Dermatology, Venereology and Leprosy
Collagen Vascular Disease and the Skin
Connective tissue disease skin signs for MBBS and NEET-PG: lupus malar and discoid rash, dermatomyositis, systemic sclerosis, CREST and Raynaud's, mapped to NMC codes DR16.
MedNext Academy | 3 min read
Collagen Vascular Disease and the Skin
Connective tissue disease skin signs for MBBS and NEET-PG: lupus malar and discoid rash, dermatomyositis, systemic sclerosis, CREST and Raynaud's, mapped to NMC codes DR16.
The collagen vascular or connective tissue diseases produce skin signs that often reveal the systemic diagnosis. This chapter covers the cutaneous lupus patterns and their serology, the heliotrope rash and Gottron's papules of dermatomyositis, systemic sclerosis with the CREST features and Raynaud's phenomenon, and the treatments that centre on photoprotection and immunosuppression.
High-yield: Collagen Vascular Disease and the Skin
- Connective tissue diseases such as lupus, dermatomyositis and systemic sclerosis frequently show diagnostic skin signs.
- The malar or butterfly rash of systemic lupus erythematosus spans the cheeks and nasal bridge but characteristically spares the nasolabial folds.
- Lupus skin disease is photosensitive, so lesions worsen on sun-exposed areas.
- Discoid lupus erythematosus produces chronic scaly plaques with follicular plugging that heal with scarring and pigment change.
- Antinuclear antibody is a sensitive screening test for lupus, while anti-double-stranded DNA and anti-Smith antibodies are more specific.
- Dermatomyositis features a heliotrope rash, a violaceous discolouration of the eyelids, and Gottron's papules over the knuckles.
- Dermatomyositis in an adult can be a paraneoplastic marker, so a search for an underlying malignancy is warranted.
- Systemic sclerosis causes skin tightening, and the CREST variant comprises calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia.
- Raynaud's phenomenon, a triphasic colour change of the digits to cold, is common to several connective tissue diseases.
- Sclerodactyly is tightening of the skin of the fingers, which can restrict movement and cause ulceration at the tips.
- Photoprotection and sun avoidance are important in all photosensitive connective tissue skin disease.
- Topical corticosteroids and antimalarials such as hydroxychloroquine are used for cutaneous lupus.
- Systemic immunosuppression is required when internal organs are involved.
- The skin findings often precede or point to the systemic diagnosis, which makes their recognition clinically valuable.
Skin signs of connective tissue disease
- **Lupus:** Malar rash sparing nasolabial folds, photosensitive, discoid scarring plaques.
- **Dermatomyositis:** Heliotrope eyelids, Gottron's papules; may be paraneoplastic in adults.
- **Systemic sclerosis:** Skin tightening, sclerodactyly, CREST features.
- **Common thread:** Raynaud's phenomenon; photoprotection and immunosuppression.
NMC competencies in this chapter
- **DR16.1:** Identifying and distinguishing the skin lesions of SLE
- **DR16.2:** Cutaneous features of connective tissue diseases and their management
Frequently Asked Questions
What is characteristic of the lupus malar rash?
A butterfly-shaped erythema over the cheeks and nasal bridge that spares the nasolabial folds and is photosensitive, worsening after sun exposure.
What skin signs suggest dermatomyositis?
A heliotrope violaceous rash on the eyelids and Gottron's papules over the knuckles. In adults, dermatomyositis can be a paraneoplastic marker, warranting a search for underlying cancer.
What does CREST stand for?
Calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia, a limited form of systemic sclerosis.
How is cutaneous lupus treated?
With strict photoprotection, topical corticosteroids and antimalarials such as hydroxychloroquine, escalating to systemic immunosuppression when internal organs are involved.
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