Tutorial 30

 Topics discussed in this Tutorial.

Leg ulcers, Papules face, Eosinophilic syndromes, Central clearing syndromes, Vegetating lesions dd, Oral ulcers, Histiocytosis syndromes, Hypermelanosis circumscribed, Cold lesions, Paraproteinaemias, 

The Differential Diagnosis of Leg Ulcers

Leg ulcers can be arteritic, venous ,undermined due to surrounding disease, tumour, neuropathic with trauma due to anaesthetic skin, infective plus or minus lymphadenopathy. The mnemonic AEST TAIL is sometime used. In an elderly individual always consider diabetes and skin malignancy.

The image below is a neuropathic ulcer in a diabetic.

Monomorphic Papules on the face

If they are infiltrates they are amyloid and lipoid proteinosis. If they are tumours they are trichilemomas, trichoepitheliomas, adenoma sebaceum and syringomas.

Grouped Monomorphic Micro Papules on Face

Consider lichen nitidus, lichen scrofuloderma, lichen myxoedema, follicular mucinosis, follicular eczema, lichenoid sarcoid, keratosis pilaris and micropapular granuloma annulare or granulomatous rosacea.

The image below is of lipoid proteinosis. Infiltrates are seen along the eyelid margin and the patient has associated hoarseness.

Eosinophilic Syndromes

If there are systemic causes then usually the skin is not involved. However cutaneous eosinophilic syndromes include insect bites, scabies, drugs, atopy, hyper eosinophilic syndrome, transient neonatal erythema, Ofugi’s disease, job’s syndrome, Well’s syndrome, incontinentia pigmenti, Kimura’s disease, and eosinophilic panniculitis.

Comment Eosinophilic pustular folliculitis- 3 types Classic eosinophilic pustular folliculitis (Ofuji disease) 1. Typically occurs in Japanese patients, who have chronic, recurrent follicular pustules, with a tendency to form circinate plaques, in a seborrheic distribution 

2. Eosinophilic pustular folliculitis associated with immunosuppression.Most often occurs in patients with human immunodeficiency virus infection, who have severely pruritic papules of the face and upper trunk 

3. Eosinophilic pustular folliculitis of infancy/neonatal period. Follicular pustules of the scalp. Tendency for recurrences and chronicity (except eosinophilic pustular folliculitis of infancy) Characterized by follicular and perifollicular eosinophil infiltration Associated with peripheral blood eosinophilia.

Deckchair sign Papuloerythroderma of Ofugi

The image below is of Kimura's disease dermal nodule rich in eosinophils

Syndromes with Central Clearing    

Consider SIGN DIP. The S for squamous includes pityriasis rubra pilaris, psoriasis and subacute lupus erythematosus. The I for infective includes impetigo and a fungal infection. The G for granulomatous includes syphilis, leprosy, granuloma annulare and sarcoidosis. The N for malignancy includes some regressing melanomas, lymphoma and basal cell carcinoma especially the superficial type. Infiltrates with central clearing include lymphocytic infiltrates such as Jessner’s, neutrophilic such as Sweet’s syndrome and eosinophilic infiltrates such as Well’s syndrome or Ofugi’s disease. Pustular conditions with central clearing include subcorneal pustular dermatosis, the erythemas include erythema annulare centrifugum, erythema marginatum and acrodermatitis chronica atrophicans. The epidermal syndromes with central clearing include Hailey Hailey disease.

The differential diagnosis of vegetating lesions include SIGN DI HILL and the HILL stands for hereditary, intertrigo, lymphedema and lymphoma.
Clinically when I see a big vegetating plaque on an arm or leg I immediately think of Chromoblastomycosis which is shown in the image below.

Oral Ulcers

On the palate it may be pemphigus. On the buccal mucosa it is pemphigoid. On the gingiva it is cicatricial pemphigoid. Kawasaki’s disease, toxic shock syndrome, staph, scalded skin syndrome and toxic epidermal necrolysis all may affect the inside of the mouth as may lichen planus and acute erythema multiforme.

