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Diseases of the Epidermis

5  Non-tumorous skin diseases

5.1  Diseases of the Epidermis

5.1.1  Ichthyosis

Introduction:

Heterogenous group of diseases characterized by excessive thickening of the stratum corneum, producing fish-like scales, hence ichthyosis.

Pathogenesis:

increased cohesiveness of cells of the stratum corneum, abnormal keratinization and increased proliferation

Clinical signs:

Begins usually in early childhood, white scales cover trunk, extensor surfaces and face.

Ichthyosis vulgaris is the most common type; autosomal dominant mode of inheritance

Pictures

Examples of ichthyoses are at:

Histology:

Increased thickness of the corneal layer, changes in granular layer.

5.1.1.2  Ichthyosis lamellaris

Introduction:

(Recessive congenital ichthyosiform erythroderma)

Histology:

Thickened stratum corneum, thickened granular layer, no epidermolysis. (lamellar or x-linked ichthyosis)

5.1.1.3  X-linked ichthyosis

Clinical signs:

  • x-recessive transmission
  • affected are esp. males
  • the disease developes during the childhood

Histology:

Hyperkeratosis, often brightly eosinophilic, parakeratosis. Granular layer is preserved and thickened. Slight superficial lymphocytic perivascular infiltrate.

5.1.1.4  Epidermolytic hyperkeratosis

Etiology:

  • autosomal dominantly inherited disease

Clinical signs:

  • rare disease
  • affects the whole body
  • changes present at birth
  • erythema, widespread scaling
  • vesicles and bullae formed during the early course of the disease
  • the skin is dry

Pictures

Erythrodermia ichthyosiformis congenita bullosa, flexures affected:
Bullous congenital ichtyosiform erythroderma, CLINIC (764)

Erythrodermia ichthyosiformis congenita bullosa:
Bullous congenital ichtyosiform erythroderma, CLINIC (765)

Histology:

Hyperkeratosis, degeneration and desintegration of the epidermis (granular degeneration, epidermolytic hyperkeratosis), perinuclear clearing, keratohyaline granules, intraepidermal vesicles, increased mitotic activity.

5.1.1.5  Ichthyosis acquisita

Clinical signs:

  • appears as a part of the paraneoplastic syndrome, in metabolic, infectious diseases and in some vitamin deficiencies
  • dry skin
  • peeling of the skin

Pictures

Ichthyosis acquisita, palms:
Ichthyosis acquisita, CLINIC (766)

5.1.1.6  Keratosis palmoplantaris, palmoplantar keratoderma

Introduction:

Group of disorders characterized thickening of the stratum corneum in various locations, mainly on palms and soles. Some forms affect other organs as well.

The classification is based on the clinical picture, distribution of changes, heredity and genetic defects.

Pictures

Keratosis palmoplantaris paraneoplastica:
Keratosis palmoplantaris paraneoplastica, CLINIC (6361)

Histology:

Variable histological picture: hyperkeratosis, in some diseases epidermolysis or other irregularities of cornification.

5.1.2  Ectodermal dysplasia

Clinical signs:

Large group of diseases, affecting the epidermis and skin adnexa. Various classifications (anhidrotic, hidrotic and other classifications).

Hidrotic ectodermal dysplasia:

  • the ability to perspire is preserved
  • palmar and plantar hyperkeratoses
  • nail disorders

5.1.3  Psoriasis

Introduction:

Chronic disease with multiple recurrences, affecting 1 – 2% of Western population. Severity variable (from several patches to severe exfoliation).

Etiology:

Unknown, multifactorial, genetic, autoimmune. Trigger factors: stress, infections, drugs and other.

Pathogenesis:

Abnormal cellular proliferation of keratinocytes; proliferation is not limited to basal layer; mitotic rate is many times higher than in normal skin.

T-cells mediated inflammation.

