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.

Tutorial 28

Topics discussed in this Tutorial

Neuromas

DD of Angiokeratomas

Lipoatrophy DD

Scleroderma of the Hands

Alopecia Mucinosa

Perianal fistulas

Abscesses

Animal contact lesions

DD of an Epidermal nevus

Streptococcal infections

Keratoacanthomas

DD of upper respiratory Obstructions

Cysts with clear fluid

Sarcoidosis of the face

 Neuromas

There is the schwanoma, the neurofibroma which can be both syndromal and non-syndromal, neurofibrosarcoma and cutaneous meningioma. Another name for a swannoma is a neurilemmoma.   

The histiocytoses are described as histiocytosis X types and the non X. The non X include benign cephalic histiocytosis, juvenile xanthogranuloma, self limiting benign histiocytosis, generalised disseminated histiocytosis, xanthoma disseminatum and multicentric reticulo histiocytosis.

The differential diagnosis of angiokeratomas includes angiokeratoma circumscriptum, Fabry’s disease, Mibelli’s disease that is seen in females with acrocyanosis of the feet and hands and angiokeratoma Fordyce

The image below is of benign cephalic histiocytosis. courtesy of Dr Nameer Al Sudany

Lipoatrophy Differential Diagnosis

There is a localised panatrophy, localised fat atrophy, symmetrical partial atrophy and syndromal atrophy.

The image below is of lipodystrophy See source

The differential diagnosis of scleroderma of the legs is stasis, Lipodermatosclerosis, acrodermatitis chronica atrophicans, Eosinophilic fascitis, Scleromyxedema, Nephrogenic systemic fibrosis , Werner’s disease, scurvy, acrogeria and localised graft versus host disease.

Scleroderma of the Hands

The differential diagnosis is scleroatrophic syndrome of Huriez. There is no Raynaud’s and it is a familial condition. Lichen myxedematosus, juvenile diabetes, amyloid and drugs such as Bleomycin, Lavamizole and vinyl chloride disease. There is also acrogeria, shoulder hand syndrome and phenyl ketonuria.  

 Scleroderma of the neck is scleredema, PCT and the carcinoid syndrome.

Alopecia Mucinosa

This can be the localised plaque type, generalised plaque type or mycosis fungoides plaques.

The image below is from the article linked above on the Scleroatrophic syndrome of Huriez See these other examples in GlobalSkinAtlas

Perianal Fistulas

Consider Crohn’s disease and also hidradenitis suppurativa. Fistulas in the neck consider scrofuloderma and branchial fistulas. Fistulas on the face consider nasal, lip, ear and dental.

Perianal Crohn's disease

The image below is of scrofuloderma courtesy of Dr Nameer Al Sudany

Lymphadenopathy

Rapid onset of lymphadenopathy occurs in infectious mononucleosis, syphilis, Hodgkin’s disease and leukaemia.

Abscesses

These are divided into Animal contact such as glanders and necrobacillosis or Travellers such as tularaemia, plague or melioidosis and Surgical or immunosuppressed such as gangrenous cellulitis, protothecosis and Jobs syndrome and then the Rest including bacterial folliculitis or furunculosis, carbuncles, salmonella, mycobacterium fortuitum and botryomycosis.

Animal Contact Lesions

These include conditions such as orf, cat scratch disease, plaque, erysipelothrix, pasturella multocida, listeriosis, anthrax, tularaemia, necrobacillosis and glanders.

The Differential Diagnosis of an Epidermal Nevus   

This includes ILVEN, Conradi’s disease, epidermal hyperkeratosis, epidermodysplasia verruciformis, ichthyosiform erythroderma, incontinentia pigmenti and also the epidermal nevus syndrome.

The image below is an ILVEN

Streptococcal Infections

Watch ecthyma and also streptococcus viridans causing sub-acute bacterial endocarditis. There is also strep millieri associated with hidradenitis suppurativa.

Compare acnitis versus acne necrotica versus a tuberculid.
In Acnitis the active lesions are on the forehead, it scars and there is pigmentation. In Acne necrotica it is umbilicated, there is itch and it goes through many stages quickly but a tuberculid stays a while and is usually seen on the elbows.

The image below is of acne necrotica

Milia

Question

What are the types of milia?

Answer

There is the congenital type as seen in epidermolysis bullosa and Marie una hypotrichosis. It is familial when it is an autosomal dominant. There are milia after trauma particularly dermabrasion, there are milia after bullae heal particularly epidermolysis bullosa dystrophica and porphyria cutanea tarda and there are milia associated with congenital ectodermal defects.

Milia in EBA after blisters heal

Types of Keratoacanthomas   

There is the solitary type but there are the atypical forms for example keratoacanthoma centrifugum or coral reef keratoacanthomas or giant keratoacanthomas. There is the multiple Ferguson Smith self healing epithelioma and there are the eruptive keratoacanthomas which are generally very small sparing the palms and soles and associated with severe pruritus. Multiple small eruptive KAs have also been reported in red tattoos.

Keratoacanthoma centrifugum Image of Dr John Rowlands

Differential Diagnosis of Obstruction of the Upper Respiratory Tract

Rhinoscleroma presents as an atrophic rhinitis followed by an infiltrative stage and later the larynx can be involved and patients can die from respiratory asphyxia. There is Wegener’s granulomatosis, there is lethal midline granuloma, there is mucocutaneous leishmaniasis, South American blastomycosis which generally affects the lung with dissemination but there is a mucocutaneous type with perioral abscesses and lymphadenopathy. North American blastomycosis has a granular appearance and there is a sporotrichoid type, chromomycosis type and a bromoderma type. It often has central clearing with white peripheral scars.

Rhinosporidiosis

This gives rise to cauliflower like masses in the nose or the mouth and should be differentiated from tertiary syphilis, nasal TB, lymphoma or neoplasms, yaws and leprosy.

The image below is of North American Blastomycosis

Cysts that Present with Clear Fluid

They are either due to impetigo or hidrocystomas. Fox Fordyce disease can affect the axillae but often there is itch only at first and it may be seen around the nipples provoked by emotion and has a prolonged course.

Lymphocytoma Cutis

It is usually circumscribed, it can be solitary but may be grouped. Rarely it has a hyperkeratotic surface like lupus and it may also cause a temporary alopecia in males. Jessners lymphocytic infiltrate in contrast is usually pink, red to brown in colour, can be annular and it is not circumscribed

View the image below at source

Sarcoidosis affecting the face

The types that you see are the plaque type which is rare, the angiolupoid type, lupus pernio particularly in the nose or the ears, a papular type and an annular on the forehead and also a scar sarcoid.

View the image below at source