The image below is of Cicatricial pemphigoid involving the hard palate just to be awkward!

Histiocytoses Both X and non-X Age associations

At three months it is self-healing histiocytosis and it may last two to three months. At six months it is benign cephalic histiocytosis and it may last two to three years. In infants it is juvenile xanthogranuloma.  Aged more than 20 it is sinus histiocytosis with massive lymphadenopathy. In adults it is xanthoma disseminatum or generalised eruptive histiocytosis or papular xanthoma and in older people it is multicentric reticulo histiocytosis and progressive nodular histiocytosis.

This list is taken from Fitzpatrick. The lesions of histiocytosis are papular , red/yellow in colour, usually in flexures and the scalp. Vesicles and pustules can be seen especially in the neonatal period. The presence of purpura in lesions is a poor prognostic sign.

Note that early pyoderma grangrenosum lesions can look like orf, anthrax can look like ecthyma grangrenosum, Majocchi’s can look like vasculitis on the legs and watch papulo squamous syphilid which can look a bit like pityriasis lichenoides or lymphomatoid papulosis.

The image below is of Histiocytosis X a tumour of Langerhans Cells      

Localised Hyperpigmentation

DAMN standing for drugs, autoimmune, metabolic and neoplastic. PIG standing for post inflammatory, infective and genodermatoses. PAPA standing for pigmented purpuric dermatosis, amyloid, parapsoriasis and acanthosis nigricans. The metabolic causes of localised hyperpigmentation are ochronosis, chloasma and porphyria cutanea tarda.

Clinically localised hyperpigmentation should have you thinking about a fixed drug reaction or morphoea if on the trunk and a pigmented purpuric dermatosis if on the lower leg where the pigmentaion is due to hemosiderin from extravasated red cells..

Circumscribed hypermelanosis
• nevus of Hori • lentigo • Becker’s nevus • ephelides • post-inflammatory    hyperpigmentation • familial periorbital    hyperpigmentation • partial unilateral    lentiginosis • erythema ab igne • prurigo pigmentosa • xanthoma planum • inherited pattern    lentiginosis in blacks • acquired brachial    cutaneous    dyschromatosis • Riehl melanosis • cutaneous amyloidosis • Laugier-Huinziker syndrome • atrophoderma of    Pasini-Pierini • fixed drug eruption • erythema dyschromicum perstans

Erythema dyschromicum perstans

Lichen Planus

The thing to remember about it is it is five of everything. Five lesions namely papular, hypertrophic, atrophic, pigmented and bullous. Five shapes guttate, plaque, confluent, annular and linear. Five sites hair, palms, soles, nails, mucus membranes and face with lichen planus actinicus. Associated diseases include drugs, colour film exposure, graph versus host, lichen planus and lupus erythematosus, lichen nitidus and Nekams disease.

The image below is LP back

Lichen planus actinicus

Perforating Disorders

They are primary or secondary. The primary causes are elastosis perforans serpiginosa, reactive perforating collagenosis and uraemic follicular keratoses. A lot of other conditions can perforate or eliminate tissue through the epidermis from the dermis. eg perforating granuloma annulare particularly on the fingers  and some deep fungal or mycobacterial infections.

Perforating Kyrle's disease

Cold Lesions Infantile cold lesions include cold injury, sclerema and fat necrosis of the newborn. Excess cold exposure gives frostbite and trench foot. Single tissue disease gives cold erythema, cold urticaria and cold panniculitis. Blood problems can be due to cryoagglutins, cryoglobulins and cryoprecipitins and the seven syndromes are perniosis, livedo reticularis, Raynaud’s, acrocyanosis, erythrocyanosis and Rowell’s syndrome which is erythema like lesions plus or minus perniosis and vasculitis or Lupus erythematosus. The image below is cold vasculitis Other Diseases with Multiple Myeloma Like Blood Features    Include amyloid, plasmacytoma and cryoglobulinemia. Other Diseases with Paraproteinemia’s Include scleredema, scleromyxedema, necrobiotic xanthogranuloma, Sweet’s syndrome, pyoderma gangrenosum and epidermolysis bullosa acquisita. The other one is subcorneal pustular dermatosis. The image below is necrobiotic xanthogranuloma

These were some of Ken Paver’s Favourite slides and if you have a visual imagination you can see how I would get them wrong.