Clinical signs:

  • acute (guttate) psoriasis: generalized symmetric eruption
  • chronic psoriasis: the elbows, knees, scalp, sacral region, palmoplantar psoriasis
  • psoriasis inversa: mostly intertriginous localization, psoriasis pustulosa: the palms, soles or generalized eruption
  • psoriatic papule with silvery hyperkeratosis
  • pustules in psoriasis pustulosa
  • papules coalesce in round marginated lesions sometimes resembling geographical maps
  • nail changes (pits, oil spots, subungual hyperkeratosis); more on nail changes in psoriasis see the corresponding chapter
  • changes of the genital and oral mucosa, heavy scaling in the scalp
  • Köbner's phenomenon in acute phase
  • healing with temporary depigmentation: leukoderma
  • psoriatic arthropathy, hepatopathy
  • onset at any age, peak in adolescence
  • Auspitz's sign: pinsize hemorrhage from the elongated capillaries after the removal of the scales (thinned epidermis)

Pictures

Psoriasis vulgaris:
Psoriasis, CLINIC (886)

Psoriasis vulgaris chronica:
Psoriasis, CLINIC (887)

Psoriasis vulgaris chronica exacerbans:
Psoriasis, CLINIC (888)

Psoriasis vulgaris chronica:
Psoriasis, CLINIC (889)

Psoriasis vulgaris chronica:
Psoriasis, CLINIC (890)

Psoriasis vulgaris:
Psoriasis, CLINIC (893)

Psoriasis vulgaris:
Psoriasis, CLINIC (1111)

Psoriasis chronica:
Psoriasis, CLINIC (1112)

Psoriasis chronica:
Psoriasis, CLINIC (1113)

Psoriasis vulgaris:
Psoriasis vulgaris, CLINIC (5846)
Psoriasis vulgaris, CLINIC (5847)
Psoriasis vulgaris, CLINIC (5848)
Psoriasis vulgaris, CLINIC (5849)

Psoriasis, detail:
Psoriasis, CLINIC (1114)

Psoriasis vulgaris:
Psoriasis vulgaris, CLINIC (3277)
Psoriasis vulgaris, CLINIC (3278)
Psoriasis vulgaris, CLINIC (3279)

Psoriasis guttata:
Psoriasis, CLINIC (1117)

Psoriasis guttata:
Psoriasis, CLINIC (1118)

Psoriasis guttata:
Psoriasis vulgaris guttata, CLINIC (3275)
Psoriasis vulgaris guttata, CLINIC (3276)

Psoriasis inversa:
Psoriasis, CLINIC (1119)

Psoriasis, Köbner's sign:
Psoriasis, CLINIC (1120)

Psoriasis of the nails (onycholysis, pits):
Psoriasis, CLINIC (1121)

Psoriasis chronica:
Psoriasis, CLINIC (1122)

Palmar psoriasis:
Psoriasis, CLINIC (1115)

Psoriasis palmaris, palms:
Psoriasis, CLINIC (894)

Plantar psoriasis, plantar:
Psoriasis, CLINIC (892)

Psoriasis geographica, psoriatic arthropathy:
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2808)
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2809)
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2810)
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2811)
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2812)
Psoriasis vulgaris, arthropatia psoriatica, psoriasis geographica, CLINIC (2813)

Psoriasis, scalp:
Psoriasis vulgaris capilitii, CLINIC (3274)

Psoriasis inveterata:
Psoriasis vulgaris inveterata, CLINIC (3281)
Psoriasis vulgaris inveterata, CLINIC (3282)
Psoriasis vulgaris inveterata, CLINIC (3283)
Psoriasis vulgaris inveterata, CLINIC (3284)
Psoriasis vulgaris inveterata, CLINIC (3285)

Psoriasis, penis:
Psoriasis vulgaris, penis, CLINIC (3291)
Psoriasis vulgaris, penis, CLINIC (3292)
Psoriasis vulgaris, Macro (3933)
Psoriasis vulgaris, Macro (3937)
Psoriasis vulgaris, Macro (3931)

Psoriatic arthropathy:
Psoriasis vulgaris, arthropathy, Macro (3707)
Psoriasis vulgaris, arthropathy, Macro (3708)
Psoriasis vulgaris, arthropathy, Macro (3709)
Psoriasis vulgaris, arthropathy, Macro (3710)

Further examples of psoriasis:

Histology:

The picture of the developed lesion of the most common variant is quite typical:

  • thickened epidermis with regular, club shape acanthosis
  • dermal papillae richly vascularized, close to the surface
  • hyperkeratosis, parakeratosis
  • decreased or disappeared stratum granulosum
  • ectatic, proliferating and tortuous capillaries of dermal papillae
  • neutrophils trapped in the stratum corneum (Munro microoabscesses)
5.1.3.1  Psoriatic erytroderma

Clinical signs:

  • variant of psoriasis, affecting the whole body in the form of erytroderma

Pictures

Psoriatic erytroderma:
Psoriasis, CLINIC (1116)

Histology:

Generally all signs of the psoriasis are present, corneal layer, however, is usually not high (desquamation).

