Tutorial 16

Topics covered in this Tutorial

Apthous Ulcers

White lesions in the mouth

Types of Oral Candidiasis

Gingivitis

Skin lesions of Behcet's

Annular Erythemas

Larva migrans

Leprosy reactions

Flushing reactions

Skin lesions of Rheumatoid arthritis

This tutorial mainly covers oral lesions.

Aphthous ulcers do not affect the gingiva, herpes simplex and Vincent’s angina do. The interpapillary area of the gums is affected by Vincent’s angina but not herpes. 

A spreading erosion in the mouth is pemphigus vulgaris. 

Bullous pemphigoid in the mouth is rare. Note that it seldom involves the tongue. If you have intact blisters in the mouth then in bullous pemphigoid there are few of them and it is mainly in the elderly. 

In erythema multiforme it is a younger age group and there are more of them.

 Inherited leukoplakias may all involve the tongue except a white sponge nevus.

 If you have funny ulcers in the mouth the cause is either traumatic or artifactual, especially if the ulcers have a sharp edge. 

In the mouth pemphigus rarely has any inflammatory change around it and this should be compared with bullous pemphigoid which has.  Image pemphigus Oral

What is the differential diagnosis of aphthous ulcers?

Answer

Herpes simplex both primary and secondary, Sutton’s ulcers but they scar unlike aphthous ulcers, which do not, hand foot and mouth disease, which tends to give linear vesicles, herpangina which involves the soft palate and acute pharyngitis. 

Image of Apthous ulcers herpetiform pattern

What is the differential diagnosis of white lesions in the mouth?

Answer

Congenital causes include dyskeratosis congenita, pachyonychia congenita, white sponge nevus, familial intraepithelial dyskeratosis and Darier’s disease.

Squamous disorders are psoriasis, lichen planus and lupus erythematosus or candidiasis, syphilis and oral hairy leukoplakia. Image of severe candida   Image of oral syphilis

Neoplastic conditions are squamous cell skin cancer and traumatic conditions are cheek biting. Submucosal fibrosis is also due to trauma. It is seen in Indians on the hard palate and they also show some mottled pigmentation. Image of Leukoplakia SCC floor of mouth

The image below is oral hairy leukoplakia

What types of oral candidiasis do you know?

Answer

Acute atrophic, acute pseudomembranous, which is the ordinary type we see, chronic atrophic sometimes known as the edentulous sore mouth, median rhomboid glossitis, chronic hypertrophic and chronic mucocutaneous candidiasis. The types of the latter include familial, endocrine with hypoparathyroidism, thymoma and the immunosuppressed and acquired immunodeficiency types.

Note that a white sponge nevus is in the floor of the mouth or the buccal mucosa. It does not involve the tongue. Also if you have a white lesion in the mouth always look at the anus and perianal skin. In dyskeratosis congenita and pachyonychia congenita the oral lesions occur in the second decade. Lupus erythematosus of the mouth involves the buccal mucosa with a leukoplakia like lichen planus. Patients who have secondary syphilis white lesions in the mouth often have an associated pharyngitis. Note that the gingiva and the hard palate are keratinising surfaces. Aphthous ulcers never go on the gingiva and this can be compared with herpetic ulcers which do.
The image below is oral candidiasis

Causes of gingivitis   

Consider herpes simplex, Vincent’s angina, leukaemia, vitamin C deficiency, bacterial infection, cyclic neutropenia and histiocytosis X, lichen planus and mucocutaneous pemphigus.

Question

What are the causes of marked cheilitis of the lips?

Answer

Stevens-Johnson syndrome, lupus erythematosus, pemphigus, herpes simplex and toxic epidermal necrolysis. These will involve both lips generally although lupus and lichen planus can just involve the lower lip and of course actinic cheilitis mainly involves the lower lip. Rarely contact dermatitis.

The image below is granulomatous cheilitis

Discoid lupus erythematosus lips

Question

What are the skin lesions of Behcet’s disease?

Answer

Aphthous ulcers, genital ulcers, pustules at the sites of trauma, pyoderma gangrenosum, thrombophlebitis erythema nodosum and vasculitis. There may also be compliment deficiencies. Image scrotal ulcers Behcet's

 Last thoughts. Note that mucous membrane pemphigoid or cicatricial pemphigoid does not necessarily give scarring in the mouth and that junctional epidermolysis bullosa may give mottled teeth and severe bullae in the mouth.

