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Diseases of Subcutaneous Fat. Endocrine Diseases.

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Title: Diseases of Subcutaneous Fat. Endocrine Diseases.


1
Diseases of Subcutaneous Fat. Endocrine Diseases.
  • Michael Hohnadel
  • KCOM
  • 1/20/04

2
Diseases of Subcutaneous Fat
  • Panniculitis
  • May appear similar due to depth
  • Two broad categories
  • Septal (classically E.N.)
  • Lobular (Vascular)

3
Erythema Nodosum
  • Acute Erythema Nodosum
  • Crops of bilateral, deep, tender, 1-10 cm
    diameter, red nodules, on pretibial area (or
    other). Lesions resolve over several days or
    weeks, leaving a bruise like area. (erythema
    contusiforme) which resolves without scarring.
  • Fever, malaise, leg edema, arthritis and other
    systemic symptoms may be present
  • Associated states (reactive process)
  • WgtgtM, TB, Streptococcal inf., Yersinia,
    Salmonella, deep fungal infections, Sarcoidosis,
    drug (oral contraceptives). Pregnancy. Many
    others.

4
Erythema Nodosum
  • Chronic Erythema Nodosum
  • Distinguishing features
  • Older women
  • Unilateral, Single lesion which spreads
    centrifugally to form annular plaques. Sometimes
    bilateral.
  • No systemic symptoms ( Possibly arthralgia )
  • Less tender
  • No underlying disease
  • Prolonged course of months / years.
  • Key to diagnosis Pretibial, no systemic
    complaints. Biopsy seldom required.
  • DDX erythema induratum (post. Calf), syphilitic
    gummas (unilateral). Subcutaneous fat necrosis
    assoc with pancreatits ect. systemic.

5
Erythema Nodosum
6
Erythema Nodosum
  • Histologic Features
  • Septal infiltrate of neutrophils (early), other
    mononuclear cells. Histiocytes and Multi Giant
    cells may predominate older lesions. Some foamy
    histiocytes may be seen.
  • Meischers radial granulomas Aggregates of
    histiocytes around stellate clefts is
    characteristic not diagnostic.

7
Erythema Nodosum
Infiltration of inflammatory cells between the
adipocytes
8
Erythema Nodosum
9
Erythema Nodosum
  • Treatment
  • Treat underlying causes.
  • NSAIDS, bed rest / reduced activity
  • Potassium Iodide Increase to control.
  • Watch for hypothyroidism.
  • Intralesional / systemic Steroids
  • Refractory cases anti-malarials, colchicine.
  • Most cases resolve in 3-6 wks.

10
Nodular Vasculitis
  • Tender subcutaneous nodules of calves of middle
    aged, thick legged women. Bilateral, often
    ulcerate and recur over years.
  • Pathology Arteritis, venulitis of septal vessels
    with substantial necrosis of lobular adipocytes
    resulting in suppuration and perforation.
    Granulomatous inflammation.
  • DDX Erythema induratum. TB testing, PCR of
    affected tissue for mycobacterium.
  • Treatment SSKI (50 effective). Other agents
    used in E.N.

11
Nodular Vasculitis
12
Sclerosing Panniculitis(Lipodermatosclerosis)
  • 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.
  • Pathogenesis venous insufficiency with hypoxia
    of center of fat lobule. May not be apparent
    venous disease.
  • Histopathology Necrosis of fat lobules with
    ghost cells (pale cells no nuclei). Foamy
    histiocytes, inflammatory cells and septal
    thickening with fat microcyst.
  • Biopsy should be avoided. Heals poorly. Venous
    eval.
  • TX - Treat venous insufficiency.
  • Compression stockings (may be painful).
  • Stanozolol decreases perivascular fibrin,
    decreases pain in 3 wks and induration in 8 wks.

13
Sclerosing Panniculitis
14
Physical Panniculitis
  • Sclerema Neonatorium
  • 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.
  • Mobility affected by induration.
  • Rapidly fatal.
  • Child is very ill.
  • Histopathology Enlarged adipocytes filled with
    needle like clefts in radial array.
  • TX treat underlying disease.

15
Sclerema Neonatorium
16
Subcutaneous Fat Necrosis of the Newborn.
  • Presentation Asymptomatic, firm, rubbery nodules
    on upper back, buttocks, cheeks and proximal
    extremities. May fuse into plaques which resolve
    over several months without scarring.
  • Occurs during first 4 weeks of life.
  • Child is otherwise healthy
  • Associated with perinatal hypothermia, asphyxia
    and difficult labor and maternal drug use.
  • Rarely, hypercalcemia occurs during the episode.
  • Histopathology Lobular panniculitis with radial
    needle clefts in adipocytes.
  • Fine needle aspiration has been used to DX.

