Case study - Eczematous rash
A 58-year-old man presented for review following a four-week trial of emollient, topical clobetasone butyrate 0.05% and oral antihistamine for a troublesome eczematous rash on his shins.
The rash, which was initially patch-like, irregular and hyperkeratotic, had advanced to the chest and abdomen with dotted, itchy, red papules and friable pustules.
In view of the non-responsive and atypical progression of the rash, the patient was referred to the rapid access dermatology clinic.
The primary concern was a potential HIV-related skin condition or a paraneoplastic phenomenon.
Skin biopsy revealed features suspicious of necrolytic migratory erythema (NME). Blood tests showed Hb 109g/L, glycogen 348pmol/L (normal: 0-50pmol/L) and high chromagranin A and B (glycoprotein found in neuro-endocrine tissue). He had an elevated oral glucose tolerance test and HbA1c of 52mmol/mol.
CT imaging and an octreotide scan exposed a large heterogenous mass of 10cm in the tail of the pancreas, suggestive of a pancreatic glucagonoma.
During the next eight weeks, the erosive rash continued to spread, affecting the torso, arms, legs and perineum in a painful scattered distribution.
Under a multidisciplinary team of dermatologists, oncologists, endocrinologists and surgeons, the patient was initiated on oral zinc, emollients, topical clioquinol 3%/betamethasone valerate 0.1% and gliclazide 40mg twice daily.
He subsequently had an open partial pancreatectomy and splenectomy and made an excellent postoperative recovery.
The patient was commenced on phenoxymethylpenicillin lifelong post-splenectomy and attended a neuroendocrine tumour centre for follow-up, where he started a course of monthly injectable lanreotide (somatostatin analogue).
Glucagonomas are rare tumours of pancreatic alpha cell origin and one of a series of neuroendocrine tumours that can present to primary care.
These tumours are slow-growing, highly metastatic and clinically silent until their size and hormone-producing capability results in herald symptoms.
Incidence in the UK is estimated at one in 1m per year, with 70-80% of cases metastatic at diagnosis.
The estimated 10-year survival rate is 52% in patients with metastases and 64% in those without.
They typically present in the fifth to sixth decade of life and can be associated with MEN type 1 syndrome (which includes tumours of the parathyroid glands, endocrine gastroenteropancreatic tract and anterior pituitary gland).
Clinical features are due to a hyperglucagonaemic state and include the ‘five Ds’ (dermatitis, diabetes, DVT, depression, diarrhoea) and weight loss. Thromboembolic phenomenon occurs in up to 30% of cases and stomatitis in 30-40% of individuals.
NME is associated with 70% of glucagonoma syndrome cases. It has a predilection for intertriginous and pressurised areas and has been linked to coeliac disease, lung cancer, hepatic cirrhosis and nutritional deficiencies.
Lesions are characterised by migratory, itchy, red papules and plaques that become painful and irregular, with central blistering and crust formation.
Further complication with Candida albicans or Staphylococcus aureus infection can also occur. It is not uncommon to misinterpret NME as chronic eczema or psoriasis, leading to diagnostic delay.
Early surgical resection is the only curative treatment for glucagonomas. Control of blood sugar, oral zinc, amino acid supplementation and somatostatin analogues are important medical treatments.
|Table: Key cutaneous manifestations of internal malignancy|
|Rash type||Description||Common association|
|Necrolytic migratory Erythema||Erythematous, painful, itchy, erosive, blistering rash||Glucagonoma syndrome|
|Acanthosis nigricans||Dark, poorly defined, hyperkeratotic, "velvety" areas at skin folds||Adenocarcinoma GI tract Prostate, breast or ovarian cancer Lymphoma|
|Flushing||Plethora (persistent redness), telangiectasia with associated diarrhoea, wheeze, tachycardia, hypotension and enlarged liver||Carcinoid tumour/td>|
|Erythema gyratum repens||Rapidly migrating, concentric waves of classic erythematous ‘wood grain’ appearance||Lung cancer Transitional cell carcinoma of the kidney|
|Aquired icthyosis||Thick, dry, ‘fish-scale’ skin||Hodgkin lymphoma Sarcoma|
|Paraneoplastic pemphigus||Painful, erosive, mucosal papules and bullae||B-cell lymphoproliferative disorders|
GPs should remain vigilant towards any non-responsive rash associated with systemic upset or routine blood test abnormality.
Awareness of NME is paramount in the detection of pancreatic glucagonomas.
A persistent, erosive, blistering dermatitis, accompanied by hyperglycaemia, weight loss and anaemia, serves as a key diagnostic clue to a life-threatening cause of a rash in primary care.
- Dr Amin is a GP in London
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