The image below is multiple apthous ulcers in Behcets syndrome

Some Annular  Erythemas   

Consider the following:

1.        In erythema marginatum the illness may last several weeks but individual lesions are short lived. They may be raised, rarely purpuric and they are urticarial in the centre.
2.         With Still’s disease there is a diffuse macular eruption with sometimes central pallor. Differential diagnosis is often rubella, adeno viruses and infectious mononucleosis but they tend to have just an evanescent rash.
3.        Erythema infectiosum may clear up and then recur again. It can go on for up to six weeks. Note the rash on the buttocks and the outer aspects of the arms is often reticulate or net like. It particularly involves the buttocks, thighs and arms and this reticulate erythema occurs after the slapped cheek presentation.

The image below is Erythema marginatum

Erythema marginatum

Erythema infectiosum

Larva Migrans

Larvae migrans has an advancing edge but it may curl back on itself. Larvae currens moves faster and it is due to Strongyloides stercoralis whereas larvae migrans is the hook worm Ancylostoma Braziliensis.  Image of larva migrans

The image below is Larva migrans 

Leprosy Reactions

When there is some downgrading, i.e. there is less of an immune reaction, you get more lesions and there are more bacilli in the lesions. When there is upgrading you will get a swelling of existing lesions and nerves, i.e. you are developing a more intense immune reaction. Erythema nodosum leprosum is actually an allergic reaction and is a common cause of vasculitis.  Image in GSA

What are the clinical features of leprosy lesions?

Tuberculoid leprosy has hypopigmented lesions with a lack of sweating, lack of hair, thickened nerves and papules just inside the advancing edge. Borderline tuberculoid leprosy has a sharp inside edge and diffuse outer edge. Sensation is down and sweating is down as well. Image  Indeterminate is macular with hypopigmentation in darker skin. The adnexae are usually okay. There are a few bacilli. Indeterminate leprosy is pink in light skin. Image Borderline lepromatous leprosy has a raised ring surrounding a central flat immune area. Lepromatous leprosy has macules, nodules, infiltrates mainly in the ears and the nose with some congestion. There is a negative lepromin test.
Lucio’s phenomenon gives diffuse erythema, often with a lack of the eyebrow edge and it is seen in Mexico. In tuberculoid leprosy the histology shows tuberculoid granulomas around the adnexae with associated Langerhans giant cells.

The image below is a leprosy reaction

Flushing Syndromes       

These include the carcinoid syndrome, phaeochromocytoma, Zollinger Ellison, mastocytosis, hyperthyroidism, perimenopausal and flushing associated with angiokeratoma corporis diffusum.

Skin Lesions in Rheumatoid Arthritis

Remember leg ulcers due to vasculitis or pyoderma gangrenosum. You can get sclerodermoid changes, bullae, nodules and other types of vasculitis. On the fingers you will get gangrene and infarcts

The image below is flushing in Carcinoid syndrome

Other bon mots

Hydro vacciniforme occurs in boys and causes scarring of the face.  Image
The Lawrence Seip syndrome gives acromegalic looking children with lipodystrophy and from memory I think they also have acanthosis nigricans.

If you have an advancing edge on a lesion then always think of fungi, tumour, granuloma annulare, leprosy, other granulomas and erysipelas.
   
Common causes of hard firm fingers are diabetic stiff skin syndrome, scleroderma, scleromyxedema and amyloidosis.

Periocular papules may be due to acnitis or granulomatous rosacea, syringomas, Cowden’s disease or lipoid proteinosis.
   
Leprosy can involve the tip of the tongue but a tuberculous ulcer is almost always on the back of the tongue.

What are the types of lipodystrophy?

There is the localised which can be ringed or due to trauma. The ringed type can be inflammatory and nevoid. Inflammatory is especially likely with the collagen diseases. There is also the Parry Romberg syndrome on the face with hemiatrophy.   Partial lipodystrophy involves the upper half of the body. Image  The legs are usually okay and in total lipodystrophy all the parts of the body can be involved and an example is the Lawrence Seip syndrome.   

The image below is acnitis of the scalp margin.