17
Subcutaneous Fat Necrosis of the Newborn.
18
Subcutaneous Fat Necrosis of the Newborn.
19
Cold Panniculitis
  • Synonyms Popsicle / equestrian panniculitis.
  • Presentation Within several days of cold
    exposure, affected skin becomes mildly
    erythematous with nontender firm Sub-Q nodules.
    Face, thighs and scrotum.
  • Typical patients (Hx of cold exposure).
  • Infants and young children. Black gt Whites.
  • Scrotal disease prepubertal, 9-14 yr old male
    who is over weight. Bilateral, painful, no
    transillumination.
  • Resolves in several days to weeks without TX.

20
Cold Panniculitis
21
  • Post Steroid Panniculits
  • Firm sub-Q nodules form within one month of rapid
    withdraw from high dose systemic steroids.
  • Predominantly children.
  • Most cases resolve. May have to restart steroids
    and wean more slowly.
  • Traumatic Panniculitis
  • Trauma, often not recalled, induces a firm mildly
    tender lipoma like sub-Q mass.
  • Heals with fibrosis of septa.

22
  • Factitial Panniculitis
  • Factitial injection of foreign substances into
    the skin. Medical personal are common patients.
  • DX
  • Careful HX. Fits no other pattern.
  • Healing injection site, biopsy revealing foreign
    material, polarized light micro, Mass
    spectroscopy in difficult cases is suspicion is
    high.

23
Sclerosing Lipogranuloma
  • Granulomatous and fibrotic reaction from
    intentional, often cosmetic, injection of mineral
    oils, silicon or guayacol (Mexico).
  • 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.
  • 66 autoimmune finding () ANA, Raynauds,
    Sjogrens ect. 10 connective tissue disease
    usually scleroderma.
  • Histologically Swiss cheese appearance of
    panniculus. Histiocytes with ingested material.
    Fibrosis.

24
Sclerosing Lipogranuloma
2nd to Cosmetic Paraffin injections
25
Sclerosing Lipogranuloma
26
Pancreatic Panniculitis
  • Fat necrosis 2nd to Pancreatitis or pancreatitc
    carcinoma. May be first sign of malignancy (40).
    Digestive enzymes cause damage.
  • Presentation Tender or painless, erythematous
    Sub-q nodules 1-5 cm. In diameter. Lower leg is
    affected 90. Lesions typically number lt10 (may
    be 100s). Involutes with scarring.
  • Assoc findings abd pain (sometimes), arthritis
    (FFA in synovial fluid), pulm. infiltrates.
  • DX pancreatic workup amylase, lipase.
  • Peripheral eosinophila in Pancreatic CA.
  • Histology fat necrosis, Ghost cells. Finely
    stippled Basophilic material (Calcium) with rim
    of necrotic cells. Inflammatory infiltrate at
    periphery.

27
Pancreatic Panniculitis
28
Pancreatic Panniculitis
29
Alpha-1 Antitrypsin Deficiency Panniculitis
  • 12500 of European decent have homozygous
    deficiency of enzyme which inhibits neutrophil
    elastase. The genetic defect results in a
    molecule that cannot be released from its
    production sites in hepatocytes.
  • AAT is 1 of the 3 most common lethal genetic
    diseases among whites.
  • Presentation After minor trauma, painful nodules
    appear on extremities and trunk. Lesions may form
    draining sinuses.
  • MF, 20-40 years.
  • Histopathlogy Dissolution of septae. Islands of
    normal fat floating in spaces of destroyed septae.

30
Alpha-1 Antitrypsin Deficiency Panniculitis
31
Alpha-1 Antitrypsin Deficiency Panniculitis
  • DX
  • Constellation of Liver dysfunction and Pulmonary
    dysfunction with skin lesions
  • Serum AAT levels
  • AAT function analysis
  • TX
  • Replace enzyme (prolastin)
  • Dapsone, doxycycline

32
Cytophagic Histiocytic Panniculitis
  • Multi-system disease with widespread, painful
    sub-q nodules which may break down and form
    ulcerations.
  • Progressive febrile illness with heptomeg,
    pancytopenia, HTG.
  • Etiology Proliferation of histiocytes. Triggered
    by viral infection or 2nd to lymphomas.
  • Benign (-) EBV
  • Malignant () EBV, B or T cell Lymphoma.
  • HIV assoc.
  • Histopathology Infiltration of fat lobules with
    histiocytes with fat necrosis. Bean bag cell
    stuffed with RBC.
  • Clonal B or T cell proliferation malignant.
  • TX Benign - cyclosporine induces permanent
    remissions. Malignant - Chemo or bone marrow.