The blue lower leg due to phlegmasia caerulea dolens.

 The claw like hand with traumatised nails due to syringomyelia.

 The insect bitten leg actually due to follicular perniosis. 

The dripping urticaria due to cold shower urticaria. 

The carcinomatosis erysipelas looking like a plant contact dermatitis.

 Malignant syphilis, the blonde haired boy with bad impetigo. 

The atypical childhood PRP looking like ichthyosis vulgaris on the face. 

The fat child with red/blue face and arms due to a cold injury.

 The lichen plano pilaris slide looking like Darier’s or transient acantholytic dermatosis.

The image below is of Cold Urticaria

Tutorial 31

 Topics discussed in this Tutorial

Squamous diseases DD, Purpuric blisters, Intertrigo non healing, Earlobe infiltrates, Interesting Slides, Papules Acral, Penile plaques, Vegetating lesions dd,  Desquamation, Angular cheilitis, Ear fissures, Seborrhoeic dermatitis, Photosensitivity, 

The next few sections will cover the differential diagnosis of squamous disorders, of purpuric blisters and axillary erythematous squamous rashes.

The Differential Diagnosis of Squamous Diseases

The three Ps, the three Ls and the keratoses. The three Ps are psoriasis, the pityriasis types and post inflammatory. The three Ls are lichen planus, lupus and the loose epidermis of bullae as in pemphigus foliaceus and Grover’s disease. The keratoses - punctate keratoses, the ichthyoses, the porokeratoses and Norweigian scabies.

The image below is of a patient with generalised scabies and erythroderma

Purpuric Blisters

Generally indicates sub basal lamina lucida split. You can see them with vasculitis and infection. They can be traumatic or factitial or other blistering disorders in someone with a bleeding problem. Blood in blisters is also commonly seen in EBA , PCT , cicatricial pemphigoid and surprisingly sometimes in bullous pemphigoid. Of these four conditions bullous pemphigoid blisters occur on a red inflamed base. The other three occur on normal coloured skin.

Intertrigo That Will Not Heal

Consider the diagnosis is actually Hailey Hailey or Darier's  disease.  Consider also Sneddon-Wilkinson disease and secondary infection with candida. Axillary granular parakeratosis is a more modern persistent contact irritant reaction that might present as a non healing intertrigo. It may be a contact allergy to some medication being applied.

Axillary granular parakeratosis

Hailey Hailey disease

Symmetrical Lividity

Seen in young men with cold sweaty cyanotic extremities with some hyperkeratosis. The hyperhidrosis can follow tinea.

The image below is of Hailey Hailey disease

Infiltrates in the Earlobe

If there is a squamous reaction as well consider lupus vulgaris but the differential diagnosis is leprosy, sarcoid, lupus, chilblains and lymphocytoma cutis.

This is an image of leprosy of the earlobe

Other Slides of Interesting Features

Acropustulosis of Hallopeau (sometimes called acrodermatitis). It involves the fingers and the nails are also involved. 

Soft corn presents as a white intertriginous area between the fourth and fifth toes. 

Scabies may present as keratotic lesions in and on the ears especially in the Norwegian scabies. 

Psoriasis, note the salmon pink colour, the three types of scale, the abrupt edges and it is monomorphic. Consider also psoriasis over the sacrum versus lichen simplex chronicus. The latter is usually itchy and may show superficial features of lichenification. Psoriasis in a child can be atypical. 

