Tutorial 17

Topics covered in this Tutorial

Vasculitis

Bullae

Herpes Gestationis

Hemorrhagic Bullae

Pemphigus and Pemphigoid

Flexural rashes

Drug Induced Necrosis

Impetigo

Bullous disease in Neonate

Orf

Vasculitis

Vasculitis can sometimes be classified on the basis of the morphology of the lesions and those with other systems involved. Those causing plaques include leukocytoclastic vasculitis, granuloma faciale, erythema elevatum diutinum, Sweet’s syndrome and systemic lupus.

Those with nodules are nodular cutaneous vasculitis, erythrocyanosis, erythema induratum, erythema nodosum, erythema nodosum migrans and cutaneous polyarteritis nodosa, (sometimes the latter is referred to as livedo with nodules), plus polyarteritis nodosa.

Sometimes vasculitis presents as ulcers and there can be livedo with ulcers, leukocytoclastic vasculitis, lymphomatoid papulosis, Calciphylaxis,  lymphomatoid granulomatosis, papulo necrotic tuberculid and midline granuloma and Wegener’s.

Sometimes vasculitis can be subdivided into those with other systems involved, for example hypersensitivity angiitis, Henoch-Schoenlein, polyarteritis nodosa and syndromes such as Sjogren’s.

The image below is vasculitis Henoch Schonlein type

Bullae

Note that if there is little in the way of surrounding reactions, this suggests toxic epidermal necrolysis, pompholyx, pemphigus, epidermolysis bullosa, diabetes and insect bites.

If you have an umbilicated bulla like lesion consider orf. Images in GSA
If you have bullae and non red papules consider urticaria pigmentosa, PCT, lichen sclerosis and incontinentia pigmenti.

If you have erythematous papules and plaques with a blister superimposed on it, in an acute situation consider erythema multiforme but in a chronic situation consider pemphigoid, especially in an older person. Also Linear IgA disease and Chronic bullous disease of childhood.  Image in GSA

Bullous morphea occurs very rarely only really when lymphatics are strangulated.

Other Blistering Facts

In toxic epidermal necrolysis, the normal surrounding skin is tender. In dermatitis herpetiformis the skin is itchy and non tender. In pemphigus and pemphigoid the lesions themselves are tender. In pemphigus foliaceous you find that it is symmetrical with a slow build up and the face is scaly. In pemphigoid it is generalised after weeks of being localised and in pemphigus it takes months before it becomes generalised.
In erythema multiforme there are three types, those with papules, those with vesicles and those with blisters. Note it is usually on the face and hands. In bullous impetigo you generally have a positive culture and even when it generalises you have a positive culture but in the generalised staph scalded skin syndrome you often have few bullae and it may not be positive on culture.

The image below is skin sheeting in Toxic epidermal necrolysis.

Herpes Gestationis

In herpes gestationis, better named as pemphigoid gestationis, there are often firm blisters with a ring of pearls distribution similar to linear IgA disease. This can be compared with erythema multiforme in which the blister is in the middle of the red areas. In Snedden Wilkinson syndrome the blisters will sometimes show a hypopyon where there is a fluid level in the blister. Necrolytic migratory erythema will have pustules in the advancing edge but it tends to leave pigmentation and in pustular psoriasis the pustules are often thick walled. They are quite deep.

Other Comments

Pemphigus erythematosus gives pigmentation plus bullae plus scarring.   See Image In erysipelas you can occasionally get bullae at the advancing edge. Watch for bullae associated with necrotising fasciitis. Image

Haemorrhagic Bullae

This can be a feature of pyoderma gangrenosum, disseminated intravascular coagulation and ecthyma gangrenosum due to pseudomonas, vasculitis and pemphigoid and EBA. Occasionally you will get bleeding into PCT blisters.  Image

The image below is pemphigoid gestationis.

Pemphigus and Pemphigoid

Note than when comparing blisters in pemphigus they spread, they are painful and they bleed easily. This is not the case in pemphigoid where they are much more fixed and less likely to form erosions. In pemphigoid there are few lesions on the face. Pemphigus vulgaris and foliaceous often have facial lesions.

Pemphigus foliaceous looking like a severe Seb derm

Light Eruptions

Juvenile Spring eruption is seen in males on the ears and is non-scarring. Hydroa vacciniforme is in males on the face and there is scarring. Hydroa aestivale is in females in non-exposed areas which is unusual for a light eruption and polymorphous light eruption is polymorphic on sun exposed areas and comes out some hours after exposure and is non-scarring.

The image below is the crusting seen in Pemphigus foliaceus

Some Flexural Rashes

In subcorneal pustular dermatosis it occurs in waves with a trailing serpiginous scaly edge. In necrolytic migratory erythema you may have pustules on the edge but there is residual pigmentation. In Hailey-Hailey disease you have fissuring as also in Darier's and pustular psoriasis is very painful. You may get candidiasis with staph like pustules in immunosuppressed patients. Bullous impetigo can give central healing but with activity in the peripheral edges. Remember in pemphigus you get spreading of erosions.