Tutorial 24

 Topics discussed in this Tutorial

The difference between Necrobiosis and Necrosis

Sclerodermoid changes in the skin

Secondary syphilis

Kawasaki Syndrome

Comparing Dyskeratosis congenital and Pachyonychia congenita

Mucopolysacharidoses

Congenital syphilis

Peg teeth

Ectodermal dysplasias

Neurofibromatosis and Tuberous Sclerosus

Dyskeratosis Congenita/Fanconi’s anaemia/Naegeli’s Syndrome

Poliosis

The Difference between Necrobiosis and Necrosis

Necrobiosis means physiological death and necrosis means pathological death. Necrobiosis lipoidica diabeticorum near the hairline gives no atrophy or scarring. The differential diagnosis of NLD includes granuloma annulare, morphea, panniculitis, xanthomata. Note also that diabetic dermopathy does not scar. They still have hair follicles whereas the hair follicles are lost in necrobiosis lipoidica and there may sometimes be comedones in necrobiosis lipoidica.

The image below is necrobiosis lipoidica

Diabetic dermopathy It is often paler than this.

Sclerodermoid Changes in the Skin   

On the face these can be due to xanthosidero histiocytosis. This gives a greenish tinge and it looks like scleroderma. The carcinoid syndrome can also give an sclerodermoid change as can PCT which gives a violaceous colour like Cushing’s and also a condition called rhomboidallis cutis frontalis. Sclerodermoid features are also seen in late stage graft versus host disease. Porphyria cutanea tarda in the scalp may scar and the differential diagnosis is necrobiosis lipoidica and lupus.

Sclerodermoid PCT chest

The Flushing Syndromes   

Carcinoid and mast cell disease give flushing that mainly affects the limbs    but I still consider it most with severe prolonged facial and upper chest flushing with varying colours. Pheochromocytoma particularly occurs around the menopause with hyperhidrosis. You get over breathing with the carcinoid syndrome.

The Colour of Lymphomas

B-Cell lymphomas are usually reddish whereas T-Cell lymphomas such as mycosis fungoides are often violaceous. Lymphocytoma Cutis can be polymorphic and very rarely will it scale whereas Jessners is never polymorphic and it never scales.

Inherited Ichthyotic Disorders and CNS Features

Sjogren Larsen gives spasticity and Refsum’s disease gives ataxia.

The image below is a B cell lymphoma

Secondary Syphilis

Note such unusual features such as a nocturnal headache. Also the papules come inside the macules and then they gradually become more papular as the immunity changes and that is what causes the morphological change.

Oral Lesions of Syphillis

You get the snail track ulcers, there is a loss of papillae on the tongue and there is a glossitis.

The image below is of secondary syphilis

Oral lesions  of secondary syphilis

Comparing Dyskeratosis Congenita and Pachyonychia Congenita   

In dyskeratosis congenita you get aplastic anaemia and paronychia. You will get hyperhidrosis in both pachyonychia congenita and dyskeratosis congenita. Both also give oral leukoplakias. The nail bed is also involved in pachyonychia congenita with hyperkeratosis  but the matrix is involved with grossly thickened nails like hooves. Dyskeratosis congenita starts at ages 5 to 15 and damage to the matrix gives a sort of sticking up plug like deformity of the nail.

Pachyonychia congenita nails 

Kawasaki Syndrome

You get a glove and stocking type erythema and you also get oedema of the hands and feet with a red tongue. It may also classically give a buttock erythema.

Compare and Contrast Scleromyxedema versus Scleroderma   

In scleromyxedema the skin is thickened but it is moveable on the subcutis whereas in scleroderma the skin is bound down and it is not moveable

The image below is scleromyxedema

The Inherited Mucopolysaccharidoses - Compare Hurler’s Syndrome with Hunter’s Syndrome   

In Hurler’s syndrome it is an autosomal recessive with cloudy corneas. In Hunter’s syndrome it is an x linked with clear corneas and ivory white nodules arranged in rows on the lateral trunk.. The San Filipo also has a clear cornea. All the other mucopolysaccharidoses have cloudy corneas.

The image below is of Hurler's syndrome

Congenital syphilis

It may present :

a.        With disease in which case the antibody tests will be positive. The child may look apelike with yellowish skin, rhinitis, the skin features of secondary syphilis and also a mild paronychia.
b.        Secondly it may present as a latent stage with no obvious features. The standard treponemal tests are positive in about 60% of cases.
c.        Or it may present as the late stage where it presents with an interstitial keratitis, synovitis, bone changes especially affecting the nose or sabre tibia and often 8th nerve deafness with optic atrophy and paroxysmal cold haemoglobinuria.

The other features associated with congenital syphilis presenting with disease are osteochondritis, syphilitic dactylitis, hepatitis splenomegaly and meningitis. The stigmata of congenital syphilis are notched incisors and mulberry molars. Hutchinson’s triad is interstitial keratitis, Hutchinson’s teeth and 8th nerve deafness.   Optic atrophy in a young person should arise suspicions of congenital syphilis.

