An amalgamation of different sources as mentioned below as a part of a CME on the topic of acquired elastic tissue disorders. Images of ocean - open access - Google images The original article - Credits -J AM ACAD DERMATOL VOLUME 51, NUMBER 1 Kevan G. Lewis, MS,a Lionel Bercovitch, MD,a Sara W. Dill, MD,a and Leslie Robinson-Bostom, MDa,b Providence, Rhode Island Introductory passage taken from - lever's histopathology Solar elastoses images - dermnetz website

1. ACQUIRED
DISORDERS OF
ELASTIC TISSUE: PART
I.
Presented by : Dr Maitri Patel
PGY1 Dermatology

2. ◦J AM ACAD DERMATOL VOLUME 51, NUMBER
1
◦Kevan G. Lewis, MS,a Lionel Bercovitch, MD,a Sara
W. Dill, MD,a and Leslie Robinson-Bostom, MDa,b
Providence, Rhode Island

5. Histochemical demonstration of elastin fibers within
papillary dermis (A) and reticular dermis (B). Silver
stain. (source : lever histopathology )
Source : lever’s
histopathology
◦ Other special elastic tissue stains are orcein/ resorcin-fuchsin

6. ◦ special elastic tissue stains: orcein/ resorcin-fuchsin.
◦ elastic fibers are thickest in the lower portion of the dermis, where
they are arranged, like collagen bundles, chiefly parallel to the surface
of the skin.
◦ become thinner as they approach the epidermis.
◦ Elastic fibers of the papillary dermis are oriented parallel (elaunin
fibers) or perpendicular (oxytalan) to the dermal-epidermal junction
and are thin and few in number.

7. Schematic diagram of the elastic fiber network showing
oxytalan, elaunin, and elastic fibers.

8. Vertically oriented oxytalan
fibers arise from the
horizontal plexus of elaunin
fibers.
10x magnification

9. elastic stain;
40x magnification
showing a normal
pattern of elastic
tissue.

10. ◦The elastic fibers of the dermis consist of two components:
microfibrils(15%) matrix elastin(85%)

11. ◦elastin stains with elastic tissue stains, is
removable by elastase, and is markedly
extensible,
◦whereas the microfibrils are the elastic resilient
component of the elastic fiber.

12. ◦Elastic fibers undergo significant changes during life.
◦Aging/ Elastotic degeneration d/t chronic sun exposure.
◦young children  microfibril predominate.
◦Physiologic aging  by age 30 - 50 years.
◦Gradual in the no. of peripheral microfibrils / ultimately there
may be none; surface of the elastic fiber  irregular/granular.
◦old persons fragmentation/ disintegration of some of the
elastic fibers.

13. ◦Elastin and microfibrillar glycoproteins  synthesized by
fibroblasts.
◦Elastin is initially secreted as tropoelastin, which is
crosslinked with desmosine to form elastin.
◦Lysyl oxidase, a copper-dependent enzyme deaminates
lysine to form crosslinks in both elastic and collagen fibers.

14. ◦Iatrogenic inhibition of this enzyme occurs with D-
penicillamine, a copper-chelating agent used in the treatment of
Wilson disease.
◦Several acquired elastic tissue disorders have been reported in
association with D-penicillamine therapy;
 elastosis perforans serpiginosa (EPS), cutis laxa,
anetoderma, and pseudoxanthoma elasticum (PXE) like skin
changes.

15. ◦Elastin
◦ metabolized by serine-type elastases, which are secreted by
neutrophils, macrophages, human fibroblasts
◦ degraded by matrix metalloproteinases (MMPs).
◦(MMP-12), (MMP2 and MMP-9), and matrilisin (MMP-7)
◦mid-dermal elastolysis, anetoderma, and granulomatous skin
diseases

16. LATE-ONSET FOCAL DERMAL
ELASTOSIS
◦Definition: Late-onset focal dermal elastosis is characterized
by a PXE-like papular eruption and a focal increase in
normal-appearing elastic tissue.
◦Epidemiology: reported in 4 patients, (65 to 85 years)