Lesions are much more fixed in pemphigoid. Early pemphigoid often presents as urticarial plaques. Dermatitis herpetiformis often gives larger bullae on the extensor surfaces whereas pemphigoid tends to involve flexural areas.

The image below is of the submammary fissuring seen in Hailey Hailey disease.

Drug Induced Necrosis

Heparin, Warfarin, Dequalinum and cytotoxics.

The Causes of Gangrene

Gangrene of the skin can be due to haematogenous factors, vessel wall damage, vasospasticity, external physical agents, severe infections or abnormal tissue response to infections or other stimuli. Necrosis and levido is typical of calciphylaxis in renal failure patients.

The image below is of Warfarin Necrosis

Lymphadenopathy and Erythema

In this circumstance think of erythema multiforme, Kawasaki’s syndrome, infectious mononucleosis, scarlatina, syphilis and the AIDS conversion syndrome.

The image below is of Kawasaki's disease.

Impetigo

The Tillbury Fox type is a macule that becomes a crusted lesion and it is often a mixed staph and strep infection. Bullous impetigo is essentially a staph infection with an erosion and scale. The blisters rupture easily because they are subcorneal.  It is often not crusted. The scale is around the periphery of the lesion. There are three types. It may be generalised in neonates and those who are immunocompromised. There is a type with erythema all over and there is the TENS type similar to the staph scalded skin syndrome.

The image below is of bullous impetigo merging into Staph scaled Skin

Bullous Disease in the Neonate

The common causes are epidermolysis bullosa, bullous impetigo, incontinentia pigmenti, bullous ichthyosiform erythroderma, neonatal pustular melanosis, transient neonatal erythema, urticaria pigmentosa and scabies.

See Image collection

The image below is of the damage caused by recessive epidermolysis bullosa.

Orf

Note that in orf the lesion itself is haemorrhagic and not the base of the lesion. This is often reflected in the papillary oedema and the extravasated red blood cells in the histopathology.

The image below is of Orf on the forearm of a Vet.

Tutorial 18

Topics covered in this Tutorial

Polymorphous light eruption

Phototoxicity due to Drugs

Nail changes

Pachydermoperiostosis

Bullae in neonates

Perianal fistulae

Ulcers in Rheumatoid arthritis

Ecthyma gangrenosum

Unilateral flushing

Types of Porokeratoses

Polymorphous Light Eruption

Morphology of the lesions can be anything you want but generally it is erythema, urticaria, papules, eczema, plaque like, almost like lupus and prurigo like. It is rare to get full-blown blisters. Generally the areas affected are those that are less often exposed to the sun. Note often the first web space and around the elbows especially the lateral surface. Also you tend to find it on the dorsum of the feet and over the thighs. Images of PMLE in GSA

Phototoxicity is usually due to drugs. All areas reached by the sun are affected and there is usually a very sharp clothing cut off. If there is an external photo allergen there are usually patchy areas where the chemical has touched the skin and it has reacted with light. There may be a mixture of cutaneous features depending on the depth of the UV light through the skin, its intensity and any local repair factors. There may just be erythema or bullae due to phototoxicity if there is superficial damage. Images in GSA

The differential diagnosis of polymorphous light eruption includes actinic prurigo, discoid or subacute lupus, lymphocytoma and acute contact dermatitis.
Polymorphous light eruption usually you see in people under 30, it is more chronic and it is usually in females. Actinic prurigo is usually seen under the age of 10, it improves, it is very itchy and it is again in females. In photosensitive atopic dermatitis you will usually find evidence of atopy elsewhere. In hydroa vacciniforme you have bigger scars than actinic prurigo and they are not itchy and appear in males more than females. In hydroa vacciniforme there can be blisters that are umbilicated and have a red base. They all scar. It is worse in summer and it is quite episodic. You can get associated photo onycholysis. The eyes may be involved but it tends to cease by late adolescence. Compare this with porphyria cutanea tarda where you will get skin fragility and milia but there is no red base to the blisters and it is seen an older age group.
The image below is of juvenile spring Eruption

What nail changes may you get with Tetracyclines?

Photo onycholysis, which can occur with both Minocycline and Doxycycline.    Minocycline can also give a longitudinal brown band as well. Tetracycline Hydrochloride, which is no longer available here in Australia, can cause onycholysis. It can cause a bluish residue in the nail bed and nail. It can cause splinter haemorrhages and pain and it can cause yellow lunulae with fluorescence.

The image below is of onycholysis.

Pachydermoperiostosis

This condition is usually seen in the scalp as a primary type. It is an autosomal dominant condition that comes on at puberty where you can get the face thickening as well as folds in the scalp or you can get thick hands and feet. They can get clubbing. There is some degree of mental retardation.  Images in GSA
A secondary type can occur with an underlying lung carcinoma, it is painful and the skin changes are mild. The other condition to remember is cutis verticis gyrata. This can occur first of all at puberty with hypertrophy and folding of the scalp only, it is an autosomal dominant and a lot of people have a low IQ. There is a marked increase in seborrhoea. Secondly it can be part of pachydermo periostosis. Thirdly it can be part of acromegaly and fourthly secondary to local inflammatory disorders such as infection and cellulitis.  Images in GSA

eMedicine on Pachydermoperiostosis

The image below is of pachydermo periostosis.