Pityriasis rosea, note the papular variant. Often you get three crops of pityriasis rosea. 

When pityriasis rubra pilaris affects the scalp you have an early scaling stage before the more typical thick psoriasiform scale will occur. Fine scale on the scalp is due to atopy, ichthyosis, pityriasis rubra pilaris or a fungus or seborrhoeic dermatitis. 

Secondary syphilis on the soles of the foot is a lovely copper colour with a very fine scale.

 Acanthosis nigricans presenting as angular cheilitis is often not black but you have the wart like thickening.

 Scabies of the nipple present as excoriated dermatitis of the nipple. 

Candidiasis of the vulva can be papular but it is still itchy.

 Watch xantho erythroderma, which is a variant of large plaque parapsoriasis with yellow splashed on lesions, which can look digitate. 

Levido with summer ulceration. There is usually an associated venous stasis with fibrinolytically exhausted vessels leading to thrombosis and ulceration. 

Cat scratch disease may present with a sore on the face and big glands in the neck. 

Annular lesions on the forehead are due to sarcoid, necrobiosis lipoidica, granuloma annulare and leprosy.

The image below is of Acropustulosis of Hallopeau The atrophy and loss of the nails are the features that distinguishes this condition from pustular dyshidrotic eczema.

The Differential Diagnosis of Papules on the Palms and Soles

Papules include Reiter’s disease, yaws, arsenic, syphilis and porokeratoses. Squamous disorders include psoriasis, pityriasis rubra pilaris, lichen planus and tinea. If you suspect tinea on the palms of the hands look on the back of the hands and look for follicular inflammation.

Other Observations Pityriasis rosea is often atypical in children. Often it is on the face or acral and it can be papular. 

The papules of secondary syphilis are seen at the angles of the mouth and the nose. 

Paronychia in Reiter’s disease is painless. 

In PRP of the face look for the overall erythema and fine scale.

 In Darier’s disease there is no real erythema. 

In seborrheic dermatitis there is a greasy scale that is easy to remove and in pityriasis rubra pilaris there is erythema with an acuminate scale and keratin. 

In the secondary stage of syphilis look for the pigmentation, especially the late secondary stage, as a measure of the inflammatory process.

 Remember that oval lesions on the chest can be seen in pityriasis lichenoides as well as pityriasis rosea.

The image below is vasculitis of palms

Red Plaques on the Penis   

Consider Erythroplasia of Queyrat, syphilis, Reiter’s, psoriasis, candidiasis and Bowen’s disease.

Compare tinea barbae with bacterial folliculitis.   In tinea barbae the hairs fall out and it is often in a country worker whereas in bacterial folliculitis the pustule is around the hair follicle and it is usually painful and the hairs are often intact.

The image below is of psoriasis on the glans.

Vegetating Lesions   

On the legs these are often deep mycoses, psoriasis, lichen planus, Darier’s and secondary to lymphedema.
On the flexures consider all the pemphigus types, pemphigoid, Darier’s, Hailey Hailey disease and iododerma. Pemphigus vegetans when it affects the mouth is often worse at the edges. Epidermodysplasia verruciformis can give psoriasis like plaques on the arms but curiously it can also give small white hypopigmented spots like plane warts.

The image below is Darier's disease

Desquamation    

Desquamation is really non-recurring exfoliation. Consider the bullous diseases such as staph scalded skin syndrome, toxic epidermal necrolysis and epidermolysis bullosa. Secondly consider dermatitis particularly pompholyx and post dermatitis. Thirdly consider drugs particularly the Retinoids and Vitamin A and fourthly consider toxins as in scarlatina, toxic shock syndrome and staph scalded skin. Palms and soles can take up to 56 days to re epithelialise the epidermis.

