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Connective tissue disorders

6  Non-tumorous skin diseases

6.8  Connective tissue disorders

6.8.1  Lupus erythematosus

Etiology, pathogenesis:

  • multisystem autoimmune disease (systemic LE)
  • variety of autoantibodies, especially against double stranded DNA
  • immune complex formation, which trigger inflammatory reactions

Clinical signs:

  • acute (or subacute) forms are serious, involve other organs (esp. kidneys)
  • fatigue, fever
  • chronic cutaneous form (chronic discoid LE, CDE) is limited to the skin: red, slightly raised lesions above the neck; butterfly rash is typical
  • photosensitivity

Pictures

Another chapter on lupus erythematosus with images is available at:

6.8.1.1  Lupus erythematodes, acute

Clinical signs:

  • acute (and subacute) lupus erythematodes is dangerous (affects other organs, esp. kidneys)
  • skin: face, scalp, fingers
  • photosensitivity
  • maculopapulous exanthema, generalised
  • butterfly exanthema on the cheeks
  • at the same time lesions of CDE, ev. oral ulcerations
  • defluvium, diffuse scarring alopecia
  • vasculitic and hyperkeratotic lesions of the fingers, Raynaud's phenomenon
  • purpura, urticaria, panniculitis, ulcerations, pigment disorders etc.

Pictures

SLE, butterfly exanthema:
Systemic lupus erythematodes acute, CLINIC (925)

Acute SLE, butterfly exanthema:
Systemic lupus erythematodes, CLINIC (995)

SLE, butterfly exanthema, discoid:
Discoid lupus erythematodes chronic, CLINIC (993)

Histology:

Vacuolar degeneration of the basal cell layer; perivascular and periadnexal lymphocytic infiltration. The infiltration in the acute erythematodes are usually present neutrohils as well. Fibrosis; occasionally vasculitis.

Immunofluorescence: deposits of all kinds of immune complexes and c3 along the dermo-epidermal junction (also in clinically uninvolved skin); rarely anti-Ro and anti-La antibodies among the cells of the basal layer (subacute LE, SLE, Sjögren's syndrome).

6.8.1.2  Acute neonatal lupus erythematodes

Clinical signs:

Affects newborns (more often girls), whose mothers have Ro/SSA antibodies. The symptoms are not visible after the delivery, but appear during the first weeks of life.

  • rare
  • affects 1% of mothers with lupus erythematosus
  • affects the skin, kidneys, liver
  • systemic lupus erythematosus in mother may appear later
  • skin lesions dissappear, but damage of the heart may be permanent (arrythmia)

Etiology:

IgG antibodies against Ro (SSA) a La (SSB) pass through the placenta and may cause lupus erythematosus in the child. Similar condition may occurr in other autoimmune diseases (Sjögren's syndrome, rheumatoid arthritis).

Histology:

Histological picture correspond to acute lupus erythematosus.

Pictures

Lupus erythematodes of a newborn. In this case the diagnosis was not evident at first, the child has received blood transfusions and there was suspition of sepsis with circulating immature white blood and red blood cells. Immunohistological reaction to myoglobin was negative, immature white blood cells were shown by myeloperoxidase reaction. The basal membrane of the epidermis focally damaged and the amount of mucin in the corium was slightly increased. The mother had increased levels of serum antibodies:
Lupus erythematodes, acute, congenital, HE 60x (6277)
Lupus erythematodes, acute, congenital, PAS 60x (6280)
Lupus erythematodes, acute, congenital, alcian blue 60x (6274)
Lupus erythematodes, acute, congenital, Halle 60x (6276)
Lupus erythematodes, acute, congenital, myeloperoxidase 60x (6279)
Lupus erythematodes, acute, congenital, hemoglobin 60x (6278)
Lupus erythematodes, acute, congenital, CD20 60x (6275)

6.8.1.3  Lupus erythematodes, subacute

Clinical signs:

  • locations: areas of insolation, symmetric exanthema of the dorsal parts of the extremities
  • red, annular, multiform or papulosquamous lesions
  • severe photosensitivity
  • systemic lesions are mild: CNS, joints, kidneys
  • associated with Sjögren's syndrome

Pictures

Annular lesions of the subacute lupus erythematosus:
Lupus erythematodes, subacute, CLINIC (1078)

SCLE, subacute lupus erythematosus of the skin:
Lupus erythematodes, subacute, CLINIC (1079)

Histology:

Vacuolar degeneration of the basal layer; perivascular and periadnexal lymphocytic infiltrate; fibrosis; sometimes vasculitis.