Bullae in Neonates

You can get blistering dactylitis which is streptococcal and involves the tips of the fingers. You can have bullous impetigo and a long list starting with epidermolysis bullosa. Note you will get eosinophils in transient neonatal pustular melanosis and also in the Ofugi’s syndrome and also often in insect bite reactions. Images

Dermnet on other causes of dactylitis

The image below is of Blistering Dactylitis

Vascular disease

If you have an ulcer with no sweating and no surrounding hair then it is probably an arterial ulcer. With microvascular disease you will get distal gangrene on several toes and this is commonly seen in diabetes.

Ecthyma Gangrenosum

This is generally seen as part of a pseudomonas infection. It is usually seen in a very toxic immunosuppressed individual. You get erythema first and then bullae and then an eschar formation.

The image below is of Ecthyma gangrenosum in an infant.

What are the causes of perianal fistulae?

Answer

The first condition to think about is underlying Crohn’s disease. However it can also occur with TB, granuloma inguinale, secondary to diverticulitis or malignancy, secondary to ischiorectal abscesses, syphilis, amoebiasis and botryomycosis.

The image below is of the perianal fissures and fistulae of Crohn's disease.

Ulcers in Rheumatoid Arthritis

They can be due to stasis ulcers, vasculitic ulcers, due to prostheses, weight bearing problems and pyoderma gangrenosum. 

If you have an ulcer induced by an X-Ray there will be a lack of vascularity and they also have some evidence of scarring and telangiectasia.

Granuloma Inguinale

This causes ulcers, which are painless. There are no local glands and the ulcers are sharply defined.

What are the causes of the punctate keratodermas?

They include Darier’s disease, lichen planus, porokeratosis, Richner Hanhart syndrome, arsenic and familial.

Punctate keratoderma

Punctate keratoderma

Tyrosinemia Type 2   

It causes punctate, palmar plantar keratodermas particularly in infancy, which may improve. They get severe keratitis and mental retardation. The palmar and plantar lesions are painful and tender to touch. It is helped by a diet low in tyrosine and phenylalanine.   

The image below is of granuloma inguinale

What are the types of granuloma annulare?

First of all you can have localised and generalised. You can have subcutaneous, perforating, micropapular, annular, linear , elastolytic and there is a giant form. It is also seen in Mauriac’s syndrome where you have juvenile diabetes, stunted growth and hepatomegaly.

Perforating granuloma annulare

What types of sarcoidosis do you know?

Clinically you could have papular, micropapular, anular, nodular, scar, lupus pernio, erythema nodosum, angiolupoid and a miscellaneous group including ulcerative, psoriasiform, palmar plantar and inguinal. Nearly all types of sarcoid appear on the face.

What are the features of rosacea?

First of all you can have papules, telangiectasia, pustules, erythema and oedema but you have sparing generally of the nasolabial areas. Sebaceous hyperplasia is seen in some cases giving rinophyma. Involvement of the naso labial areas with scaling is usually due to seborrhoeic dermatitis.

What causes of unilateral flushing do you know?

There is Sphenopalatine syndrome where you get pain, oedema, rhinitis, excess tearing and erythema. There is the Auriculo Temporal syndrome. You get no pain but you will get erythema and hyperhidrosis. There is the Ciliary syndrome where you have pain, erythema, headaches, no rhinitis but you get cluster headaches. You have the Melkersson-Rosenthal syndrome (oral facial granulomatosis) but there is no redness or pain and there is oedema. Compare this with the Sphenopalatine syndrome. In the hypertensive Diencephalic syndrome you get excess sweating and blotching of the skin of the face and the neck with salivation and tachycardia and a sustained hypertension. You have to exclude pheochromocytoma and also consider the Riley Day syndrome in adults. Note you have pain in the skin in Sphenopalatine syndrome but no pain with Melkersson-Rosenthal syndrome.

The image below is of severe rosacea.

Types of Porokeratosis    

First of all there is DSAP, this can be a familial type in women in their 40s, autosomal dominant, especially on the legs or they can be solar induced where there are a lot fewer of them. Generally DSAP begins as a 1mm to 3mm papule around the follicle containing a keratotic plug. The central area becomes atrophic, hypopigmented or occasionally hyperpigmented.

DSAP Disseminated Superficial Actinic Porokeratoses

DSAP Disseminated Superficial Actinic Porokeratoses

Porokeratosis of Mibelli varies in size. Oral lesions can occur. It is often in covered areas. You can get a systematised zosteriform or linear variant in childhood. SCC may develope in lesions in the fourth decade. You can have punctate and palmar planta porokeratoses. Porokeratoses Mantoux are not hereditary. They occur in young adults particularly on the fingers where you get a parakeratotic horny mass, overlying dilated capillaries and small amount of haemorrhage. They shed over several weeks leaving small pits that then disappear.

The image below is of a large porokeratosis of Mibelli.