Tutorial 10

 

Topics discussed in this Tutorial

The development of lesions

Vasculitis as plaques, nodules, ulcers and guttate

Lichen planus erythematosus

The DD of white macules

Parakeratosis variegata

Various ichthyotic skin syndromes

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The development of lesions

        The early phases of various diseases and the pathophysiology involved. Note it usually depends on severity and time.

Vasculitis under the headings of
systemic features
plaques
nodules
ulcers and
guttate
all due to damage to blood vessels but is a matter of degree and time.

Panniculitis drug reaction

Vasculitis

Vasculitis legs

Note the earliest feature may be urticaria.
Acute vasculitis gives rise to vesicles, bullae, haemorrhage, necrosis and ulcers.
Subacute to papules, urticaria, plaques and macules and
Chronic to nodules.
(a)        Systemic, mainly hypersensitivity angiitis, which is usually monomorphic with rapid onset and death
(b)        Polyarteritis nodosa
(c)        Henoch-Schloenlein, and
(d)        Other syndromes, for example rheumatoid arthritis, lupus erythematosus and scleroderma.

Plaques Granuloma faciale, erythema elevatum diutinum, Sweet's Syndrome and systemic lupus erythematosus.

Nodules Nodular vasculitis, erythrocyanosis and cold, erythema induratum, erythema nodosum, erythema nodosum migrans and cutaneous polyarteritis nodosa.

Ulcers Livedo with ulcers, lymphomatoid papulosis, lymphomatoid granulomatosis, papulo necrotic tuberculid, midline granuloma and Wegner's.

Guttate Pityriasis lichenoides and malignant atrophic papulosis.

These pictures can all be seen with leukocytoclastic vasculitis. Lymphocytic vasculitis is usually seen with the more chronic diseases such as pityriasis lichenoides and lymphomatoid papulosis and perniosis.
GlobalSkinAtlas     PubMed   eMedicine

The Development of Lesions

Morphology is really a manifestation of severity and time. For example, lichen planus initially gives rise to dilated vessels with a sluggish blood flow, basal cell damage and epidermal reaction and pigmentation. Hence, erythema, bluish, oedema, bullae. Papules nodules if chronic and heals with pigmentation. Bullae occur early and are part of an acute reaction whereas nodules are part of a chronic reaction and late.

Lichen Planus Erythematosus

This is a more erythematous variant of lichen planus with cherry red papules on the forearms and legs, usually in women. The papules sometimes are confluent, there are no Wickham striae. There may be a suggestion of atrophy in some of them. On pressure the red colour goes but a brownish stain underneath remains. Note well that these lesions are symptomless. They are not itchy like lichen planus. They may persist for an indefinite period.
There is also an overlap syndrome showing features of lichen planus and lupus erythematosus which also goes under this name. See this article.

Compare lichen planus with lichen sclerosus atrophicus.
In the latter there are red papules then white papules due to oedema, then bullae or blisters, then perhaps hemorrhage into weakened skin.

Other causes of white macules include Dagos's Disease, Darier's Disease, lichen sclerosis et atrophicus and albo-papuloid variant of epidermolysis bullosa. In lichen sclerosis there are bullae early with erosions and purpura later. Note its major characteristics of its location and the follicular plugging seen with it. Also there is no post inflammatory hyperpigmentation in lichen sclerosis et atrophicus. Follicles may be accentuated in an area of palor because of their different blood supply, hence they look redder.

Other disorders covered

Poikiloderma vasculare atrophicans. In this condition you can see atrophy, scaling with underlying erythema, telangiectasia and both hypo and hyperpigmentation.

Parakeratosis Variegata Try This Link

You get striped areas of scaly erythema forming a zebra like pattern on the trunk, shoulders and thighs. There may be an increased incidence of lymphoma associated with it.
Dermatology is plagued with different names for essentially the same condition!

SYNDROME        CLINICAL FEATURES        SKIN FINDINGS        DEFECT

Conradi-Hünermann disease (chondrodysplasia punctata)        Chondrodysplasia punctata, limb defects, cataracts, cardiovascular and renal abnormalities, mental retardation

        
Congenital ichthyosiform erythroderma (CIE), whorled hyperpigmentation, palmoplantar keratoderma        Sterol isomerase emopamil–binding protein

CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome        Hemidysplasia and limb defects with sharp midline demarcation        CIE        NAD(P)H steroid dehydrogenase–like protein

Sjögren-Larsson syndrome        Spasticity, mental retardation, retinal degeneration        Lamellar scales        Fatty aldehyde dehydrogenase

Chanarin-Dorfman syndrome (neutral lipid storage disease)        Fatty liver, myopathy, cataracts, deafness, CNS defects        CIE        Impaired long-chain fatty acid oxidation

Netherton’s syndrome        Trichorrhexis invaginata (bamboo hairs), atopy, aminoaciduria        Ichthyosis linearis circumflexa        SPINK5 gene