5.1.3.2  Palmoplantar psoriasis and palmoplantar pustulosis

Introduction:

Psoriasis affecting palms and soles.

Histology:

Signs of the psoriasis are present here as well, but in chronic course the appearance changes: neutrophils are not present constantly, granular layer regenerates etc.; in some of these cases the differentiation between chronic palmoplantar psoriasis and chronic eczematous dermatitis is not possible.

5.1.3.3  Generalized pustular psoriasis of Zumbusch

Introduction:

Large areas of the skin affected by pustular psoriasis.

Histology:

Widespread formation of pustules with many neutrophils.

5.1.3.4  Lingua geographica

Clinical signs:

  • reddish patches with pale borders, change size and shape
  • location: tongue
  • considered to be a variant of psoriasis

Histology:

Acanthosis, prominent exocytosis with many neutrophils within the mucosal epithelium (pustular spongiosis).

5.1.3.5  Psoriatiform keratosis

Introduction:

Local lesion. Histology corresponds to psoriasis.

5.1.4  Infantile acropustulosis

Clinical signs:

  • rare disease of unknown ethiology
  • affects infants of 0 – 3 years of age (most often 2 – 12 months)
  • recurrent crops of vesicles and pustules
  • location: palms, the soles, and the lateral surfaces
  • intense pruritus
  • affected children are otherwise healthy
  • disease is self-limited, heals spontaneously, sometimes with residual pigmentation

Histology:

Intraepidermal pustules with neutrophils and some eosinophils, changing into subcorneal pustule. Slight superficial mixed dermal infiltrate.

5.1.5  Darier's disease

Introduction:

Disease characterized by keratinisation disorder.

Etiology, pathogenesis:

  • monocellular keratinisation
  • acantholysis

Clinical signs:

  • rare chronic familial disease
  • symetric eruption on the trunk in seborrhoeic pattern, on the face, neck, nails, palms, scalp, mouth
  • multiple closely packed warty papules
  • grey-brown, brown, brown-red colour
  • erosions, oozing, foul smell

Warty dyskeratom is regarded as an isolated form of Darier's disease.

Pictures

Darier's disease, lesions of the breast and temples:
Darier's disease, CLINIC (696)

Darier's disease:
Darier's disease, Macro (3853)
Darier's disease, Macro (3854)
Darier's disease, Macro (3855)
Darier's disease, Macro (3856)

Darier's disease, nail lesions:
Darier's disease, CLINIC (698)

Darier's disease, typical lesions of the intertriginous areas under the breasts:
Darier's disease, CLINIC (699)

Darier's disease, intertriginous areas under the breasts:
Darier's disease, CLINIC (700)

Darier's disease, widespread dissemination on the trunk with irritation:
Darier's disease, CLINIC (701)

Keratosis follicularis, back:
Keratosis follicularis, CLINIC (1063)

Darier's disease, detail of eroded papule:
Darier's disease, CLINIC (702)

Darier's disease, lesions of the back with erosions:
Darier's disease, CLINIC (703)

Darier's disease:
Darier's disease, CLINIC (5594)
Darier's disease, CLINIC (5595)
Darier's disease, CLINIC (5596)

Histology:

Hyperkeratosis, dyskeratotic cells (corps ronds and grains), suprabasal acantholytic clefts.

5.1.6  Porokeratosis

Introduction:

Group of keratinization disorders.

Etiology, pathogenesis:

  • autosomal dominant mode of transmision
  • several types

Clinical signs:

  • onset at any age, more often in men, chronic course
  • the extremities, trunk, face
  • slightly atrophic lesion with hyperkeratotic border
  • patches with irregular borders, several cm in diameter (Mibelli type)
  • disseminated small lesions, sometimes linear
  • increased risk of the squamous cell carcinoma

Histology:

Typical is cornoid lamella, narrow ridge of hyperkeratosis with parakeratosis. In typical cases we can see two cornoid lamellae leaned one against another. The epidermis in between is often thinned, sometimes dyskeratotic.

