Tutorial 12

Topics discussed in this Tutorial

White spots DD

Papular dermatitis childhood DD

Localised hyperpigmentation

Congenital localised hyperpigmentation

Lipoatrophy and Lipodystrophy

Granulomas with scars

Violaceous coloured face DD

Compare pemphigus foliaceous, vulgaris and Pemphigoid

 Differential Diagnosis of White Hypopigmented Spots

This is a difficult differential. Using the SIGN DIP mnemonic, the main Squamous diseases are psoriasis, pityriasis rosea, pityriasis lichenoides and pityriasis Alba. The infective ones are syphilis, pinta and leprosy, granulomatous is sarcoid, neoplastic is halo nevus. Drugs are various hypopigmenting chemicals such as phenols. The immunological inflammatory diseases are lichen sclerosis , morphea, scleroderma, lupus and the Vogt-Koyanagi syndrome. Physical causes are radiodermatitis, and idiopathic guttate hypomelanosis. Remember also Degos syndrome and Albo papuloid epidermolysis bullosa.

The image below is pityriasis alba.

The differential diagnosis of papular dermatitis in childhood includes the Gianotti-Crosti syndrome. Juvenile papular dermatitis, which is like Gianotti-Crosti but there is no liver involvement, lymphadenopathy and there are discreet lichenoid lesions on the hands and forearms. There is also a frictional lichenoid eruption of childhood, which is on the knees and the back of the hands. Caterpillar dermatitis, mites and scabies can all cause a papular dermatitis. This image is of the Gianotti- Crosti syndrome Localised hyperpigmentation. It is difficult to get an encompassing Mnemonic that works! Clinically look out for fixed drug reaction and localised scleroderma or the Pasini Pierini variant of morphoea.  Image The mneumonic we used is DAMN PIG PAPA . D for drugs, A for autoimmune, M for metabolic diseases such as ochronosis and N for neoplastic including metastases and mast cell tumours. P is for post inflammatory, I was infective causes and G was granulomatous diseases. The PAPA is P for parapsoriasis, A for amyloid, P for pigmented purpuric dermatosis and the other A for acanthosis nigricans. The image is of a fixed drug reaction.    Congenital localised hyperpigmentation. If multiple spots it was the Peutz-Jeghers syndrome, urticaria pigmentosa or the leopard syndrome. If it occurred in a patterned form it was incontinentia pigmenti, Albright’s disease and Fanconi’s syndrome. If there were café lait spots it was neurofibromatosis, Albright’s or Gaucher’s syndrome. Naegeli’s syndrome is like incontinentia pigmenti but with no previous inflammation. The image is a case of mastocytosis Localised Lipoatrophy can be annular lipodystrophy or post inflammatory, for example after leprosy, lupus or scleroderma. It can be due to drugs such as insulin and corticosteroids. It can be nevoid. The other drugs that commonly cause Lipo atrophy are the drugs used to treat AIDS. HAART drugs commonly cause this problem. Lipodystrophy presents either as a depression, lipoatrophy , or as an induration of the skin. Lipohypertrophy is due to changes in the subcutaneous tissue. Some of the cases of Lipoatrophy associated with HAART have seen a redistribution of subcutaneous fat with accumulation of fat in abdominal and cervical areas. Some 25% to 50% of patients taking HAART drugs develop some form of Lipo dystrophy. Protease inhibitors reduce fat formation in a dose responsive manner. The most common form of Lipoatrophy probably follows the various types of panniculitis. Remember some of the acquired partial Lipodystrophies can be associated with a reduction in the C3 level and with nephritis. Lipohypertrophy can be due to repeated injections of insulin. Normally we think of insulin causing some degree of Lipoatrophy. It is independent of the type of insulin used. Growth hormone injections have also caused Lipohypertrophy. Rotating injection sites tends to stop the Lipohypertrophy forming. Total fat atrophy can occur in a congenital form when it usually is before the age of two with hepatomegaly, xanthomata, an increase in bone growth and an insulin resistant diabetes. In adults when it occurs there is often acromegaly but no hepatomegaly and they may well show acanthosis nigricans as a measure of some insulin resistance. A degree of fat atrophy commonly occurs in scleroderma and there is the Parry Romberg variant when you get facial hemiatrophy. See Dermnet on Morphoea  Fat atrophy from HAART drugs Lupus Vulgaris It presents as a granuloma with scars. The diffuse edge shows that it is not a neoplastic disorder. On the legs it may look like psoriasis but it has scars. What are the various tuberculids? See Dermnet on Cutaneous TB The image is of lupus vulgaris Compare pemphigus foliaceus, pemphigus vulgaris and pemphigoid. Pemphigus foliaceus is usually in age groups of less than 60. Involves the chest and the face, slow with scaling and crusting and spreads to the upper body, mucous membrane involvement is uncommon. Pemphigus vulgaris is also in the less than 60 age group. The mouth is commonly involved. It is slow to spread. Presents as erosions and crusts and can go anywhere. Mucous membrane involvement is common. Pemphigoid typically is in over 60 year olds. It begins often in the flexures and can be localised to the leg. It evolves much faster than pemphigus. You tend to have intact bullae, which may be haemorrhagic. It is seen particularly on the trunk and limbs and mucous membrane involvement is uncommon. This is pemphigus foliaceous If you have a violaceous hue on the face what do you consider in the differential diagnosis? Answer You should consider dermatomyositis or porphyria variegata. If it is all over the face consider Cushing’s or porphyria. Always watch for lupus erythematosus and porphyria occurring together and watch the pill aggravating porphyria variegata. The Carcinoid syndrome can cause an intermittent violaceous flushing.