The image below is bullous impetigo

Causes of Angular Cheilitis   

Consider the mnemonic VISA. The V stands for vitamin deficiency particularly Iron, acrodermatitis enteropathica, agranulocytosis, leukemia and Downs syndrome. The I stands for infection, candida, staph, strep and syphilis. The S stands for other skin diseases including atopic dermatitis, lichen planus, pemphigus and acanthosis nigricans and A for anatomical which simply means a drop in the vertical dimensions of the face due to resorption of the gums with aging. This is probably one of the commonest causes in the elderly of angular cheilitis.
In younger individuals on oral retinoids for acne, this problem is particularly common.

Fissures Around the Ear

Consider granuloma fissuratum due to glasses that are ill fitting. Note that 30% of atopic children have a superolateral fissure behind the ear and also consider seborrheic dermatitis and psoriasis behind the ear causing fissures. Darier’s disease when it is severe can cause fissures here as well but the keratotic papules are usually quite obvious.

Probably better named acanthoma fissuratum

Lupus Vulgaris

90% of cases occur on the face. They are usually symmetrical plaques with brown colour with scarring. The other types that can be seen include plaques, which ulcerate or tumour like or vegetating and there are multiple small papule type, sometimes known as acnitis.   The lesions are soft and should show the apple jelly sign. In non-bullous ichthyosiform erythroderma the erythema tends to persist whereas in bullous ichthyosiform erythroderma the erythema goes first. So if you see marked erythema it is non bullous.

Seborrhoeic Dermatitis

Crusting is superficial and greasy with an underlying erythema. If you are considering seborrhoeic dermatitis of the groins check just beyond the erythema for the typical scale. The types of seborrhoeic dermatitis are the petaloid, annular with a finer scale on the inside area, seborrhoeic dermatitis of the scalp gives erythema and starts around a hair follicle, Darier’s disease of the scalp gives no erythema and a pit is left when the scale or papule is removed. Follicular seborrhoeic dermatitis gives an acne like rash on the back. Note that seborrhoeic dermatitis morphologically can be eczematous, psoriasiform, pityriasis like or generalised with erythema. Note that seborrhoeic dermatitis on the body is mainly central and symmetrical. Consider how you would separate Darier’s disease and seborrhoeic dermatitis. Consider the nature of the scale, erythema, the nails, the palms, the mouth and also the white macules you may see in Darier’s.

The image below is mild seb derm

Poikiloderma

Note the types of poikiloderma. Check for a lack of pigment around the follicles on the neck. In dyskeratosis congenita and Fanconi’s syndrome you do get poikiloderma. In dyskeratosis congenita the nails are involved early. You start to get paronychia at 8 to 10 years of age. You may get leukoplakia and form bullae in the mouth and it is an X linked recessive.

Poliosis

Consider the Alezzandrini syndrome giving unilateral vitiligo, retinitis which is unilateral and bilateral deafness.

Compare nevus anemicus and nevus depigmentosis. In nevus depigmentosis the hair is white and in nevus anemicus the stroke test to reduce surrounding erythema will cause the lesions to look the same as surrounding skin.

Nevus anaemicus

Photosensitivity Disorders Such as Pellagra

Note the distribution of this rash particularly in sun exposed areas on the back of the hands, the V of the neck and the face. The eroded split skin dermatitic look of it and if anyone has a light eruption always look in the mouth and see if there is any evidence there. In pellagra the tongue is involved.

Pellagra back of hand

The image below is pellagra of the face. 

Some other points

 In non-addisonian generalised pigmentation the mnemonic is DAMN. (Drug Autoimmune Metabolic and Nutritional) The metabolic ones are due to porphyria, hemochromatosis or pellagra. 

Note also in the lesions of secondary syphilis the scale is all over the lesion compared with the peripheral scale of pityriasis rosea and the scale of candidiasis is right on the outside.

 If at any time you seem to have a unilateral seborrhoeic dermatitis on the face be aware of Bowen’s disease and a superficial BCC in the nasal labial fold leading you astray.