Paediatrics: Connective tissue disorders

Connective tissue disorders

Skin manifestations of connective tissue disor-ders include the following:

Systemic lupus erythematosus

Widespread or ‘butterfly rash’ facial erythema, scalp alopecia, chronic dis-coid patches, light sensitivity.

Dermatomyositis

Violaceous erythema +/– oedema of face (especially eyelids), upper chest, elbows, knees, knuckles, around nails. Rash may become scaly.

Morphoea

In this idiopathic disorder there is localized sclerosis of the skin. Usually an enlarging large oval plaque of violaceous hue develops which then gradu-ally becomes indurated, smooth, and shiny. Usually resolves spontane-ously. Treat severe facial or restrictive linear morphoea with pulsed IV methylprednisolone and oral methotrexate.

Systemic sclerosis

This condition manifests as Raynaud’s phenomenon; finger tip ulceration; skin of the face and hands becoming progressively indurated and ‘bound down’ to underlying tissues; restricted facial movements; beaked nose; mouth puckering; skin atrophy; telangiectasia; pigmentation; calcinosis.

Cutaneous polyarteritis nodosa

Tender  nodules  (usually  lower  legs)  surrounded  by  livedo  reticularis.

Nodules may ulcerate or become necrotic.

Lichen sclerosus et atrophicus

In this idiopathic chronic inflammatory skin disorder localized distinct atrophic changes with associated pallor usually affect genital and perianal regions, almost always in females (the male variety is balanitis xerotica obliterans, which causes phimosis). Pruritus, blistering, or erythema may occur. Treat with emollients, or potent topical steroids if severe.