Immunofluorescence: deposits of various kinds of immunocomplexes and C3 along dermo-epidermal junction (the deposits are present in clinically uninvolved skin as well). Direct immunofluorescence sometimes show anti-Ro and anti-La antibodies between the cells of the basal layer (SLE, subacute LE, Sjögren's syndrome).

6.8.1.4  Lupus erythematodes, chronic

Clinical signs:

  • insolated areas: face, ears, scalp, neckline
  • red, targetoid lesions if fresh
  • firmly adhering squames
  • follicular hyperkeratosis
  • heals with depigmented scars
  • reactive hyperpigmentations on the periphery

Histology:

Vacuolar degeneration of the basal layer; perivascular and periadnexal lymphocytic infiltrate; fibrosis; sometimes vasculitis. Follicular plugging, atrophy and/or dysmaturation of the epidermis, thickenning of the basement membrane, lymphocytic periadnexal and perivascular infiltrate.

Immunofluorescence: deposits of various kinds of immunocomplexes and C3 along dermo-epidermal junction (the deposits are present in clinically uninvolved skin as well). Direct immunofluorescence sometimes show anti-Ro and anti-La antibodies between the cells of the basal layer (SLE, subacute LE, Sjögren's syndrome).

Direct immunofluorescence sometimes show anti-Ro and anti-La antibodies between the cells of the basal layer (SLE, subacute LE, Sjögren's syndrome).

Pictures

Lupus erythematodes, chronic:
Chronic lupus erythematosus of the skin, HE 10x (197)

Another case:
Discoid lupus erythematodes chronic, HE 100x (2523)

Lesion of CDE form the face after long lasting corticosteroid treatment:
Chronic lupus erythematosus with epidermal atrophy, HE 40x (196)

Another case:
Chronic lupus erythemathosus of the skin, HE 20x (194)
Chronic lupus erythemathosus of the skin, HE 40x (195)

Another case:
Chronic lupus erythematodes of the skin, HE 5x (192)
Chronic lupus erythematodes of the skin, HE 20x (190)
Chronic lupus erythematodes of the skin, HE 40x (191)

Another case:
Discoid lupus erythematodes chronic, HE 40x (6137)

Another case:
Chronic erythematodes of the skin, HE 40x (193)

Another case:
Discoid lupus erythematodes chronic, HE 40x (2706)

Another case:
Discoid lupus erythematodes chronic, HE 20x (5354)

Another case, with dense lymphocytic infiltrates:
Discoid lupus erythematodes chronic, HE 40x (3654)

Direct fluorescence in chronic lupus erythematosus (anti-IgM, FITC):
Chronic lupus erythematodes of the skin, anti-IgM, FITC, Evans 20x (470)
Chronic lupus erythematodes of the skin, anti-IgM, FITC, Evans 40x (471)
Chronic lupus erythematodes of the skin, anti-fibrin, FITC, Evans 10x (535)
Chronic discoid lupus erythematodes, anti-IgM, FITC, Evans 20x (488)
Chronic discoid lupus erythematodes, anti-IgM, FITC, Evans 40x (489)
Lupus erythematodes, anti-IgG, FITC 20x (570)
Lupus erythematodes, anti-IgG, FITC 40x (571)

Alopecia, CDE
Chronic lupus erythematodes, alopecia, HE 10x (490)
Chronic lupus erythematodes, alopecia, HE 10x (491)
Chronic lupus erythematodes, alopecia, HE 40x (492)

6.8.1.5  Lupus erythematodes profundus

Pictures

Lupus erythematosus cutaneus profundus:
Discoid lupus erythematodes chronic, CLINIC (990)

Histology:

Form of chronic lupus erythematosus with dense lymphocytic infiltrates of the deep dermis. Epidermal changes similar to CDE are not present.

Amount of the dermal mucin is increased. Rarely granulomatous infiltrate is present.

6.8.2  Dermatomyositis

Clinical signs:

  • location: most often face, hands, trunk
  • heliotropic periorbital erythema
  • Gottron's papules over metacarpophalangeal joints
  • erythemas of the trunk and extremities
  • hyperkeratoses of lateral aspects of the forefinger, so called sign of a mechanic

Histology:

The picture is that of lupus erythematosus; epidermis is normal or atrophic; dermal edema and increased acid mucopolysaccharides; slight perivascular lymphocytic infiltrate. Old lesion sometimes calcify.