Trichothiodystrophy (PIBIDS)        Photosensitivity, ichthyosis, brittle hair, intellectual impairment, decreased fertility, short stature        CIE        Xeroderma pigmentosa D or B gene

KID (keratitis-ichthyosis-deafness) syndrome        Keratitis, neurosensory deafness, alopecia        Grainy, spiculated scaling        Connexin 26 gene

Below images of the double scale and skin lesions of Netherton's syndrome

Below image of Sjogren Larsson disease 

Tutorial 19

Topics discussed in this Tutorial

Angiokeratomas

Childhood syndromes

Epidermolysis bullosa

Aquired Ichthyosis

Light eruptions in Children

Bullous impetigo

Causes of Painful Skin

Infantile acne

Sporotrichosis

Pseudomonas infections

 Angiokeratomas     See Dermatoscopy

There are several types of angiokeratomas including Fordyce, Mibelli, angiokeratoma circumscriptum and angiokeratomas associated with Fabry’s. Those of Fordyce occur on the scrotum. The patients may be quite young. They are benign and they do not blanch. Those of Mibelli are usually on the fingers and toes and legs, associated with cold, particularly in the 10 to 15 years old age group and can be treated with diathermy. Angiokeratoma circumscriptum is again in young people mainly on the limbs and trunk. They may enlarge considerably in adolescence. The differential diagnosis is a verrucous haemangioma and generally they require surgery.  Images in GSA

  Angiokeratomas associated with Fabry’s disease occur on the scrotum, umbilicus and mucosal surfaces. They may die from haemorrhage from these lesions or from renal failure. Often oedema of the eyelids is an early feature. They can have corneal opacities. The full syndrome is seen in men. It is rarely that it is seen in females. Inheritance is usually x linked recessive. There is an absence of an enzyme that leads to deposition of fat in vessels of an abnormal lipid. People with Fabry’s will often complain of lancinating pain in their hands or fingers without anything obvious to see or apparent cause.
View Dermnet images

The image below is of Fabry's disease

Other Syndromes

Incontinentia pigmenti again is an x linked recessive. It is seen in females. Males tend to die in utero. There is usually a vesicular phase following Blaschko’s lines but this can occur in utero and may not be obvious. The patient may present with pigment problems. Associations include central nervous system problems, skeletal problems and dental.

The LEOPARD syndrome we have discussed recently. Remember the acronym LEOPARD.

Gardener’s Syndrome

These patients present with sebaceous cysts and lipomas. They also get osteomas, fibromas, particularly desmoid tumours, multiple pilomatricomas  and get quite marked diarrhoea in the late teens.

Cronkite Canada Syndrome

Patients with this disorder present with marked alopecia and pigmented macules of both the dorsal and palmar surfaces of the hands. They also have intestinal polyps.

Osler’s Disease or Hereditary Haemorrhagic Telangiectasia

Telangiectasia do not pulsate. They are particularly found on the upper half of the body. True spider nevi do pulsate because they are arteriolar. Patients with Osler’s disease often have an enlarged liver and high output failure due to pulmonary fistulae. Images in GSA

Peutz-Jeghers Syndrome

Lentigines are present at birth and early childhood particularly on the lips and hands. They can fade later. Images in GSA

Darier’s Disease

Darier’s disease on the hands can give rise to pits and diffuse and punctate keratoderma.

Cowden’s Syndrome

Patients with this disease have tricholemmal lesions on the face, papules on the lips and tongue. Sometimes translucent papules can be removed leaving a pit. They are susceptible to tumours in the thyroid and breast.

Fabry’s disease can present early with oedema of the eyelids and hereditary haemorrhagic telangiectasia can present as nose bleeds. 

Trichoepitheliomas can be inherited as an autosomal dominant disorder particularly in association with cylindromas in the Brooke Spiegler syndrome. They come on at puberty particularly on the face and the upper neck.  Images in GSA

The image below is of Incontinentia Pigmenti

Epidermolysis Bullosa Simplex

There are three types which are all autosomal dominant and are all non scarring. There is a generalised type present at birth where heat makes it worse. There is a localised type or Weber Cockayne which occurs in late childhood and there is the Ogna variety which again is at birth, localised acrally and sometimes haemorrhagic.

 Kindler Syndrome also gives neonatal acral blisters  but also photosensitivity, poikiloderma and cutaneous atrophy

eMedicine on Pediatric EBS

Dermnet images of Epidermolysis bullosa

Acquired ichthyosis can occur in disorders other than malignancy. For example essential fatty acid deficiency, leprosy, sarcoid, SLE and due to certain drugs.

View these other forms of Ichthyosis

The image below is of Ichthyosis vulgaris.

Compare Necrolytic Migratory Erythema, Sneddon Wilkinson Disease and Pustular Psoriasis

In necrolytic migratory erythema there is central healing with a crusted edge and some pigmentation. Sneddon Wilkinson typically has a faint pigmentation with pustules at the edge. In pustular psoriasis there is no pigmentation. It is monomorphic and there are sheets of erythema and pustules.