Tutorial 29

 

The following topics are discussed in this Tutorial.

White spots DD, Bites, Lymphedema DD, Pigmentation, Molluscum bodies, Bywaters lesions, Subcorneal pustular dermatosis, Ehlers Danlos,Sclerodermoid changes, Sinuses, Verrucous elbows,

Differential Diagnosis of White Spots

The mnemonic for congenital causes is WANT A DIP (of COLOUR) and then SIGN DIP. Remember albopapuloid epidermolysis bullosa, Degos disease, lichen sclerosis et atrophicus, halo nevi, vitiligo, anetoderma, follicular atrophoderma, idiopathic guttate hypomelanosis and drugs. Try searching in GSA under hypopigmentation

The image below is extragenital lichen sclerosus

Bites

Caterpillars tend to give lots of lesions, they are grouped and there may be associated urticaria. Ticks give few lesions, nodules with necrotic centres. Spiders tend to give few lesions, with pain, induration and necrosis. Bird mites are often on the top of the body and look for a central punctum. The Centipede has a single bite like a spider. Scabies gives multiple urticarial lesions sometimes with burrows particularly in the web spaces, axillae and groin and myiasis are found in a wound with maggots or as cutaneous myiasis presenting as boils with discharge or as a creeping eruption.

The image below is of a centipede bite

Pediculosis

Pediculosis capitis tends to present with an itchy scalp. Pediculosis corporis presents with urticarial bites and pediculosis pubis presents with eczema, infection and sometimes pigmentation.

Pediculosis pubis 

Differential Diagnosis of Lymphedema

There is primary and secondary lymphedema. The congenital type is due to hypoplasia or absence of lymphatic vessels. The acquired type can follow infection, tumour, irradiation, surgery, frostbite or worms.

Congenital lymphedema and secondary skin changes

Pigmentation

The mnemonic is DAMN ANNE. Non Addisonian pigmentation is DAMN standing for drugs, autoimmune, metabolic and nutritional. Addisonian pigmentation is ANNE standing for autoimmune, neoplastic, nutritional and endocrine.

Pigmented Spots in a White Area

Pigmented spots in a white area is due to vitiligo with repigmentation or piebaldism.

Piebaldism

Blotchy Hypopigmentation of the Skin

Consider incontinentia pigmenti, post inflammatory hypopigmentation and pinta

The image below is post inflammatory hypopigmentation

Thickening of Folds

This is present in scleromyxedema, it is bound down in scleroderma and smoothed out in scleredema.

