoma most associated. Esp G.I. </li></ul></li></ul><ul><li><ul><li><ul><li>Nonobese male &gt;40 yrs old. </li></ul></li></ul></li></ul></li> <li> 76. Malignant Acanthosis Nigricans </li> <li> 77. Acanthosis Nigricans <ul><li><ul><li>Tripe Palms thickened velvety palms with demoglyphics. </li></ul></li></ul><ul><li><ul><li><ul><li>95 % assoc with cancer. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>77% occurs with AN. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>If only palms were only presenting sign then lung CA most common. </li></ul></li></ul></li></ul><ul><li><ul><li><ul><li>If palms and AN then gastric cancer most common. </li></ul></li></ul></li></ul><ul><li>Type II : Familial Acanthosis Nigricans. </li></ul><ul><li><ul><li>Present at birth or early childhood. </li></ul></li></ul><ul><li><ul><li>No cancer assoc. </li></ul></li></ul><ul><li><ul><li>Accentuated at Puberty. </li></ul></li></ul></li> <li> 78. Tripe Palms </li> <li> 79. Acanthosis Nigricans <ul><li>Type III: DM assoc. </li></ul><ul><li><ul><li>Most common </li></ul></li></ul><ul><li><ul><li>Grayish, velvety thickening of neck, axillae and groin. </li></ul></li></ul><ul><li><ul><li>Occurs in obesity and with endocrine disorders such as DM, acromegaly, Stein Leventhal, Cushing, Addisons, thyroid d/o. Renal transplant pts. Many others. </li></ul></li></ul><ul><li><ul><li>Insulin resistance either clinical or subclincal. </li></ul></li></ul><ul><li><ul><li>Drugs: nicotinic acid, glucocorticoids, diethylstilbestrol, trizineate, BCP. </li></ul></li></ul></li> <li> 80. Acanthosis Nigricans </li> <li> 81. Acanthosis Nigricans <ul><li>Histopathology: papillomatosis, hyperkeratosis and slight hyperpigmentation. Without thickening of the Malpighian layer. </li></ul><ul><li><ul><li>Darkening of skin 2 nd to hyperkeratosis. </li></ul></li></ul><ul><li>Treatment </li></ul><ul><li><u