Pictures

Dermatomyositis:
Dermatomyositis, HE 40x (2272)
Dermatomyositis, HE 100x (2270)
Dermatomyositis, HE 100x (2271)

The same case, skeletal muscle biopsy:
Dermatomyositis, muscle, HE 40x (2274)
Dermatomyositis, muscle, acid phosphatase 40x (2275)
Dermatomyositis, muscle, MAC 40x (2273)

Another case, with focal atrophy of the epidermis:
Dermatomyositis, HE 40x (4075)

Another case of dermatomyositis, immunofluorescence:
Dermatomyositis, HE 40x (3480)
Dermatomyositis, HE 40x (3479)
Dermatomyositis, anti-fibrin, FITC 20x (3481)
Dermatomyositis, anti-IgM, FITC 20x (3482)
Dermatomyositis, anti-fibrin, FITC 40x (3483)

Paraneoplastic dermatomyositis, skin, muscle and low differentiated serous cystadenocarcinoma of the ovary:
Dermatomyositis, paraneoplastic, HE 40x (6220)
Dermatomyositis, paraneoplastic, HE 40x (6221)
Dermatomyositis, paraneoplastic, HE 40x (6227)
Dermatomyositis, paraneoplastic, NADH 20x (6226)
Dermatomyositis, paraneoplastic, ATP 20x (6222)
Dermatomyositis, paraneoplastic, HLA 20x (6223)
Dermatomyositis, paraneoplastic, acid phosphatase 20x (6224)
Dermatomyositis, paraneoplastic, MAC 20x (6225)
Cystadenocarcinoma ovarii, serous, HE 40x (74054)

Another case of dermatomyositis, immunofluorescence:
Dermatomyositis, HE 40x (3513)
Dermatomyositis, HE 40x (3514)
Dermatomyositis, anti-IgG, FITC 20x (3516)
Dermatomyositis necalcifying, early, anti-IgG, FITC 20x (3517)
Dermatomyositis necalcifying, early, anti-IgM, FITC 20x (3518)
Dermatomyositis necalcifying, early, anti-IgG, FITC 20x (3519)

Dermatomyositis, widespread calcifications:
Dermatomyositis, calcifications, HE 10x (204)

Dermatomyositis:
Dermatomyositis, HE 20x (4228)  [zoomify]

Dermatomyositis:
Dermatomyositis, HE 40x (4886)  [zoomify]

Dermatomyositis, acute, with edema of papillary dermis:
Dermatomyositis early, HE 20x (4452)

6.8.3  Osteoarthritis, Heberden nodes

Clinical signs:

Osteophytes in the distal interphalangeal joints in osteoarthritis are called Heberden nodes; Bouchard nodes affect proximal interphalangeal joints.

6.8.4  Scleroderma, morphea

Introduction:

Two forms exist: diffuse and localized.

Etiology, pathogenesis:

  • autoimmune disease: antinucleolar and anticentromeral antibodies
6.8.4.1  Diffuse scleroderma

Clinical signs:

  • diffuse sclerosis of the skin, disorders of the connective tissue of inner organs and joints
  • hypomimia, microstomia with radial wrinkles
  • sklerodactyly
  • calcifications, teleangiectasiae
  • vasculitis, loss of adnexa
  • multisystemic disease, in addition to the skin (sclerodactyly, Raynaud's phenomenon, changes of the skin of the face, subcutaneous calcifications) the disease affects many other organs: oesophagus, lungs (fibrosis), kidneys, joints
  • CREST syndrome: Calcinosis cutis, Raynaud's phenomenon, Esophageal dysfunction (dysfagia), Sclerodactyly, Teleangiectasia
  • usually slow progression, the course is variable

Pictures

Sclerodermia diffusa, teleangiectasias, mask-like face:
Scleroderma, CLINIC (821)

Sclerodermia diffusa, sclerodactyly, contractures, vasculitis:
Scleroderma, CLINIC (822)
Scleroderma, CLINIC (1082)

Sclerodermia diffusa, face:
Scleroderma, CLINIC (1083)

Sclerodermia diffusa, face, teleangiectasiae:
Scleroderma, CLINIC (1086)

Skleroderma, hand:
Scleroderma, hand, CLINIC (1980)

Hands, diffuse scleroderma:
Scleroderma, CLINIC (5865)
Scleroderma, CLINIC (5866)
Scleroderma, CLINIC (5867)
Scleroderma, CLINIC (5868)
Scleroderma, CLINIC (5869)

Systemic sclerosis, sclerodermia diffusa:
Scleroderma, CLINIC (3330)
Scleroderma, CLINIC (3331)
Scleroderma, CLINIC (3332)
Scleroderma, CLINIC (3333)
Scleroderma, CLINIC (3334)
Scleroderma, CLINIC (3335)

Further examples of systemic sclerosis:

6.8.4.2  Localized scleroderma, morphea

Clinical signs:

  • localized scleroderma — morphea —  roundish lesions, usually on the trunk
  • linear scleroderma: linear scars of the head and extremities
  • red-brown borders of the lesions in acute disease, later stiffening and sclerosis, hypo- or hyperpigmentation