17. ◦ ETIOLOGY AND PATHOGENSIS:
◦ disorder of aging / sun-exposed areas
◦ Occurrence of lesions on the neck /antecubital and popliteal fossae  elastic fiber turnover
may be accelerated in these areas mechanical stress.
◦ increased elastin synthesis >> reduction in the degradation process.
◦ CLINICAL FEATURES -Asymptomatic, 1-3-mm yellow papules on extremities
◦ a peau-d’ orange appearance on the neck, thighs, groin, axillae, and antecubital and popliteal
fossae.
◦ H/P : Increased normal-appearing elastic tissue in the mid and deep reticular dermis
(Verhoeffe-van Gieson staining)

19. Late-onset focal dermal elastosis: a
case report and review of the
literature.
Heather J Higgins, Michael Whitworth

20. D/D of Late-onset focal
dermal elastosis
1. elastoma,
2. PXE and pseudoxanthoma-like papillary
dermal elastolysis,
3. white fibrous papulosis of the neck,
4. and linear focal elastosis.

21. ◦H/P/E of elastoma and late-onset focal dermal elastosis overlap
significantly
◦elastomas and other connective tissue nevi typically occur on the
extremities and lower trunk, whereas the papular eruption in late-
onset focal dermal elastosis has been reported in the axillae, groin,
and flexural surfaces of the extremities.
◦Connective tissue nevi often present early in life, while the youngest
reported case of late-onset focal dermal elastosis occurred in a 65-
year-old.

22. ◦may appear similar to PXE clinically, but H/P/E of lesions
reveals the absence of mineralization of elastic fibers that is
characteristic of PXE.
◦Both PXE-like papillary dermal elastolysis and late-onset focal
dermal elastosis present as yellow papules in the flexural surfaces;
◦however, late-onset focal dermal elastosis is characterized by a
focal increase in normal-appearing elastic tissue in the mid and
deep reticular dermis and the absence of any elastolytic change in
the papillary dermis.

23. ◦ White fibrous papulosis of the neck prominent increase in
collagen
V/S
Late-onset focal dermal elastosis the elastic tissue change is
more prominent.
◦ Linear focal elastosis  lower part of the back
V/S
the flexural surfaces and neck  late-onset focal dermal
elastosis.

24. ◦Treatment: There is no known
treatment.

25. LINEAR FOCAL ELASTOSIS
◦Definition.
◦aka elastotic striae, is characterized
◦clinically by yellow palpable, linear plaques
◦and on H/P by an increase in abnormal elastic tissue.

26. Megha Garg1 and Aman Gupta2*
Departments of 1Dermatology and Cosmetology
and 2Pediatrics, MEDENS Hospital, Panchkula, Haryana, India
A 12-year-old boy presented
with asymptomatic linear skin
lesions over the back. There was
no history of exercise, trauma,
excessive or rapid weight gain
or loss, and topical or systemic
drug use. Examination showed
multiple transverse, slightly
elevated, yellow streaks of
varying lengths over back (Fig.
1). A diagnosis of linear focal
elastosis (LFE) was made.
Parents were counselled and no
specific therapy was initiated.

28. ◦Etiology and pathogenesis.
◦cause unknown,
◦occurrence of lesions in sun-protected areas suggest that it is not an
actinic process.
◦Early lesions may appear more ‘‘active’’ and thus grow, appear
erythematous, and at biopsy show elastolysis, whereas older lesions
lack erythema, appear more static, and show elastogenesis.
◦This pattern is s/o degenerative-regenerative process that results in
the eventual formation of elastotic lesions.

29. Clinical manifestations
◦Asymptomatic, horizontal, palpable, yellow or red, indurated,
hypertrophic or atrophic, scaly linear plaques that are
distributed symmetrically about the lumbar-sacral region of
the vertebral column.
◦also been reported on the lower legs and on the face.
◦Asymptomatic

30. H/P
◦ massive, well-demarcated basophilic fibers and increased elastic tissue staining;
◦ elastic fibers in the subpapillary to lower reticular dermis fragmented
◦ EM: fragmentation of elastic tissue / presence of microfibrillar, granular components,
and elastin, aggregated in various stages of maturation.
◦ Elastic fiber microfibrils may appear to be continuous with intracytoplasmic filaments of
fibroblasts, which suggests that elastogenesis is occurring.
◦ Mild perivascular lymphocytic infiltration
◦ Elastin, fibrillin-1, fibrillin-2, and microfibril-associated glycoprotein-1 and -4 may be
decreased in or absent from the papillary dermis of lesional skin.

