CLINICAL HISTORY:

Signs and symptoms:  The patient experienced a painful, pruritic and progressive rash located in the groin. The rash began on the sides of his scrotum and had steadily progressed to involve the entire scrotum, sides of the penis and upper thighs. He had difficulty ambulating and washing the area due to the intense pain. He denied any dysuria, hematuria or discharge. He could not recall similar outbreaks and denied any constitutional symptoms. He complained of occasional diarrhea.

Previous Treatment:  Lotrisone cream, Mentax cream and OTC Hydrocortisone creams had been applied with little relief.

Other information:  The patient had been living at a psychiatric group home for the past three months. Medications included Remeron, Wellbutrin and Zyprexa. All medications were started approximately three months prior to presentation. In addition, Trazadone had been added to his regimen within the last week. The patient stated he had previously been treated with chemotherapy for a “tumor of the liver” that was now in “remission.”

PHYSICAL EXAM:

Physical exam revealed a 34 year-old cachectic white male with a flat affect. The patient had diffuse erythema with scale on the entire scalp. The scalp hair appeared thinned and brittle. The eyebrows, nasolabial folds and perioral region displayed faint erythematous patches with light scale. The tongue was erythematous and enlarged with lateral imprints of dentition. Bilateral oral commisures displayed erythema and fissuring. No nail changes were noted. Beefy red, slightly erosive patches with surrounding erythema and scale were noted on the scrotum, base of the penis and bilateral groin. The palms were noted to have erythema and scale with increased skin markings. Vesicles or bullae were absent.

LABORATORY TESTS:

Complete blood count revealed a normochromic/normocytic anemia. ALT and alkaline phosphatase were elevated at 74 (nl 7-40 IU/L) and 195 (nl 37-107 U/L), respectively. Intact PTH and serum calcium were normal. Serum zinc was normal at 1014 (nl 600-1200 UG/L). Insulin was elevated at 27.5 (nl 1-19 UU/ML). Gastrin was elevated at 112 (nl 0-90 PG/ML). Glucagon was elevated at 1650 (nl 40-130 NG/L).
CT of the Abdomen revealed calcification of the tail of the pancreas with multiple areas of metastatic lesions in the liver.

DERMATOHISTOPATHOLOGY:

Microscopic description: Histological specimens revealed psoriasiform hyperplasia with a moderate neutrophilic infiltrate. Dyskeratotic cells with cytoplasmic eosinophilic homogenization were noted in the upper epidermis along with marked hydropic swelling. Candida was also present.

DIFFERENTIAL DIAGNOSIS:

1.   Acrodermatitis Enteropathica
2.   Seborrheic Dermatitis
3.   Necrolytic Migratory Erythema
4.   Subcorneal Pustular Dermatosis
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Necrolytic Migratory Erythema

DISCUSSION:

Necrolytic Migratory Erythema (NME) is seen in paraneoplastic syndromes resulting from gastroenteropancreatic tumors. The most common tumor associated with NME is a glucagonoma. It is usually a single, large and slow-growing tumor found in the body or tail of the pancreas. Affecting the islet cells of the pancreas, various peptides (somatostatin, insulin, gastrin) are synthesized and secreted. In each case, the common link is hyperglucagonemia. It may be seen as part of MEN 1 and more than 75 percent have metastasized at the time of diagnosis. Metastases predominate in liver and bone tissue.

Manifestations of glucagonoma syndrome include dermatitis, glossitis, stomatitis, angular cheilitis, anemia and weight loss. There may also be associated psychiatric disturbances. Occasionally, cutaneous lesions are the earliest signs. Malabsorption and diarrhea lead to deficiencies of essential fatty acids, zinc and amino acids. A fasting glucagon level > 1,000 ng/L establishes the diagnosis. The high levels of glucagon are responsible for most of the observed signs and symptoms.

NME is the characteristic skin manifestation of glucagonoma syndrome. It may also be seen in hepatic cirrhosis, a jejunal adenocarcinoma and malabsorption syndromes. It appears in periorificial, flexural and acral areas in addition to the perineum. Lesions may begin as coalescing papulovesicles that erode and crust in circinate patterns. Rapid healing and development of new lesions result in daily fluctuations of the eruption. Post-inflammatory hyperpigmentation may be evident.

TREATMENT:

Actual treatment for this patient: The groin outbreak cleared subsequently with application of a mixture of nystatin, hydrocortisone cream and zinc oxide. Facial and scalp lesions were controlled with topical steroids and antifungals. Because of the extensive visceral involvement, the patient was referred to Hematology/Oncology. The patient began a regimen of alternating streptozocin and adriamycin. Repeat imaging after two cycles revealed stabilization of the pancreatic and liver masses and near normalization of glucagon levels. Psychiatric medications were continued.

Other Treatment options: Surgical debulking may lead to resolution and cure if the syndrome is diagnosed early enough. Supplementation with amino acids and essential fatty acids is helpful in rare cases. Palliation is achieved with chemotherapy, somatostatin infusions and hepatic artery embolization.

REFERENCES:

1. Chastain, MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci 2001 May; 321 (5): 306-320.

2. Fauci, Anthony S. Harrison’s Principles of Internal Medicine. 14th edition. McGraw-Hill, 1998.

3. Freedburg, Irwin M. Fitzpatrick’s Dermatology in General Medicine. 5th edition. McGraw-Hill, 1999.

4. Odom, Richard B. Andrew’s Diseases of the Skin. 9th edition. W.B. Saunders, 2000.

5. Wermers RA. The glucagonoma syndrome. Clinical and pathological features in 21 patients. Medicine 1996 Mar; 75 (2): 53-63.

Additional Comment:

© Copyright 2003-2006 by AOCD Grand Rounds

Top of Page