July 2019 | Case of the Month

The diagnosis is the CREST syndrome, which comprises:

• Calcinosis (the piece of white tissue present in the photograph) 
• Raynaud's phenomenon (which the patient suffered from) 
• OEsophgaeal involvement (hence the proton pump inhibitor in the history)
• Sclerodactyly
• Telangiectases

The CREST syndrome is best viewed as a subgroup of systemic sclerosis, with a better overall prognosis.

Hand changes can include:

• Atrophy begins first in the nail pulps causing small painful ulcers that leave pitted scars. With time the nails become much smaller and the whole distal finger becomes atrophic 
• Sclerodactyly - fingers become swollen, and the skin feels tight and has a shiny appearance. The fingers become less mobile
• Nailfold changes - include enlargement of capillary loops, disorganisation of the normal distribution of capillaries, budding capillaries, extravasates, and loss of capillaries. There does not appear to be a correlation between the degree of nailfold change and the severity of systemic sclerosis 
• Telangiectases
• Calcinosis (calcium deposits) develops in the skin, particularly the fingers, hands and other bony areas. These can breakdown and discharge chalky material
• Ulceration and gangrene may occur with minor trauma, or at the tips of the fingers, as a result of poor circulation

For more information refer to the chapter on systemic sclerosis.

On other matters, for those interested in dermoscopy I have just written an interesting blog on white networks.