33
Cytophagic Histiocytic Panniculitis
Subcutaneous nodules with purpura
34
Cytophagic Histiocytic Panniculitis
35
Misc. Panniculitis
  • Eosinophilic Panniculitis
  • Prominent infiltration with eosinphils assoc with
    arthropod bites, parasites, contact derm, Wells,
    bacteria, injections.
  • Gouty Panniculitis
  • Uric acid in Sub-Q leading to fat necrosis.

36
Lipoatrophies
  • Total
  • Partial
  • Centrifugal
  • Semicircular
  • Lipoatrophia Annularis
  • Localized
  • HIV associated

37
Total Lipodystrophy
  • 2 types Congenital vs Acquired.
  • Both are assoc. with D.M. (lipoatrophic diabetes)
  • 1.) Beradinelli - Seip (congenital)
  • Autosomal recessive. Hypermetabolic state.
  • General Paucity of fat at birth with voracious
    appetite, increased height velocity, muscular
    hypertrophy, genital enlargement, mild MR,
    protuberant abdomen, HTG. Hypertrophic
    cardiomyopathy, pul stenosis. DM resistant to
    therapy.
  • Cutaneous AN (often generalized), Hypertrichosis
    with abundant curly scalp hair.
  • Death in young adulthood from DM, cardiac, liver
    dx.
  • TX fenfluramine reduces hypermetabolic state.

38
Beradinelli - Seip (congenital)
39
Congenital Total Lipodystrophy
AN and Loss of Bichats fat pad
40
Total Lipodystrophy
  • 2.) Seip - Lawrence (acquired)
  • Begins lt15 yrs old, often lt5yrs (not at birth)
  • Well defined illness precedes 30 of cases.
  • Presentation As in congenital, but less
    striking. AN with DM is common. Severe liver
    involvement is more common (death). Loss of fat
    may begin local and generalize or start
    generalized.
  • TX etretinate has helped AN in some.

41
Seip - Lawrence (acquired)
42
Partial Lipodystrophy
  • (Inherited Forms)
  • Kobberling-Dunnigan syndrome
  • Autosomal dominate.
  • At puberty adipose tissue is lost from
    extremities, gluteal and truncal areas with fat
    accumulation on the neck and face.
  • DM with AN, HTG, hirsutism, PCOS after age of 20
    years.
  • Other inherited forms
  • Onset early infancy with adipose tissue loss
    localized to the face and buttocks. Rieger
    anomaly tooth and eye abnormalities hypoplasia
    of the iris and iris strands to the peripheral
    cornea.
  • Bone age and dentition are retarded.
  • DM late. No AN.

43
Partial Lipodystrophy
  • (Acquired Forms)
  • Progressive Partial Lipo. (Barraquer-Simons)
  • W gt M, Present first or second decade.
  • After a febrile illness, diffuse and progressive
    loss of fat beginning on face and scalp and
    progressing downward to iliac crest. Sparing of
    LE.
  • No discomfort or inflammation.
  • Low C3 levels are assoc. and proteinuria occurs
    in in 50 of these cases.
  • 3rd trimester intrauterine death may occur.

44
Progressive Partial Lipo.
Loss of buccal fat and AN.
45
Lipoatrophies
  • Centrifugal
  • One type is seen primarily in infants from Japan.
    90 are under 5 years.
  • Centrifugally spreading loss of abdominal fat
    over 3- 8 years with regional lymphadenopathy.
  • Resolves completely after progression stops.
  • Semicircular
  • Adult women affected with single or multiple,
    asymptomatic, symmetric depressions of
    anterolateral thigh.
  • Often after trauma. Resolves in several years.
  • Annular Atrophic panniculitis
  • 10cm band of atrophy, bilaterally, around the
    ankles of children and young adults. Rare.