31. D/D: linear focal elastosis
1. striae distensae,
2. PXE,
3. Dermatofibrosis lenticularis disseminata.

32. ◦ Striae distensae : white, red, or violaceous bands of atrophic, wrinkled skin
◦ Linear focal elastosis and striae distensae may co-exist in the same distribution in
affected individuals.
◦ Ass. with topical and systemic steroid use, pregnancy, and weight gain.
◦ On the axillae, abdomen, thighs, arms, or breasts
◦ H/P/E: flattened epidermis, abnormal collagen fibers, and variable
changes in elastic tissue.
◦ linear focal elastosis is characterized by palpable yellow plaques that
show characteristic changes in elastic tissue; the epidermis and collagen
fibers are not affected.

33. ◦PXE may be differentiated from linear focal elastosis
by the presence of calcified elastic tissue fibers.
◦Dermatofibrosis lenticularis disseminata is
characterized by oval, skin-colored papules rather than
linear yellow plaques, as seen in linear focal
elastosis.

34. ◦Treatment:
◦No effective treatment for linear
focal elastosis has been described.
◦Topical steroid and topical retinoid h
ave shown no benefit [1].

35. ELASTODERMA
◦Definition: characterized by localized skin laxity
and an abundance of elastic tissue in the dermis.

36. Etiology and pathogenesis
◦ Abundance of elastic tissue results from increased synthesis, as
evidenced by the presence of active fibroblasts with prominent rough
endoplasmic reticulum.
◦ in conc.of desmosine, is consistent with an abnormality in elastin
synthesis; this finding could also represent decreased degradation of
elastic tissue.
◦ It may be a localized disorder of elastin synthesis analogous to
morphea as a disorder of collagen.

37. (A) Clinical picture
showing delayed recoil
after stretching the skin
overlying the left elbow.

38. ◦Clinical presentation: Localized areas of pendulous, extensible,
lax, wrinkled skin on the neck, trunk, and arm with markedly
reduced elasticity and delayed recoil.
◦H/P/E : dense deposits of dermal elastic tissue that extend into
the subcutaneous fat.
◦Increased elastic tissue staining is also seen in the papillary and
superficial reticular dermis.
◦Elastic fibers are pleomorphic; both normal-appearing and
abnormal fibers may be seen.

39. B). van Gieson staining
increase in elastic tissue fibres in the reticular dermis with clumping
and fragmentation

40. C) H and E staining revealing an increase in elastic tissue fibres in the
reticular dermis with clumping and fragmentation.

41. ◦EM: grapelike globular structures that contain
abnormal elastic tissue in addition to normal-
appearing elastic fibers;
◦irregular deposits of amorphous elastotic
material along the periphery of elastic fibers
produces a spike-like pattern.

42. Transmission electron microscopy revealing irregular elastic
tissue fibres (arrows), with electronic dense extensions, and
fibroblasts.

43. Tx
◦There is no standard therapy
available for Elastoderma.

44. ELASTOFIBROMA
◦Definition: benign connective tissue disorder
characterized by a subscapular tumor clinically
and prominent elastosis histologically

45. Etiology and pathogenesis
◦Cause unknown
◦Reactive hyperplastic process affects elastin and collagen
fibers  microtrauma during manual labor / excessive
scapulothoracic motion.
◦May be the result of neoelastogenesis, the accumulation of
newly synthesized elastic fibers

46. Clinical manifestations
◦Asymptomatic or mildly tender, slowly growing, solid,
unencapsulated, ill-defined nodule
◦most often deep to the greater rhomboid and latissimus dorsi
muscles and adjacent to the apex of scapula.
◦Lesions on the deltoid muscle; over the ischial tuberosity; near the
greater trochanter; over the olecranon; in the stomach, rectal
submucosa, and greater omentum; on the cornea; and on the foot
have also been observed.
◦Unilateral >>bilateral
◦MRI: poorly defined mass with hypointense internal tracts.

