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
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.
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| 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.
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| Facial nodules of Lepromatous leprosy |