46
Lipoatrophies
  • Lipoatrophia Annularis
  • Bracelet like constrictions of upper extremities
    1-2 cm wide on women following a period of
    swelling and erythema of extremity.
  • Arthralgias and Pain of affected extremity
  • Persist up to 20 years.
  • Localized lipodystrophy
  • 2nd to injection of medications esp. insulin.
  • HIV assoc. Lipodystrophy.
  • Occurs in effectively Treated Aids Patients with
    reverse transcriptase inhibitors and protease
    inhibitors.
  • Fat redistribution from face, buccal, buttocks
    and limbs is lost to neck , upper back and inter
    abdominal areas.

47
Localized lipodystrophy
Insulin injection lipodystrophy
48
HIV assoc. Lipodystrophy
Loss
49
  • Endocrine diseases

50
Acromegaly
  • Hypersecretion of growth hormone.
  • 2nd to adenoma
  • Changes
  • Diffuse hypertrophy of skin. Reddening and
    wrinkling of forehead.
  • Cutis verticis gyryata in 30
  • Enlargement of hands and feet and tongue.
    Drum-stick fingers.
  • Hypertrichosis, hyperpigmentation, hyperhydrosis.
  • TX transpenoidal microsurgery. Irradiation.
    Octreotide inhibits GH if surgery is not an
    option.
  • 50 of patients completely normalize with TX.

51
Acromegaly
52
Cushings Syndrome
  • Hyperfunctioning of adrenocortical tissue
  • Cushing disease microadenoma of pituitary. 10
  • Hyperfunctioning adrenals/adenomas or other
    tumors account for remainder.
  • Non Iatrogenic W gt M. Peak age 20-30s.
  • Features
  • Obesity of face, neck and trunk. Buffalo trunk.
    Moon face
  • Cutaneous hypertrichosis, dryness, fragility,
    acne, dermatphyte/pityrosporum infections,
    flushing, striae of abdomen and thighs. Thinning
    of skin.
  • Systemic HTN, weakness, reduced bone density,
    DM, atherosclerosis, osteoporosis.

53
Cushings Syndrome
54
Cushings Syndrome
55
Addisons Disease
  • Adrenal insufficiency
  • Increased Pituitary POMC leads to increased ACTH
    and melanocyte stim. hormone.
  • Cutaneous signs
  • Diffuse hyperpigmentation
  • Most prominent in sun exposed areas and sites of
    recent trauma
  • Axillae, perineum and nipples.
  • Darkening of palmar, scars, hair and nails.

56
Addisons Disease
57
(No Transcript)
58
Panhypopituitarism
  • Loss of pituitary hormones 2nd to infections
    (syphilis and TB), tumor, postpartum hemorrhage.
  • Loss of melanin 2nd to decreased ACTH/MSH.
  • Cutaneous signs Thin dry skin. Diffuse loss of
    body hair. Thin, opaque, slow growing nails.
    Light skin susceptible to sunburn. Decrease in
    facial folds.

Fine facial wrinkles and light skin
Diminished axillary hair is an early sign
59
Hypothyroidism
  • Cretinism
  • Insufficient thyroid hormone in fetal life
  • Cool, dry, white to yellow skin. Patchy alopecia.
    Hypohydrosis. Brittle nails. Thick protuberant
    lips. Enlarged tongue. Wide face.
  • Myxedema
  • Systemic mucinosis
  • Rough, dry, dull skin over areas of swelling, esp
    the face. (lips, tongue and nose)
  • Diffuse hair loss and shedding of outer third of
    eyelashes occurs
  • Brittle, coarse nails.

60
Hypothyroidism
Pretibial Myxedema with peau d orange of mucin
deposition
Cretinism
61
Hypothyroidism
  • Mild Hypothyroidism
  • Harder to detect
  • Coldness of hands and feet in absence of vascular
    disease, cool intolerance, hypohydrosis, Weight
    gain, excessive sleeping, constipation.
  • Palmoplantar keratoderma may occur and resolves
    with TX.

62

Hypothyroidism

63
Hypothyroidism
64
Hypothyroidism
Palmoplantar keratoderma
65
Hyperthyroidism
  • Cutaneous changes
  • Warm, moist, smooth textured skin.
  • Thin, downy hair with possible non-scarring
    alopecia.
  • Diffuse pigmentary changes - melanoderma. may
    result in melasma.
  • Plummerss nails concave with distal
    onycholysis.
  • Graves disease (FgtM, onset 20-30 years.)
  • Thyroid Acropachy ( 1 of graves patients)
  • Digital clubbing, diaphyseal proliferation in
    acral and distal bones.
  • Frequently accompanies exophthalmos and pretib.
    myxedema
  • May be seen in euthyroid and hypothyroid pt on
    occasion. Acromegally, Pul. osteoperiostitis.
  • Radiographic findings are pathognomonic.

