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Infiltration of the upper and deep corium



5  Non-tumorous skin diseases

5.6  Infiltration of the upper and deep corium

Introduction:

Group of diseases, classified according to the distribution and cellular composition of infiltrate. Many units are mentioned in other categories (lupus erythematosus, vasculitides, tumors).

5.6.1  Erythema annulare centrifugum (erythema figuratum)

Etiology, pathogenesis:

  • unknown

Clinical signs:

  • the trunk, extremities
  • urticarial erythematous annular or polycyclic lesions
  • centrifugal spreading, regression in the center
  • immunological reaction in tumours, infections and autoimmune diseases
  • middle-aged adults
  • healing in weeks up to years

Pictures

Erythema annulare, patient with lung carcinoma
Erythema annulare, CLINIC (1039)

Histology:

Superficial nad deep, dense, sharply demarcated perivascular lymphocytic infiltrates, epidermis normal; in superficial form the infiltrates are limited to the upper dermis only and epidermis may show spongiosis and microvesiculation.

5.6.2  Erythema gyratum repens

Clinical signs:

  • arcuate and polycyclic erythemas
  • usually migrate
  • pruritic
  • trunk, limbs
  • association: often carcinoma (esp. pulmonary)

Histology:

Perivascular lymphocytic infiltrate of variable density, slight spongiosis and parakeratosis. Sometimes slight admixture of eosinophils. Clinical appearance is important to establish the diagnosis.

5.6.3  Lymphocytic infiltrate of Jessner-Kanof

Histology:

Dense lymphocytic perivascular infiltrate, superficial and deep. Dermal mucin is usually increased. Epidermis is normal (unlike in lupus erythematosus).

5.6.4  Urticaria

Introduction:

Usually transient problem characterized by dermal edema. Chronic forms do exist, sometimes together with vasculitis.

Etiology, pathogenesis:

  • hypersentitive reaction to various stimuli: alergic (IgE or complement mediated: food, drugs, insects), non-allergic (physical stimuli, like cold, vibrations, pressure) or idiopathic.

Clinical signs:

  • anywhere on the skin including the lips, mucosal surfaces affected as well
  • color is white, pink or red
  • lesions can be generalized, localized, circinate, annular etc.
  • angioedema: edema of the lips and mucous membranes
  • acute attacs disappear within sevetal hours
  • chronic forms recurr
  • pruritus
  • papules, wheals, edematous plaques, angioedema

Histology:

Edema in upper and middle dermis, often histologically less conspicuous than clinical appearance; perivascular mixed imfiltrate containing neutrophils and/or eosinophiles.

5.6.5  Pruritic urticarial papules and plaques of pregnancy (PUPPP)

Clinical signs:

  • last trimester of gravidity, disappears after delivery
  • more common in first gravidities
  • affects the skin of the abdomen and extremities, often in striae; umbilicus is spared
  • urticarial papules and plaques of pink and red color
  • severe pruritus

Histology:

Lymphocytic infiltrate usually reaching to the middle dermis, containing eosinphils and neutrophils; dermal edema, variable spongiosis, parakeratosis and mild acanthosis.

5.6.6  Well's syndrome

Clinical signs:

  • papules, plaques, sometimes vesicles
  • location: trunk, extremities
  • acute onset, lasts several weeks
  • livid, firm papules gradually heal
  • may recurr

Histology:

Dense dermal perivascular and interstitial infiltrate with many eosinophils, which are partially degranulated. Alteration of collagen of variable extent, often surrounded by epithelioid cells: flame figures. The infiltrate may reach deep into the dermis, subcutis and even deeper.

Pictures

Well's syndrome:
Well's syndrome, HE 10x (1757)

Well's syndrome, HE 40x (1758)

Well's syndrome:
Wells syndrome, HE 40x (6093)

Well's syndrome in a 3 years old child:
Well's syndrome, HE 40x (4069)

Early Well's syndrome:
Well's syndrome, HE 20x (1759)

Another example of early Well's syndrome:
Well's syndrome, HE 40x (5126)
  [zoomify]

Well's syndrome:
Well's syndrome, HE 20x (5068)
  [zoomify]

Well's syndrome:
Well's syndrome, HE 20x (5069)
  [zoomify]

Well's syndrome:
Well's syndrome, HE 20x (5070)
  [zoomify]

Well's syndrome:
Wells syndrome, HE 40x (5127)

Well's syndrome:
Wells syndrome, HE 60x (13273)

5.6.7  Hypereosinophilic syndrome

Introduction:

Persistent unexplainable hypereosinophilia.

Clinical signs:

Variable lesions, usually papules, nodules, urticaria and other.

Histology:

Superficial and deep infiltrate with eosinophils (+lymphocytes, plasma cells, mast cells), sometimes with edema.

5.6.8  Granuloma faciale (eosinophilicum)

Etiology, pathogenesis:

  • unknown, probably immune disorder

Clinical signs:

  • papules, nodules
  • affect face (almost exclusively)
  • brown red color
  • asymptomatic
  • persistent, no therapy

This disease does not have anything common with eosinophilic granuloma (histiocytosis X variant), which can affect skin as well.