47. H/P/E
◦ Fragmented elastic fibers studded with globular aggregates of elastic
material  serrated appearance to the fibers
◦ Mixture of swollen, eosinophilic collagen fibers intertwined with
elastic fibers, along with fibroblasts and fat cells of varying sizes, is
typically seen.
◦ At low-power magnification, the tumor appears acellular and lacks the
mitotic features of a neoplastic process.
◦ No reports of malignant transformation.

48. Elastofibroma.
A). Linear arrangement of globular,
aggregated elastic fibers (arrow).
H and E stain.

49. Elongated serrated elastic fibers
Elastic stain

50. ◦Aspiration cytology :elongated, beaded, braidlike or fern leaf like
structure s/o deposits of elastic material.
◦Transmission EM: loss of the central elastin core and granular
aggregates of elastic tissue surrounded by microfilaments and
collagen.
◦Scanning EM: shows a ball-of-yarn-like structure composed of
small elastin fibrils.

51. Treatment.
◦Definitive Tx: local excision.
◦As no case of malignant transformation has been reported,
no treatment may be necessary,
◦although an excisional biopsy should be considered in cases
of a high clinical index of suspicion for a malignant process.

52. ELASTOSIS PERFORANS SERPIGINOSA
◦ Hyperkeratotic papules and plaques, trans-epidermal elimination of
abnormal elastic fibers , and focal dermal elastosis.
◦ Etiology and pathogenesis. focal irritation in the dermis  formation of
epidermal and follicular channels to extrude the irritating agent.
◦ D-Penicillamine therapy for Wilson disease, cystinuria and rheumatoid
arthritis have been associated with one subtype of EPS.

53. Pink,
hyperkeratotic
papules and
arcuate plaques.
Elastosis
perforans
serpiginosa.
A).Knee and
B).close-up

54. ◦Clinical manifestations.
◦May present on the face, neck, ear, upper extremities, antecubital
fossae, trunk, or lower extremities, the popliteal fossae as
asymptomatic or pruritic, erythematous or skin-colored
hyperkeratotic papules and plaques with central scaling,
hypopigmentation and atrophy.
◦Symmetric / arranged in a circular or serpiginous pattern /
surrounded satellite lesions.
◦Develop slowly resolve over the course of months or years,
leaving superficial scars.

55. C). extrusion of keratinous, elastotic, and inflammatory
debris
Degenerated eosinophilic elastic fibers

56. "Bramble" is also
used to describe
other prickly shrubs
such as roses.

57. Degenerated elastotic fibers with a ‘‘bramble
bush’’ appearance.

58. ◦H/P/E: Narrow trans-epidermal or perifollicular
perforating canals  extend upward from the dermis
◦contain a mixture of degenerated eosinophilic elastic fibers,
basophilic debris, and inflammatory cells
◦ in the amount and thickness of papillary dermal elastic
tissue.

59. ◦A chronic inflammatory infiltrate with granuloma-forming
multinucleated giant cells may be present.
◦The epidermis may be acanthotic and hyperkeratotic.
◦LM and EM: penicillamine-induced EPS ‘‘lumpy-bumpy’’ /
‘‘bramble-bush’’ appearance of elastic fibers.
◦Elastic tissue changes are observed in both lesional and non-
lesional skin, which distinguish it from other types of EPS

60. ◦type 1—idiopathic EPS without associated
disease;
◦type 2—reactive EPS with associated connective
tissue disease;
◦type 3—penicillamine-induced EPS.

61. ◦25% of cases of EPS occurred in persons with
Down syndrome, Ehlers-Danlos syndrome,
osteogenesis imperfecta, Marfan syndrome and
Rothmund-Thomson syndrome.
◦Additional reports of sporadic cases in patients with
morphea, renal failure, and diabetes mellitus have also
been reported.

62. ◦Treatment.
◦dry ice,
◦cellophane (Scotch) tape stripping,
◦electrodessication and curettage, cryotherapy, intralesional and
topical corticosteroids,
◦calcipotriene ointment, topical tretinoin, oral isotretinoin, topical
tazarotene, glycolic or salicylic acid,
◦NB:UVB, Er:YAG, and CO2 lasers.