The Melkerson-Rosenthal syndrome is bilateral on the cheeks. The lips are always swollen top and bottom. It can occasionally be unilateral on the cheeks.

Melkerson Rosenthal syndrome Swollen lip red face

Sycosis barbae tends to give a plaque type of erythema with deep inflammed follicles. It leaves scarring. In the lupoid form you tend to have active papules and pustules around a pink atrophic scar.

Hereditary Haemorrhagic Telangiectasia   

In hereditary haemorrhagic telangiectasia the telangiectasia can be linear or punctate. Note the pulmonary arteriovenous anastamoses or shunts.

Molluscum Bodies

This is not a good term. It can be associated obviously with molluscum contagiosum but it is usually in association with neurofibromatosis although these lesions can also be seen in the Ehlers-Danlos syndrome and acrodermatitis chronica atrophicans.   

Bywaters Lesions

Bywaters lesions( small ulcers on finger pulps) are seen in rheumatoid arthritis whereas Janeway lesions( non tender macules, papules or nodules present on the palms and soles) are seen in subacute bacterial endocarditis.

Types of Pustular Psoriasis Subcorneal pustular dermatosis has pustules at the edge, the mouth is not involved and neither are the nails. You can get it in the scalp. It is not painful. Watch for an IGA gammopathy and typically it will present with flaxid oval vesicles. Sometimes with a meniscus line in the pustule.  Compare with pustular psoriasis of the Von Zumbusch’s type where the patient is toxic and the mouth and perioral areas and nails are all involved. There is a fiery red background and it is painful. Remember also the various localised types of pustular psoriasis including palmar plantar and acropustulosis of Hallopeau. Subcorneal pustular dermatosis Leprosy Morphologies          Macules are seen in all stages. Indeterminate is always and only macular. Borderline tuberculoid has a sharp inner edge to an annular lesion. Tuberculoid has a sharp outer edge. Borderline tuberculoid also has annular and has bands. Xanthomas    Eruptive show an increase in triglycerides, xanthelasma shows an increase in cholesterol, tendinous show Type 2 lipids, tuberous Type 3 and palmar Type 2. Psoriatic Arthropathy It is usually distal, can be mutilating, its rheumatoid like, it is facetal in the spine and it can also be ankylosing spondylitis like. With spondylitis it is HLAB 27, in distal psoriatic arthropathy it is HLA B13, 17 and 37. The image below is of Tuberous Xanthomas Ehlers-Danlos Syndrome There are nine types. One to three are autosomal dominant, four to eight are autosomal recessive but five and nine are x-linked recessive. The skin signs are an increase in elasticity with a soft velvety feel, joint hyper mobility, bruising, keloids and elastosis perforans serpiginosa. There may be pseudotumors and small nodules on the shins and the palms and soles may show very lax skin. Sclerodermoid Changes in the Lower Leg Seen in stasis, morphoea, scleroderma, Werner’s syndrome, scurvy, acrogeria and localised graft versus host. Also in lipodermatosclerosis. The Image below is Ehlers Danlos Causes of Acanthosis Nigricans The mnemonic is BLACK SPOT. Blooms, Leske’s, Lawrence-Seip, ataxia telangiectasia, Cruzons disease, Sjogren Larsen, Prader-Willi, poly endocrine, Rudds and Downs Syndrome. Infected Lesions with Sinuses Conditions to consider include botryomycosis, scrofuloderma, others forms of TB, ischiorectal abscesses, amoebiasis, actinomycosis, Madura foot, osteomyelitis, lymphogranuloma venereum, pasturella multicida. Note a pyogenic granuloma like lesion on the cheek is a dental abscess. Verrucous Lesions on the Elbows These are seen as psoriasis, epidermal nevus, dermatomyositis, pityriasis rubra pilaris, lipoid proteinosis and epidermo dysplasia verruciformis. The image below is a montage of acanthosis nigricans