Pictures

Porokeratosis:
Porokeratosis, HE 40x (5498)

(Porokeratosis linearis):
Porokeratosis linearis, HE 10x (65)
Porokeratosis linearis, cornoid lamella, HE 40x (66)

Porokeratosis:
Porokeratosis, HE 2.5x (1610)
Porokeratosis, HE 10x (1609)
Porokeratosis, HE 10x (1608)

Actinic disseminated porokeratosis:
Porokeratosis, actinic disseminated, HE 20x (2027)

Actinic disseminated porokeratosis:
Porokeratosis, actinic disseminated, HE 2.5x (1612)
Porokeratosis, actinic disseminated, HE 20x (1611)

Actinic disseminated porokeratosis, another case:
Porokeratosis, actinic disseminated, HE 10x (1616)

Porokeratosis:
Porokeratosis, HE 10x (1821)
Porokeratosis linearis, HE 20x (1615)

Porokeratosis:
Porokeratosis, HE 20x (1613)

Another case, thick cornified layer:
Porokeratosis, HE 40x (4093)

Porokeratosis:
Porokeratosis, HE 60x (12120)
Porokeratosis, HE 60x (12117)
Porokeratosis, HE 60x (12118)
Porokeratosis, HE 60x (12119)

Porokeratosis:
Porokeratosis, HE 60x (10794)

Porokeratosis:
Porokeratosis, HE 60x (10795)

Porokeratosis:
Porokeratosis, HE 60x (13907)

Porokeratosis Mibelli:
Porokeratosis Mibelli, HE 20x (5400)

Porokeratosis Mibelli:
Porokeratosis Mibelli, HE 20x (5409)

Porokeratotic eccrine ostial nevus, congenital lesion, where ostia of eccrine glands are bound to cornoid lamellae:
Porokeratotic eccrine ostial nevus, HE 60x (6306)
Porokeratotic eccrine ostial nevus, HE 60x (6315)

Porokeratosis changing into squamous cell carcinoma:
Porokeratosis, squamous cell carcinoma, HE 40x (10058)
Porokeratosis, squamous cell carcinoma, HE 40x (10059)
Porokeratosis, squamous cell carcinoma, HE 40x (10060)
Porokeratosis, squamous cell carcinoma, HE 40x (10061)

Porokeratoma or porokeratotic ostial nevus (isolated lesion):
Porokeratoma or porokeratotic ostial nevus, HE 60x (13751)

Follicular porokeratosis, lamellae at ostia of hair follicles:
Porokeratosis follicularis, HE 60x (13908)
Porokeratosis follicularis, HE 60x (13909)

5.1.7  Acrokeratoelastoidosis

Clinical signs:

  • age: usually children
  • small papules, numerous
  • location: hands, feet

Histology:

Localized hyperkeratosis, sometimed diffuse, mild acanthosis, broad stratum granulosum. No inflammation. Elastic fibres are usually fragmented and ofted decreased.

5.1.8  Pityriasis rubra pilaris

Clinical signs:

  • rare, onset at any age, chronic course
  • the entire body, islands of uninvolved skin
  • follicular keratotic papules
  • erythema, scaling
  • hyperkeratosis especially on the palms and soles
  • juvenile, adult, limited forms
  • tendency to erythroderma

Histology:

Acanthosis (broad, thicker than in psoriasis), hyperkeratosis (especially palmoplantar), focal parakeratosis (often perifollicular and within the openings of the sweat glands), preserved granular layer, thickened suprapapillary plates, follicular plugging.

Histological picture is quite variable.

5.1.9  Glucagonoma syndrome

Clinical signs:

  • affects patients with glucagonoma
  • skin lesions: necrolytic migratory erythema
  • other lesions (glossitis, stomatitis, thrombosis...)
  • erythema, epidermal necroses
  • heals usually within two weeks

Histology:

Pale upper part of the epidermis, vacuoles within keratinocytes, necrosis. Sometimes subepidermal vesicles and/or subcorneal pustules are present. Slight dermal lymphocytic infiltrate.

The histological picture is similar to pellagra and acrodermatitis enteropathica.

5.1.10  Acrodermatitis enteropathica

Etiology:

Defect of zinc metabolism.

Clinical signs:

  • inheritance: recessive
  • age: children
  • location: acral, periorificial
  • vesicles, bullae, secondary infections
  • nails, hair shafts and other changes
  • may improve after zinc therapy

Histology:

Parakeratosis over normal stratum corneum, absent granular layer, acanthosis, spongiosis, pallor of the cells of upper epidermal layers, balloning of keratinocytes (may form vesicles), epidermal necrosis.


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