63. ◦ACQUIRED PSEUDOXANTHOMA ELASTICUM
◦ Localized, cutaneous form of PXE
◦ Clinically  Papular eruption that leads to lax redundant skin
◦ H/P diffuse mineralization of dermal elastic fibers. In
some cases, perforation +  k/a perforating calcific elastosis
◦Unlike inherited form, acquired PXE  limited to the skin
/ not associated with systemic involvement.

64. Periumbilical perforating PXE.
Abdomen showing coalescing erythematous plaques/
nodules in an arcuate distribution about the umbilicus

65. ◦ Etiology and pathogenesis.
◦ Farmers with a h/o exposure to calcium ammonium-nitrate salts fertilizer.
◦ Lesions  localized to areas exposed to saltpeter  antecubital fossae
◦ The perforating subtype of acquired PXE has been reported predominantly in
the periumbilical area of multiparous women and in patients with severe ascites
or a history of abdominal surgery,  mechanical stress may be important in the
pathogenesis.
◦ Also reported in pts with dialysis-dependent chronic renal failure  abnormal
calcium phosphate metabolism hyperphosphatemia may be a risk factor

66. Clinical manifestations.
• Acquired PXE asymptomatic yellow macules and papules that
coalesce into plaques on the neck, axillae, groin, and flexural surfaces/
periumbilical.
• Thickened/ inelastic  lax and redundant.
• With time tend to resolve, leaving a flat, hyperpigmented, atrophic
central plaque with a raised, scaly border.
• 2 - 15 cm in diameter / circular or oval.

67. ◦H/P/E :
◦Acanthosis  epidermis
◦basophilic staining  elastic fibers in the mid-dermis.
◦Elastic tissue staining  granular, fragmented, curled, frayed,
and thickened calcified elastic fibers in the mid and deep
reticular dermis.
◦In cases of perforation tunnels / channels extrude
elastotic debris from the reticular dermis to the overlying
epidermis.

68. ◦Treatment.
◦In 1 patient, clinical improvement was noted after
fluocinolone acetonide therapy.
◦Apparent regression of a periumbilical plaque was noted in
1 patient with chronic renal failure after normalization of
the serum calcium-phosphate product with hemodialysis.
◦Spontaneous resolution without intervention has also been
reported in 1 patient

69. ◦ELASTOMA
◦aka nevus elasticus
◦diverse group of connective tissue nevi
◦clinically formation of papules or nodules
◦H/P/E a focal increase in normal-appearing
elastic tissue.

70. ◦Epidemiology.
◦Children>> second and third decades
◦Dubreuilh elastoma, has also been reported in older persons in
association with actinically damaged skin.
◦Etiology and pathogenesis.
◦Congenital>>>>>>Acquired
◦Connective tissue nevus /embryonic dysgenesis of dermal
mesenchyme, a developmental defect in which elastic tissue.
◦may be the result of a degenerative process

71. Thigh showing firm, ill-defined
intradermal nodules of elastoma

72. Increased thickened, tortuous elastic fibers in the mid and
deep reticular dermis.

73. ◦Treatment.
There is no known treatment
for elastoma.

74. SOLAR ELASTOTIC
DERMATOSES
◦Solar elastosis refers to the H/P changes in dermal elastic
tissue associated with prolonged sun exposure and includes a
variety of clinical manifestations.
◦the unifying feature of solar elastotic dermatoses is UV
radiation as the presumed cause.
◦We shall discuss them in brief.

77. ELASTOTIC
NODULES OF THE
EAR

78. small firm warty or
pearly papules on
the sides of the
index fingers and
thumbs in patients
who have had a lot
of exposure to the
sun.
Keratoelastoidosis
marginalis

79. Favre´-Racouchot syndrome.
A, Nasofacial sulcus showing multiple punctate, waxy, noninflamed open comedones.
B, Dilated follicular infundibula with small infundibular cysts filled with lamellar keratin and lined by squamous
epithelium. Foci of solar elastosis are present in the superficial dermis.

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