66
Thyroid Acropachy
67
Hyperthyroidism
  • Pretibial Myxedema
  • 4 of graves patients. Also, during Hashimotos
    thyroiditis and primary hypothyroidism.
  • Bilateral, localized accumulations of
    glycosaminoglycans assoc with thyroid stimulating
    antibodies.
  • Exophthalamous and acropachy assoc.
  • TX
  • intralesional steroids, clobetasol under
    occlusion. (not systemic)
  • IVIG
  • Vitiligo 7 assoc with graves.

68
Hyperthyroidism
69
Hypo-parathyroidism
  • Cutaneous
  • Poor dentition if during development.
  • Dry scaly skin.
  • Scanty hair. Absence of axillary and pubic hair.
  • Brittle malformed nails with onycholysis.
  • 15 of idiopathic cases develop candidisis.
  • APECED syndrome
  • Autoimmune PolyEndocrinopathy, Candidasis,
    External Dystrophy - syndrome.
  • Hypoparathroidism most common endo. Dysfunction
    with this syndrome.

70
Pseudo - hypoparathyroidism
  • Autosomal dominate or X-linked.
  • End organ unresponsiveness to PTH.
  • Elevated PTH and phosphorus. Low calcium.
  • Clinical findings
  • 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
  • Shortened long bones 2nd to premature epiphyseal
    closure.
  • Short stubby toes with metacarpophalangeal
    dimpling (Albrights sign).
  • Albrights hereditary osteodystrophy
  • Includes Pseudo-hypoparathyroidism and
    Pseudo-pseudo PH.
  • Defect in G protein pathway leads to resistance
    to agents acting through adenylate cyclase
    pathway.

71
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).
72
Hyper-Parathyroidism
  • Multiple endocrine neoplasia (MEN-I)
  • Tumors of Parathyroid, pancreas, pituitary,
    thyroid and adrenal glands.
  • Most common abnormality is hypercalcemia from
    the tumors of the parathyroids.
  • Autosomal dominate. Presents in 4th decade.
  • Assoc. with multiple angiofibromas, collaganomas,
    café au lait macules, lipomas, confetti like
    hypopigmentation and gingival macules.
  • Tumors arise 2nd to abnormal tummor suppresor
    genes

73
Hyper-Parathyroidism and MEN-I
Angiofibromas
Collagenomas
Gingival papules
74
Acanthosis Nigricans
  • Hyperpigmentation and papillary hypertrophy in a
    symmetrical distribution. Any area of the body
    may be involved including conjunctiva, lips and
    buccal mucosa. Palms may show hyperkeratosis.
  • Type I Malignancy associated.
  • Preceeds(18), accompanies(60), follow(22)
    malignancy
  • Adenocarcinoma most associated. Esp G.I.
  • Nonobese male gt40 yrs old.

75
Malignant Acanthosis Nigricans
76
Acanthosis Nigricans
  • Tripe Palms thickened velvety palms with
    demoglyphics.
  • 95 assoc with cancer.
  • 77 occurs with AN.
  • If only palms were only presenting sign then lung
    CA most common.
  • If palms and AN then gastric cancer most common.
  • Type II Familial Acanthosis Nigricans.
  • Present at birth or early childhood.
  • No cancer assoc.
  • Accentuated at Puberty.

77
Tripe Palms
78
Acanthosis Nigricans
  • Type III DM assoc.
  • Most common
  • Grayish, velvety thickening of neck, axillae and
    groin.
  • Occurs in obesity and with endocrine disorders
    such as DM, acromegaly, Stein Leventhal,
    Cushing, Addisons, thyroid d/o. Renal transplant
    pts. Many others.
  • Insulin resistance either clinical or subclincal.
  • Drugs nicotinic acid, glucocorticoids,
    diethylstilbestrol, trizineate, BCP.

79
Acanthosis Nigricans
80
Acanthosis Nigricans
  • Histopathology papillomatosis, hyperkeratosis
    and slight hyperpigmentation. Without thickening
    of the Malpighian layer.
  • Darkening of skin 2nd to hyperkeratosis.
  • Treatment
  • Address malignancy, endocrinopathy
  • Weight Loss
  • Lipodystrophic DM improves with fish oil.
  • Etretinate and tretinoin.

81
  • The End
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