Indian Journal of Dermatology
: 2013  |  Volume : 58  |  Issue : 2  |  Page : 155--156

Widespread confluent, annular, and circinate patches and plaques in a 56-years-old woman

Mohammad Shahidi-Dadras1, Somayeh Hejazi1, Azin Ayatollahi1, Mohammad Saeedi1, Zahra Asadi-Kani2,  
1 Skin Research Center, Shahid Beheshti University of Medical Sciences, Shohada e Tajrish Hospital, Tehran, Iran
2 Department of Pathology, Danesh laboratory, Tehran, Iran

Correspondence Address:
Mohammad Shahidi-Dadras
Skin Research Center, Shahid Beheshti University of Medical Sciences, Shohada e Tajrish Hospital, Tehran

How to cite this article:
Shahidi-Dadras M, Hejazi S, Ayatollahi A, Saeedi M, Asadi-Kani Z. Widespread confluent, annular, and circinate patches and plaques in a 56-years-old woman.Indian J Dermatol 2013;58:155-156

How to cite this URL:
Shahidi-Dadras M, Hejazi S, Ayatollahi A, Saeedi M, Asadi-Kani Z. Widespread confluent, annular, and circinate patches and plaques in a 56-years-old woman. Indian J Dermatol [serial online] 2013 [cited 2021 Apr 11 ];58:155-156
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A 56-year-old female presented with 1-year history of pruritic, extensive skin eruption. The lesions tend to wax and wane in severity. The onset was from dorsal aspect of her right foot. Extremities, lower abdomen, buttocks, and intertriginous areas then involved. Different topical treatments were used with no significant improvement. She had experienced a weight loss (30 kg in 2 years). She had a 6-year history of normocytic normochromic anemia with unknown etiology. Also, she had an adult-onset diabetes mellitus (DM) since 12 years ago. Her family history was positive for hypertension (HTN) and DM in her first-degree relatives. Physical examination showed an anemic patient with bilateral angular cheilitis, glossitis [Figure 1], and nail changes (nail color change, brittleness, onycholysis, nail striations) [Figure 2]. Widespread scaly, eroded, confluent, annular and circinate patches and plaques, some as large as 15 cm in diameter, with superficial bulla and necrosis were seen [Figure 3]. She had no detectable organomegaly. Occular, oral, and genital mucosa were intact. Routine lab tests revealed mild thrombocytopenia, normocytic normochromic anemia, and hyperglycemia. A skin punch biopsy was performed from an active lesion on her left leg.

The microscopic study revealed a psoriasiform acanthosis, prominent and confluent necrosis of the upper layer of stratum spinosum with neutrophilic exocytosis. Furthermore, mild epidermal hyperplasia, vacuolar change, and deficient granular layer were noted. Perivascular lymphocytic infiltration and scattered extravasated RBCs were in the upper dermis [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}


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Necrolytic migratory erythema (NME) cutaneous manifestation of Glucagonoma syndrome.

Abdominal and pelvic CT scan performed for our patient with the clinical suspicion of glucagonoma syndrome. As expected, there was a mass in the junction of body and tail of the pancreas and it was confirmed by magnetic resonance imaging (MRI). MRI also showed multiple small low-density lesions within the liver. Mild splenomegaly was reported in both CT scan and MRI. Whole body scan with TC99m octerotate was requested. It revealed a focal area of increased activity in the midline of the abdomen, which the body of pancreas was located and multiple small focal activities at least 6 zones in the liver prominently the right hepatic lobe. Endo-ultrasonography showed a mass lesion (mixed echoic 25-29 mm) at the junction of body and tail of the pancreas. The mass adhered to the splenic vein and artery. Pancreatic head and common bile duct were normal. Serum levels of glucagon, zinc, vitamin B12, amino acid, fatty acid, vasoactive intestinal peptide (VIP), and somatostatin were checked. The serum glucagon level was markedly elevated. It was 2,040 ng/l (normal 60-177 ng/l). Serum zinc level was lower than the normal range (57 μg/dl (normal 70-114 μg/dl)). She had mild thrombocytopenia and normocytic normochromic anemia. The serum vitamin B12 level was 620.2 pg/ml (normal 197-866 pg/ml), and the VIP was < 1.5 pmol/l (normal < 17 pmol/l).

The glucagonoma syndrome is a rare paraneoplastic phenomenon caused by an alpha-cell tumor of pancreas with an estimated incidence of 1 in 20 million per year [1] and with a 5-year survival of less than 50%. [2] The incidence of malignancy in patients with glucagonoma at the initial diagnosis has also ranged from 64% to 90%. [3] The diagnosis of glucagonoma syndrome is established by fulfilling the following criteria: (1) radiographically or histologically demonstrable neuroendocrine tumor; (2) an increased circulatory level of glucagon; and (3) characteristic clinical features such as DM, hypoaminoacidemia, and skin eruption. [3],[4] Other clinical manifestations include normochromic and normocytic anemia, glossitis, weight loss, neuropsychiatric manifestation, gastrointestinal disturbances, thromboembolic tendency. [1],[4] Thromboembolic events may account for over 50% of all deaths directly attributed to the glucagonoma syndrome. [3] Only a few cases of glucagonoma syndrome display all of the complete features of syndrome. NME occurs in nearly all glucagonoma patients during the course of the clinical illness. The best known clinical pattern of NME is usually erythematous macules that evolve into circinate and polycyclic plaques with geographical serpiginous pattern. Flaccid vesicles or bullae and crust can superimpose on the plaques. After resolution of the lesions, a peripheral collarette of scale on active margins remains with frequent residual hyperpigmentation. They usually develop in perioral region, perianal, lower abdomen, thighs, buttocks, and distal extremities. However, any part of the body could be affected. [3],[4] We can usually observe lesions in all stages of development in glucagonoma patient as seen in our case. [4] The hallmark laboratory finding in glucagonoma syndrome is elevated fasting serum glucagon, which was determined by radioimmunoassay. [5],[6] The best and the most effective treatment strategy should aim to lower the serum glucagon levels. Although in the absence of metastases, a complete surgical removal of the primary tumor is the first choice of the treatment, the management of metastases is a therapeutic challenge, and a multidisciplinary program including hepatic artery embolization, partial hepatic resection, octerotide therapy, and chemotherapy is suggested. [7],[8] Long-acting somatostatin analogues are useful in the treatment of the metastatic or inoperable tumors. [1],[4] Our patient underwent surgical distal panceratectomy with total tumor resection and splenectomy. Because hepatic metastases were multiple, small, and unresectable, subcutaneous injection of somatostatin 150 mg 2 times daily was administered. As expected, the skin lesion of our patient completely disappeared with some minimal residual hyperpigmentation 2 weeks after the surgery. In addition, her blood glucose level returned to the normal range during the treatment, and the insulin therapy discontinued. She was in the surgery ward for about 1 month. She suddenly had gastrointestinal (GI) bleeding with unknown etiology. The technetium-99m-RBC scan was done to find the source of bleeding, but during the procedure, she had a cardiac arrest. The cardiopulmonary resuscitation (CPR) was unsuccessful, and unfortunately, she has died.

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