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Skin and Mucous Membranes

Several conditions in paediatrics involve the skin and mucous membranes. Some are multi-system diseases that includes musculoskeletal manifestations related with a connective tissue disorder. The Table is a quick guide to common mucocutaneous features in conditions such as Juvenile Idiopathic Arthritis, Juvenile Systemic Lupus Erythematosus, Juvenile Dermatomyositis, Vasculitis, Scleroderma, among others. More information about these conditions is available. 

Changes can be vary, be subtle and can easily missed. There is also racial variation in how mucocutaneous features may appear and the following resources are useful.

Welcome to Skin Deep - Skin Deep (dftbskindeep.com)

https://www.nottingham.ac.uk/research/groups/cebd/resources/skin-of-colour/index.aspx

Feature

Description

Related diseases

Malar rash

Butterfly red or violaceous erythema. It appears on the cheeks, bridge of the nose, but could extend to the forehead and chin.

Juvenile Systemic Lupus Erythematosus
Juvenile Dermatomyositis (similar)

Cutaneous lupus

Round scaly plaques that appear on the face, could be present with hair loss and scarring.

Juvenile Systemic Lupus Erythematosus
Juvenile
Cutaneous Lupus

Acute Rheumatic Fever

Oral ulcers / vasculitis

The most common variety are palatal erythematous ulcers, that are painless.

Juvenile Systemic Lupus Erythematosus

Vasculitis rashes

A variety of rashes, the most classic is “palpable purpura” that usually appear in the limbs.

IgA Vasculitis (Henoch-Schonlein purpura)

ANCA-associated vasculitis

Juvenile Systemic Lupus Erythematosus

Antiphospholipid syndrome

Other systemic vasculitides

Heliotrope rash

Bilateral red/purple discolouration of the upper eyelids, often with swelling of eyelids and skin around the eyes.

Juvenile Dermatomyositis

Gottron papules

Erythematous to violaceous papules and plaques found over bony prominences, especially over metacarpophalangeal and interphalangeal joints.

Juvenile Dermatomyositis

Scleroderma

The skin becomes unusually thick. Typically, a waxy shiny skin with loss of adnexa is seen. Morphoea is a common name to this condition.

Juvenile Scleroderma

Sclerodactyly

Localized thickening and tightness of the skin of the fingers.

Juvenile Systemic Sclerosis

Livedo reticularis and racemosa

A mottled or net-like violaceous skin pattern.

Some vasculitides

Juvenile Systemic Lupus Erythematosus

Antiphospholipid syndrome

Juvenile Systemic Sclerosis

Erythema nodosum

The usual presentation is as red tender nodules in the shins that gradually disappear lefting a bruising appearance or post-inflammatory hyperpigmentation.

Inflammatory bowel disease

Juvenile Systemic Lupus Erythematosus/Cutaneous Lupus

Behçet's disease

Sarcoidosis 

Psoriasis

Scaly plaques predominantly on extensor surfaces and head. 

Juvenile Idiopathic Arthritis

Raynaud’s phenomenon

Usually symmetrical and affects digits of hands and feet but may also affect ears and nose.  Pallor, cyanosis, and erythema but not all phases may be present.

If the changes are unilateral then outflow syndromes must be considered (e.g. cervical rib).

Juvenile Systemic Lupus Erythematosus

Antiphospholipid syndrome

Juvenile Systemic Sclerosis

Nail fold capillary changes

Erythema, oedema, and haemorrhages seen in the base of the nails.

Juvenile Systemic Lupus Erythematosus

Juvenile Systemic Sclerosis

Juvenile Dermatomyositis

Chilblains

Erythematous or violaceous patches that appear after cold exposure. Usually appear in the fingers and toes and a feeling of burn of itch could be present.

Juvenile Systemic Lupus Erythematosus

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