A good way of looking at the term gumma is a boil that does not hurt. The serology of congenital syphilis, if it is IGM positive then treat, if there is a rising titre then treat. Titres are usually negative by three months if they have been there in the first place because of passive transfer from a treated mother.

The image below is of secondary syphilis

Secondary syphilis palms

Peg Teeth

This can be seen in congenital syphilis, anhidrotic ectodermal dysplasia, incontinentia pigmenti and chondroectodermal dysplasia. Chondroectodermal dysplasia is an achondroplastic dwarf with six fingers. They have peg teeth and nail abnormalities.

Dermnet on the Ectodermal dysplasias

The treponnema pallidum immobilisation test is positive in late primary syphilis. It is always positive and it distinguishes a biological false positive. The treponemal pallidum antibodies are very sensitive and they are seen earlier than the VDRL test.

The image below is of Anhidrotic ectodermal dysplasia and peg teeth

Neurofibromatosis and epiloia or tuberous sclerosis.

The similarities are poliosis, café au late spots, mental deficiency, pedunculated fibromas, collagenous nevus and a high spontaneous mutation rate. The endocrine problems associated with neurofibromatosis include phaeochromocytoma, acromegaly, hyperparathyroidism, gynecomastia and precocious puberty.

Neurofibromatosis

The clinical types you can see include molluscum fibrosum, plexiform neuromas and elephantiasis neuromatosa. Crowe’s sign is axillary freckling. There may be an association with juvenile xanthogranuloma. Oral lesions are seen in 5% to 10% of cases. Kyphosis in 10%. Neurological abnormalities in 40%. Sarcomatous change may occur in 5% to 15% of cases but it is rare before the age of 40.

The image below is of Tuberous Sclerosus

Dyskeratosis Congenita/Fanconi’s anaemia/Naegeli’s Syndrome

These all have reticulate pigmentation in the new born period.

See also Reticulate pigmentation of Kitamura

dyskeratosis congenita

The image below is reticulate pigmentation of Kitamura

The Differential Diagnosis of a Breast Dermatitis

This includes Paget’s disease, eczema, Bowen’s disease and scabies.

Albright’s syndrome and Others

It will show irregular café au late spots and polyostotic fibrous dysplasia.

Albright's syndrome

Henoch-Schoenlein disease may present with urticarial papules around the elbow plus the purpura on the buttocks and lower legs.

Consider syphilis if someone has pale ulcers in the mouth.

Hereditary haemorrhagic telangiectasia can look live caviar tongue. The latter is usually due to a lymphangioma.

The differential diagnosis of scrotal ulcers includes behcet’s, a fixed drug eruption and an artifactual lesion.

If you have papules in an area that otherwise looks like tinea it usually represents fungus in hair follicles.

If a patient appears to have erythematous patches on the face but it is only in the most cold exposed areas such as the ears and the lobes and the tips of the nose then consider leprosy.

Poliosis

This is seen in tuberous sclerosis, piebaldism, vitiligo, Voyt Koyanagi syndrome, Wardenbergs syndrome and neurofibromatosis.

The Phakomatoses   

These are fibrous tumours involving the eye and the skin.

Café Au Late Spots   

These are found in Albrights syndrome, neurofibromatosis, Blooms syndrome, Silver syndrome, Leprechaunism, tuberous sclerosis, Von Hippel-Lindau syndrome, ataxia telangiectasia, Leske’s syndrome, Sipples syndrome, Cowden’s syndrome and local morphoea. Note that the differential diagnosis of café au late spots and poliosis is either neurofibromatosis or tuberous sclerosis. The EEC syndrome is ectrodactyly giving a lobster claw deformity, peg teeth and a cleft palate. AEC syndrome is ankloblepharitis, peg teeth and a cleft palate.

Poliosis

In Adenoma sebaceum angioid streaks do not go into the macular area. They do though in Pseudoxanthoma elasticum.
Naegeli’s syndrome is a reticulate pigmentation often in the neck and axilla. There is no preceding inflammation. There may be keratoderma of the palms and soles. The teeth may have a yellow discolouration of the enamel and they are mentally normal.
Fanconi’s syndrome is pigment like dyskeratosis congenita with some depigmented raindrop like macules. They have anaemia and thrombocytopenia, short hands and tapering fingers.