Tutorial 9

Topics covered in this tutorial

Generalised erythroderma in an infant and newborn

Some aspects of photosensitivity in children

The differential diagnosis of crusty papules

        The following topics will also be considered
        Features of the basal cell nevus syndrome
        Scrub typhus
        Differential diagnosis of perleche
        Acne necrotica
        Kwashiorkor

Erythrodermic psoriasis

Generalised Erythroderma in an Infant or Newborn Usually subclassified as Inflammatory, Infectious, Genodermatoses, Metabolic diseases and Immunological

The causes include -
        The staph scalded skin syndrome
        Toxic erythema of the newborn
        Erythema neonatorum
        Bullous and Non Bullous Ichthyosiform erythroderma
        Leiner's disease
        Generalised candidiasis
        Atopic dermatitis
    Netherton's Syndrome
Wiskott Aldrich Syndrome - Atopic dermatitis like rash with hemorrhagic component, skin abscesses, low platelets, diarrhoea, nose bleeds
Conradi Hunerman syndrome
Job syndrome (Hyper IgE) Acne like rash, abscesses and AD like rash
Omenn syndrome neonatal erythroderma, infections
Diffuse Mastocytosis
Staph Scalded Skin- Usually an umbilical infective source of Staph

View the video of generalised erythroderma Newborn and Infants

        

Staph Scalded Skin

Photosensitivity Disorders in Children

Consider the following
        Hydroa vacciniforme
        There is scarring. Mainly children.
        The lesions are polymorphic
        It is on the face
        Fluid filled vesicles with varioliform scarring

Polymorphous Light Eruption

        It is monomorphic
        Papules and plaques
        The lesions do not last long.

Juvenile Spring Eruption

        It is non-scarring
        It mainly in boys on the ears and it crusts

Hutchinson's summer prurigo
        non-scarring
        Covered areas as well
        Usually they are atopic with photosensitivity and it usually goes before puberty.

EPP Erythropoetic Protoporphyria 

Sun Sensitivity from an early age with smalll blisters and scarring on the bridge of the nose. Increase in red cell protoporphyrins. Later biliary and hepatic issues.

Other issues in Photosensitivity
1. Which dermatoses are exacccerbated by sunlight?
2. Which photosensitivity disorders are familial?
3. What are the clinical differences between phototoxic and photoallergic dermatoses?
4. What are the major substances causing photocontact dermatitis?
5. What are the main genodermatoses associated with photosensitivity?
6. Which porphyrias have associated photosensitivity?

View Video of Photo Disorders in Children

The image below is of Juvenile Spring Eruption

The Differential Diagnosis of Crusted Papules

These include
        Solar keratoses
        Keratoacanthomas
        Perforating diseases on the legs particularly Kyrle's disease
Also include
        Papulo necrotic tuberculid where the lesions scar, are monomorphic and painful
        Prurigo nodularis where the lesions are usually few particularly on the forearms and neck area and are very itchy

The image below is of Prurigo Nodularis

The Differential Diagnosis of Perleche    See Dermnet

These include physical causes and infective, vegetative and deficiency disease and syndromes, particularly Down's syndrome. Also consider syphilis and lichen planus and candidiasis.
In approaching the diagnosis of Perleche, always check the teeth, the mouth, the tongue.

Candida lip and angular cheilitis

Other Things to Note Trichoepitheliomas usually occur at puberty in the nasolabial folds with some telangiectasia. Compare this with adenoma sebaceum which tend to occur in childhood again in the nasolabial fold areas around the mouth but are much redder. Syringomas on the chest look like disseminated granuloma annulare. Trichoepitheliomas are very superficial. Compare this with syringomas that are usually seen around the eyes but are deeper. Trichoepitheliomas Pachyonychia Congenita - Black thick nails, palmoplantar keratoderma, leukoplakia and sebaceous cysts. Image nails    Atlas Images Pellagra - is usually seen on the hands and other photo exposed areas with a weeping dermatitic like reaction that gives hypopigmentation and hyperpigmentation. The image is of palmoplantar keratoderma due to pachyonychia congenita Pachyonychia congenita Kwashiorkor - is usually mild in the elderly with erythema, scaling and oedema. In children you get skin dyschromia. Hair may show the flag sign. The skin may show redness, scaly and oedema looking like flaky paint. There is marked hyperproteinuria. Chloracne - is generally seen in individuals who have ingested a toxin. An acne like rash is usually on the arms and in sun exposed areas. Generally it is in older people, so it is wrong people, wrong time and wrong place in relation to ordinary acne. Excess comedones in the wrong places. Pellagra face Pellagra hand Kwashiokor Skin Calcification in Scleroderma - is usually seen in the fingertips. It is congregated and seen usually in adults. Calcification in dermatomyositis is usually in the muscles. It is diffuse and is seen in children.   View Calcification Overview Healing with Pigmentation - In pemphigus there are usually few blisters and no healing with pigmentation. Compare this with pemphigoid, lichen planus and dermatitis herpetiformis where there is healing with pigmentation. These conditions involve collateral damage to the basal layer melanocytes. The image below is of extensive skin calcification around the knee in a patient with severe scleroderma.

Acne Necrotica is generally in the scalp margins. There are often secondary staph infected papules which are excoriated. They may heal with varioliform scarring. Histology shows a lymphocytic folliculitus with apoptotic cells.

Acne Necrotica Miliaris is usually seen in the scalp. The scalp is itchy and due to infection by Propionibacterium acnes. These dot not heal with varioliform scars.

Acnitis (Acne Agminata) (lupus miliaris disseminatus facei) gives translucent yellow brown papules around the eyelids, cheeks and rarely axillae. Heal with small scars. Histology - granulomas both tuberculoid and sarcoidal.

Seborrhoeic dermatitis seldom shows pustules unless there is some secondary candidiasis.

Rosacea shows papules, pustules with telangiectasia and erythematous plaques. It may also occur on the arms. There are often eye signs and watch for lymphedema. In rosacea there are perifollicular papules which are non tender. Compare this with the tender follicular papules of acne.

Acne necrotica