Histology:

Dense infiltration reaching deep into the dermis, usually separated from epidermis by the narrow layer of infiltrated corium (grenz zone). This infiltrate is composed of lymphocytes, histiocytes, neutrophils and eosinophils. Blood vessels are affected (leukocytoclastic vasculitis), therefore extravasation of erythrocytes and siderophages are common.

5.6.9  Erythema elevatum diutinum

Clinical signs:

  • scattered papules and patches
  • single or organized into annular forms
  • pink to brown
  • localization: anywhere, especially on the extensor surfaces, near joints
  • sometims painful, sometime pruritic, often asymptomatic
  • no changes in overall status of the patient
  • perist for years

Histology:

Epidermis is slightly acanthotic, sometimes infiltrated with neutrophils. Chronic vasculitis with many neutrophils, leukocytoclasia and dense perivascular infiltrate. Sometimes edema of the surrounding dermis.

In long lasting lesions can form extracellular deposits of cholesterol.

Pictures

Erythema elevatum diutinum:
Erythema elevatum diutinum, HE 20x (4238)

5.6.10  Sweet's syndrome

Etiology, pathogenesis:

  • frequent association with malignancy (AML and others), solid malignancies, colitis ulcerosa and others

Clinical signs:

  • persons in middle age
  • lasts weeks, sometimes even longer
  • affects upper extremities, head, neck
  • papules, plaques, edema

Histology:

Edema of papillary dermis, widespread infiltrates composed of neutrophils; malignant cells sometimes present as well (and lymphocytes, eosinophils sometimes).

Pictures

Sweet's syndrome:
Sweet's syndrome, HE 10x (401)

Sweet's syndrome, HE 20x (402)

Sweet's syndrome, HE 20x (611)

Sweet's syndrome, HE 40x (403)

Sweet's syndrome, HE 40x (404)

Sweet's syndrome, HE 40x (405)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 2.5x (1704)

Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 10x (1702)

Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (1703)

Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (1706)

Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (1705)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (2332)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (4038)

Another case:
Sydrome of Sweet, HE 40x (5948)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (4611)
  [zoomify]
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (4612)
  [zoomify]

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (4613)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (4614)
  [zoomify]

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (12634)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 40x (12945)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 60x (13257)

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (4615)
  [zoomify]

Another case:
Acute febrile neutrophilic dermatosis (syndrome of Sweet), HE 20x (5006)
  [zoomify]

5.6.11  Behcet's disease

Clinical signs:

  • affects especially young adults
  • painful lesions of the oral mucosa (almost constant finding)
  • genital ulcerations
  • ocular lesions (uveitis, hypopyon, iridocyclitis)
  • skin lesions:
    • trombophlebitis
    • erythema nodosum-like lesions
    • papulopustular lesions
    • sterile pustules after minor trauma
    • lesions resembling Sweet's syndrome
  • synovitis, meningoencephalitis

Histology:

Histology is quite variable.

Aphtous lesions show epithelial defect and (sometimes deep) perivascular and diffuse inflammatory infiltrate with neutrophils.

Skin pustules show deep neutrophilic infiltrate.

Erythema nodosum-like lesions show early lymphocytic infiltration within the fat and deep dermis; later many neutrophils are present.

Vasculitis and other changes are being described as well

Pictures

Behcet's disease, skin pustule and oral mucosa ulceration:
Behcet's disease, HE 40x (3611)

Behcet's disease, oral mucosa, HE 40x (3612)

Behcet's disease, perifollicular lesion:
Behcet's disease, HE 20x (5122)
  [zoomify]

Flat ulceration with dense mixed inflammatory infiltrate at the base of epithelial defect:
Behcet's disease, HE 20x (4314)
  [zoomify]

Pathergic reaction:
Pathergic reaction, HE 20x (4574)

Behcet's disease:
Behcet's disease, HE 20x (5083)
  [zoomify]

5.6.12  Pyoderma gangrenosum

Etiology:

  • in about a half of patients associated with other disease (colitis ulcerosa, rheumatoid arthritis, hepatitis)

Clinical signs:

  • affects usually lower extremities
  • inflammatory pustule or bulla, early ulcerating
  • ulcerations wiht undermined, inflammed borders, sometimes vegetations
  • no response to therapy
  • sometimes painful
  • heals with cribriform scar

Histology:

Deep and dense infiltrate with many neutrophils, abscess, blood vessels with fibrin deposits, ulcerations.

Pictures

Pyoderma gangrenosum:
Pyoderma gangrenosum, HE 20x (602)

Pyoderma gangrenosum, HE 40x (603)

Pyoderma gangrenosum in a case of ulcerous colitis (biopsy of the case above), with prominent neutrophilic infiltrate around remnants of a follicle:
Pyoderma gangrenosum, HE 40x (4153)

Pyoderma gangrenosum, patient with Crohn's disease:
Pyoderma gangrenosum, Crohn's disease, HE 40x (5495)

Another case of pyoderma gangrenosum:
Pyoderma gangrenosum, HE 20x (4591)

Another case of pyoderma gangrenosum:
Pyoderma gangrenosum, HE 40x (6299)

Another case of pyoderma gangrenosum:
Pyoderma gangrenosum, HE 40x (6298)

Pyoderma gangrenosum, HE 40x (6296)

Pyoderma gangrenosum, HE 40x (6297)



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