Phys · dermatological
Cutaneous Manifestations of Systemic Disease
Also known as cutaneous manifestations of systemic disease · skin signs of internal disease · paraneoplastic dermatology · dermatomyositis · acanthosis nigricans · necrolytic migratory erythema · glucagonoma syndrome · calciphylaxis · calcific uremic arteriolopathy · dermatitis herpetiformis · sign of Leser-Trelat · Trousseau syndrome · migratory thrombophlebitis · Sweet syndrome · acute febrile neutrophilic dermatosis · pyoderma gangrenosum · erythema nodosum · erythema chronicum migrans · nephrogenic systemic fibrosis · pretibial myxoedema
Consultant-physician-depth guide to the skin as a window to systemic disease — a system-by-system framework covering endocrine (acanthosis nigricans, necrolytic migratory erythema of glucagonoma, pretibial myxoedema, diabetic dermopathy), rheumatological (malar rash of SLE, Gottron papules and heliotrope rash of dermatomyositis, sclerodactyly, palpable purpura of vasculitis, erythema nodosum, pyoderma gangrenosum), gastrointestinal (dermatitis herpetiformis and coeliac disease, pyostomatitis vegetans), renal (calciphylaxis, nephrogenic systemic fibrosis), hepatic (spider naevi, palmar erythema), haematological (Sweet syndrome and AML, leukaemia cutis), paraneoplastic (dermatomyositis, malignant acanthosis nigricans, sign of Leser-Trelat, Trousseau syndrome), infectious (erythema chronicum migrans, secondary syphilis) and nutritional (scurvy, pellagra, acrodermatitis enteropathica) clues. Structured for FRACP DWE and DCE preparation.
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- New adult-onset dermatomyositis — roughly 15 to 25 per cent malignancy risk (anti-TIF1-gamma and anti-NXP-2 mark the highest), particularly ovarian, lung, gastric, colorectal and pancreatic; mandates a structured malignancy search at diagnosis and for three years
- Malignant acanthosis nigricans with tripe palms in an older patient — strong pointer to gastric adenocarcinoma; urgent gastroduodenoscopy required
- Sign of Leser-Trelat (sudden eruption of seborrhoeic keratoses) with or without pruritus — investigate for GI adenocarcinoma (gastric, colonic, pancreatic)
- Migratory thrombophlebitis (Trousseau syndrome) — occult pancreatic or other mucin-producing adenocarcinoma until proven otherwise
- Calciphylaxis in a dialysis or advanced CKD patient — painful purpuric plaques that necrose; one-year mortality above 50 per cent from sepsis, requires urgent sodium thiosulfate, wound care and debridement
- Necrolytic migratory erythema with new diabetes and weight loss — glucagonoma until excluded; check serum glucagon and image the pancreas
- Sweet syndrome with cytopenias or blasts — search for acute myeloid leukaemia or myelodysplastic syndrome
- Dermatitis herpetiformis — coeliac disease; strict gluten-free diet reduces enteropathy-associated lymphoma risk; check iron, folate, B12 and bone density
Cutaneous Manifestations of Systemic Disease
The answer first
The skin is a window to internal disease. A single recognised lesion can collapse a differential list from dozens of conditions to one — Gottron papules to dermatomyositis, grouped vesicles on the elbows to coeliac disease, an expanding annular lesion after a tick bite to Lyme disease, painful purpuric plaques in a dialysis patient to calciphylaxis. The discipline of cutaneous manifestations of systemic disease is built on this principle: pattern recognition first, then targeted confirmation. [1]
Three ideas frame everything on this page: [1]
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Recognise the specific lesion, then ask what system it signals. Acanthosis nigricans signals insulin resistance or a GI malignancy; necrolytic migratory erythema signals glucagonoma; palpable purpura signals small-vessel vasculitis; erythema nodosum signals sarcoidosis, IBD, streptococcal infection or a drug reaction; calciphylaxis signals advanced CKD with disordered calcium-phosphate metabolism. Each lesion carries a short, high-yield list of systemic associations — learn the list and you have the answer. [1]
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A paraneoplastic skin sign often precedes the diagnosis of the underlying cancer. Adult-onset dermatomyositis, malignant acanthosis nigricans with tripe palms, the sign of Leser-Trelat, Trousseau migratory thrombophlebitis, necrolytic migratory erythema and Sweet syndrome can all be the first manifestation of an otherwise occult malignancy. Recognising the cutaneous marker is the event that triggers the cancer diagnosis — this is the highest-stakes application of dermatology in physician medicine [8] [13].
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Some skin signs are medical emergencies. Calciphylaxis in a dialysis patient carries a one-year mortality above 50 per cent [4]. A new dermatomyositis in an adult mandates an immediate structured malignancy search. Suspected glucagonoma syndrome demands prompt measurement of glucagon and pancreatic imaging. Do not file these under "dermatology referral in outpatients" — they are urgent physician problems.
DWE high-yield: The paraneoplastic dermatology cluster — dermatomyositis (ovarian, lung, gastric), malignant acanthosis nigricans and tripe palms (gastric), sign of Leser-Trelat (GI adenocarcinoma), Trousseau migratory thrombophlebitis (pancreatic), necrolytic migratory erythema (glucagonoma), Sweet syndrome (acute myeloid leukaemia and myelodysplasia) — is the single most tested family of cutaneous-internal disease associations. [1]
References14ShowHide
- [1]van Beek AP, de Haas ER, van Vloten WA, et al. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review Eur J Endocrinol, 2004.PMID 15538929
- [2]Tolliver S, Graham J, Kaffenberger BH A review of cutaneous manifestations within glucagonoma syndrome: necrolytic migratory erythema Int J Dermatol, 2018.PMID 29450880
- [3]Yin WB, Gao Y, Lu Y Gastric Cancer Associated With Malignant Acanthosis Nigricans Am J Med Sci, 2017.PMID 28317636
- [4]Yerram P, Chaudhary K Calcific uremic arteriolopathy in end stage renal disease: pathophysiology and management Ochsner J, 2014.PMID 25249804
- [5]Westphal SG, et al. Calciphylaxis 2026.PMID 30085562
- [6]Cohen PR Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis Orphanet J Rare Dis, 2007.PMID 17655751
- [7]Cohen PR, Talpaz M, Kurzrock R Malignancy-associated Sweet's syndrome: review of the world literature J Clin Oncol, 1988.PMID 3058878
- [8]Yerolatsite M, Kekkou E, Torounidou N, et al. Cancer-associated dermatomyositis: A scoping review of the literature Autoimmun Rev, 2026.PMID 42409300
- [9]Marckmann P, Skov L, Rossen K, et al. Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging J Am Soc Nephrol, 2006.PMID 16885403
- [10]Bolotin D, Petronic-Rosic V Dermatitis herpetiformis. Part I. Epidemiology, pathogenesis, and clinical presentation J Am Acad Dermatol, 2011.PMID 21571167
- [11]Schwartz RA Sign of Leser-Trélat J Am Acad Dermatol, 1996.PMID 8682971
- [12]Bernett CN, Schmieder GJ. Leser-Trélat Sign 2026.PMID 29261959
- [13]Varki A Trousseau's syndrome: multiple definitions and multiple mechanisms Blood, 2007.PMID 17496204
- [14]Schwartz RA, Nervi SJ Erythema nodosum: a sign of systemic disease Am Fam Physician, 2007.PMID 17375516