Pictures

Morphea, sacral region:
Morphea, CLINIC (820)

Localized scleroderma (morphea), trunk, with inflammed borders:
Morphea, CLINIC (1075)

Morphea, lilac ring:
Morphea, CLINIC (1076)

Morphea:
Morphea, CLINIC (1077)

Localized scleroderma affecting the tongue and perioral skin:
Scleroderma, CLINIC (1081)

Linear scleroderma:
Scleroderma, CLINIC (1085)

Morphea and lichen sclerosus:
Morphea (and lichen sclerosus), CLINIC (5767)
Lichen sclerosus (and morphea), CLINIC (5769)
Lichen sclerosus (and morphea), CLINIC (5770)
Lichen sclerosus (and morphea), CLINIC (5771)

Further examples of morphea:

Histology:

Histological picture of the diffuse and localized forms is the same: perivascular lymphocytic infiltrate, dermal edema, later fibrosis of the dermal connective tissue and epidermal atrophy; dermal connective tissue is sclerotic, sweat glands are entrapped by dermal collagen.

Superficial morphea is limited to the upper parts of the corium.

6.8.5  Lichen sclerosus et atrophicus

Clinical signs:

  • location: genital, trunk
  • pale areas, sometimes pruritic (especially if located on the vulva: craurosis vulvae)
  • increased incidence of spindle cell carcinoma
  • atrophic, small, depigmented macules
  • merging into larger lesions
  • if located at the genital scarring may follow (with functional disorders)
  • follicular keratosis, rarely erosions, hemorrhagic blisters

Histology:

Edema of the upper dermis, subepidermal clefts, vacuolar degeneration of basal layer, hyperkeratosis (especially follicular), epidermal atrophy

6.8.6  Pachydermoperiostosis

Clinical signs:

  • rare syndrome: clubbing of fingers, periostal thickening
  • thickening of the skin of the face (cheeks, scalp)
  • cutis verticis gyrata

Histology:

Slight acanthosis, thick collagen bundles, slightly increased amount of mucin, increased number of fibroblasts. Slight perivascular and periadnexal lymphocytic infiltrate.

Pictures

Pachydermoperiostosis:
Pachydermoperiostosis, HE 20x (4917)  [zoomify]

6.8.7  Eosinophilic fasciitis

Clinical signs:

  • pain, swelling, induration
  • inflammation of the deep fascia
  • often associated with hypereosinophilia
  • location: often extremities

Histology:

Thickened subcutaneous septa and deep fascia, with variable inflammatory infiltrate with plasmocytes and often (not always) eosinophils.

6.8.8  Fibromatosis

Clinical signs:

  • slowly growing, chronic process
  • some forms are locally aggressive
  • locations:
    • palm: Dupuytren contracture
    • plantar fibromatosis (Lederhose)
    • penile fibromatosis (Peyronie)

Histology:

Nodules and fascicles of fibroblasts, slight mitotic activity.

Pictures

Plantar fibromatosis of Lederhose:
Plantar fibromatosis of Lederhose, HE 40x (6140)

6.8.9  Desmoid type fibromatosis, desmoid

Clinical signs:

  • rare tumorous proliferation
  • age: from puberty to 40 years
  • location: abdominal wall is the most common location, then shoulder, chestwal, thigh, head, neck
  • deep seated, poorly circumscribed, firm mass
  • local recurrences after incomplete resection are common

Histology:

Elongated spindle cells in bundles, infiltrative growth. Vimentin and smooth muscle actin positive.

Pictures

Desmoid:
Desmoid, HE 20x (4453)  [zoomify]

6.8.10  Striae distensae

Clinical signs:

  • wrinkled, atrophic skin
  • location: abdomen, thighs
  • early striae distensae are pink, later white
  • common association: pregnancy, obesity

Pictures

Striae distensae:
Striae distensae, CLINIC (1986)

6.8.11  Skin graft

Histology:

Fibrosis, degenerative changes in the grafted skin.

Pictures

Skin graft, face (after incomplete excision of a squamous cell carcinoma:
Transplanted skin, margin of the skin graft, HE 20x (3392)

6.8.12  Perforating collagenosis

Clinical signs:

  • rare skin disease
  • transepidermal elimination of collagen
  • types:
    • inherited
    • acquired (patients with diabetes or chronic renal failure on dialysis and some other diseases)
  • intense pruritus
  • papules, healing with scarring
  • location: extensor parts of the limbs

Pictures

Perforating collagenosis:
Collagenosis perforans, HE 20x (4744)  [zoomify]

Another case, HIV positive patient:
Collagenosis perforans, HE 20x (4745)


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