What is the Differential Diagnosis of Blisters in Children?

The mnemonic for blisters is ICI or infective, contact dermatitis and inflammatory/ immunological. Infective can be due to bacteria, virus or fungi. Inflammatory can be a condition such as psoriasis, drugs particularly causing toxic epidermal necrolysis. Inflammatory also includes the immunological causes such as linear IgA in childhood or childhood immunobullous disease. Some other causes to consider include particularly epidermolysis bullosa simplex, bullous impetigo, toxic epidermal necrolysis, miliaria, pompholyx and linear IgA disease. For blisters with red papules and plaques consider dermatitis herpetiformis, erythema multiforme, bullous pemphigoid, linear IgA disease and pemphigoid gestationis in older females.

Question

What are the major differences between linear IgA disease and dermatitis herpetiformis?

Dermatitis herpetiformis is rare before the age of 10 whereas linear IgA disease commonly occurs in children. Oral lesions are more common in linear IgA disease. Dermatitis herpetiformis patients are more often associated with underlying coeliac disease and gluten hypersensitivity.
View other images of dermatitis herpetiformis

The image below is of milia in EBA in a child.

Compare the Four Types of Light Eruptions in Children

Juvenile spring eruptions in boys particularly on the ears, sometimes blisters and occurs in the spring. Hutchinson’s summer prurigo is seen in atopics and covered areas can be involved. Polymorphous light eruption is usually delayed. It occurs particularly in springtime and clears. Hydro vacciniforme causes scarring and polymorphic lesions.
View other images of PMLE

The image below is of Juvenile Spring Eruption.

Chloroquine Hyperpigmentation

25% get it within four months. Watch it with psoriasis, porphyria and pregnancy.

Cryoglobulins produce cold symptoms but cryo fibrinogens and cryoagglutinins do not produce cold symptoms although they may produce cold disease signs. Benign or mixed forms of cryoglobulinanaemia can present as Raynaud’s, atypical ulcers, livedo, cold urticaria or vasculitis. Mixed causes are particularly seen in connective tissue disease, infections, cancers, liver disease and idiopathic. Skin lesions of cryofibrinogenemia

Bullous Impetigo

You tend to get it up to about the age of nine. It is polymorphic. There is often clear fluid in lesions at first. You may get central healing with peripheral extension of the lesions. Note it is not a crusting disease. It is a bullous disorder and you can even get bullae on the palms and soles. If someone is getting recurrent bullous impetigo, look for scabies, papular urticaria and pediculosis infections. Something is causing them to traumatise the skin and set it off.

The image below is of Bullous Impetigo

Question

What conditions can cause painful skin?

Answer

Staph scalded skin syndrome, acute onset pustular psoriasis, erythropoietic protoporphyria and Fabry’s disease. Also TENS

What are the features of infantile acne?

Answer

Acne neonatorum typically occurs in males and up to about three months of age. From three months to two years of age both sexes are seen with the condition. It is usually monomorphic. Always check the genitals if you are suspicious of adrenogenital syndrome. Note that candida in the face in infants can look like acne. Check for any topical preparations being applied and maternal drugs such as iodides.

What are the features you see in sporotrichosis?

Answer

It really depends on the person’s underlying immunity. In sporotrichosis which is due to a deep fungus you get lymphatic spread of the lesions. It can occur in days and you can feel the thickened lymphatics and perhaps the early nodules. You also get a fixed or endemic type where there is high immunity and it can be just localised to one area for some time. There is a very rare visceral sporotrichosis where it gets into the bloodstream and spreads in people who are profoundly immunosuppressed.

The image below is of a deep fungal infection Sporotrichosis.

Question  What is the characteristic feature in Norwegian scabies?

Answer

Hyperkeratosis in extremely funny areas such as around the fingers or on the ears.

Thick scale of crusted scabies

What are the commonest areas for pseudomonas infections and how do they present?

Answer

You can get bathtub folliculitis presenting as assorted papules and pustules on the trunk. You get ecthyma gangrenosum particularly on the shoulders and arms with full thickness necrosis. You can get oomphalitis around the umbilicus in newborns. You can get pseudomonas affecting the web spaces of the feet and secondary infection in tinea and commonly pseudomonas involves the external ear canals in people who have been in the water a lot developing otitis externa. Pseudomonas under onycholytic nails gives a green pigmentation to the nails from pyocyanin pigment.

The image below is of Pseudomonas folliculitis.

Regarding Lepromatous Leprosy

It may present with oedema of the legs and a stuffy nose. There may be nodules rather than macules and if they are just basically macules, the edges will be diffuse compared with the cleared sharp edges of tuberculoid leprosy. Papules and nodules are often symmetrical and dermal. The skin over them is normal and the lesions are not anaesthetic.

Facial nodules of Lepromatous leprosy