Pancreatic diseases and skin K85.90, K86.1, C25.9, D44.9

Diseases of the endocrine pancreas lead to disturbances in the hormone production of:

• glucagon (alpha cells)
• insulin (beta cells) and
• somatostatin (gamma cells).

Dermatologic disorders are mainly diabetes mellitus-associated skin lesions, which are to be expected with prolonged diabetes mellitus, and the rare erythema necroticans migrans induced by elevated glucagon.

Diseases of the exocrine pancreas, e.g. in pancreatitis of different genesis, lead to various functional disorders with an increase in lipase levels, which may present on the body surface as panniculitis.

Pancreatic cancer (C25.9)

• Paraneoplastic syndromes:
  • Thrombophlebitis migrans (I82.1)
  • Acanthosis nigricans maligna (occurrence < 1% of cases).
  • Acromegaly caused by STH-producing ectopic cells e.g. in pancreatic cancer (D44.9).
  • Dermatomyositis (M33.-).
• Skin metastases in pancreatic carcinoma (rare)
  • Sister Josef`s nodule (rare), an umbilical metastasis originating from gastrointestinal adenocarcinomas (gastric, ovarian, pancreatic, etc.)

Genetic syndromes with pancreatic neoplasms:

• Melanoma-pancreatic carcinoma syndrome (clinically BK-Mole syndrome (D48.5)
• Epidermolysis bullosa acquisita in combination with pancreatic cancer (rare combination)
• Peutz-Jeghers syndrome (Q85.9): increased risk of pancreatic cancer, among others
• Tuberous sclerosis (Q85.1): adenomas of the pancreas possible
• Wermer syndrome = MEN type I, multiple endocrine neoplasms - (D 44.8): Insulinomas detectable in 20% of cases with MEN type I. In the presence of an insulinoma, a 4% frequency with MEN type I must be expected.

Acute pancreatitis (K85.90).

• Areal facial erythema
• Cullen-Hellendahl sign: Periumbilical livedo sign in acute pancreatitis.
• Grey-Turner sign: Erythema in the flank area in 1% of patients (considered an unfavorable prognostic sign)

Chronic pancreatitis (K86.1)

• Pancreatic panniculitis (M79.8) in lipase-producing eccrine pancreatic carcinoma or also in acute or chronic (often alcohol-induced) pancreatitis
• Panniculitis with polyarthritis and eosinophilia(eccrine pancreatic carcinoma)
• Autoimmune pancreatitis (K86.1): Necrobiotic xanthogranuloma (D76.3) in 2.5% of cases; pemphigus erythematosus (L10.4) in about 1% of cases
• Cystic fibrosis (E84.9)

• Pancreatitis-panniculitis-polyarthritis syndrome

Adenomas of the pancreas

• Glucagonoma (D13.7): Erythema necroticans migrans (L99.8)

Other:

• Hippel-Lindau syndrome in combination with pancreatic and renal cysts
• Cystic pancreatic fibrosis (E84.8): nonspecific often perioral erythema already in infancy
• Calciphylaxis, cutaneous: associated with calcifications of the pancreatic parenchyma
• Eosinophilic skin lesions associated with pancreatic panniculitis (M78.9)

1. Arbeláez-Cortés A et al (2014) Polyarthritis and pancreatic panniculitis associated with pancreatic carcinoma: review of the literature. J Clin Rheumatol 20:433-436.
2. Kindle SA et al (2015) Dermatologic disorders in 118 patients with autoimmune (immunoglobulin G4-related) pancreatitis: a retrospective cohort analysis. Am J Clin Dermatol. 201 Am J Clin Dermatol 16: 125,130
3. Laureano A et al (2014) Pancreatic panniculitis - a cutaneous manifestation of acute pancreatitis. J Dermatol Case Rep 8:35-37.
4. White NR et al (2014) Retroperitoneal and cutaneous necrotizing fasciitis secondary to necrotizing pancreatitis. J Emerg Med 47:147-149.