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Case Report 


Jehad O. Hariri et al, 2020;4(3):759–762.

International Journal of Medicine in Developing Countries

Pseudogluoconoma: a case report

Jehad O. Hariri, Mohammed H. Abduljabbar, Shahad Khalid M. Alshammari*, Manal Jobran N. Alrogi, Asma Abdulwahed H. Alzahrani, Shadah Abdullah A. Babakr

Correspondence to: Shahad Khalid M. Alshammari

*King Abdulaziz University Hospital, Jeddah, Saudi Arabia.

Email: alshammarishahad6 [at] gmail.com

Full list of author information is available at the end of the article.

Received: 06 January 2020 | Accepted: 18 January 2020


ABSTRACT

Background:

Pseudoglucagonoma syndrome is an extremely rare disorder and refers to necrolytic migratory erythema (NME) in the absence of a glucagon-secreting tumor. Delayed diagnosis of glucagonoma might be attributed to the rarity of its incidence, which leads to its progression without treatment.


Case presentation:

We report a case of a 45-year-old male presented with erythematous patches on the palms and soles, surrounded by hyperpigmented scaly borders. These started on the upper and lower extremities, then spread to the genitalia as erythematous crusted pustules and patches with sloughing of the skin and severe tenderness.


Conclusion:

Improvement of these lesions was observed with improved nutrition.


Keywords:

Necrolytic migratory erythema (NME), pseudogluoconoma, nutritional deficiency, glucagonoma syndrome.


Introduction

Glucagonoma syndrome is considered as a paraneoplastic phenomenon found to be associated with necrolytic migratory erythema (NME), which is considered as the most common feature of this syndrome [1]. This represents the most specific manifestation of glucagonoma syndrome and has therefore provided the most valuable indication for diagnosis in the majority of previous cases [2]. Due to the rarity of this disease, rates of mortality remain unclear. Glucagonoma induces various types of manifestations, such as diabetes mellitus, hyperglucagonoma mellitus, anemia, weight loss, glossitis, cheilitis, venous thrombosis, and psychiatric disturbances. NME may be associated with other conditions that are related to pseudoglucagonoma syndrome. Surgery is the most optimal treatment for glucagonoma. Early recognition and detection of NME lead to a more rapid diagnosis of glucagonoma, thereby allowing surgical resection and achieving a promising therapeutic outcome [3]. This study presents a case of pseudoglucagonoma in a male patient with NME.


Case Presentation

A 45-year-old male was presented with erythematous patches on the palms and soles, and surrounded by hyperpigmented scaly borders that started in the upper and lower extremities. Later on, it spread to the genitalia as erythematous crusted pustules and patches with sloughing of the skin and severe tenderness. Finally, a few erythematous pustules appeared perioral a day later. There was a history of cough, fever, loss of appetite, and loss of weight. Tuberculosis was suspected, then excluded. The final diagnosis was melioidosis. His condition worsened and was admitted to the intensive care unit for sepsis. Finally, he was treated and discharged. Two days later, he came with the above-mentioned skin rash, and our dermatology assistance was as consulted to rule out Steven Johnson Syndrome. On general examination, he was underweight and malnourished, and vital signs and other system review were normal. On cutaneous examination, erythematous patches on the palms and soles, surrounded by hyperpigmentation scaly borders, started in the upper and lower extremities, which then spread to the genitalia as erythematous crusted pustules and patches with sloughing of the skin associated with severe tenderness. A few erythematous pustules appeared in a perioral distribution the day after.

Routine investigations showed microcytic anemia, low albumin levels and high random blood sugar (RBS). In renal function test, serum electrolytes were within normal limits. Skin biopsy showed full-thickness epidermal necrosis with underlying focal ballooning of keratinocytes, the diagnosis was consistent with NME. The patient was treated with topical antibiotics (Mupirocin) and high potency topical corticosteroids (Figures 13).

Figure 1. Erythematous patches on the palms and soles, surrounded by hyperpigmented scaly borders. Clinically, NME was suspected and the case was evaluated.

Figure 2. Skin biopsy showed full-thickness epidermal necrosis with underlying focal ballooning of keratinocytes.


Discussion

As pseudo-glucagonoma syndrome is extraordinarily rare. One of the major issues with this syndrome is delayed diagnosis. The time to diagnose this condition might range from three to four years [4]. NME is considered to be the hallmark of glucagonoma syndrome and is characterized by an annular pattern of erythema with centrally formed fragile vesicles, bullae, and crusts [5]. It is present in ~65%–70% of glucagonoma cases at the point of diagnosis [6]. Although a skin biopsy of NME offers limited indications for the pathological diagnosis of glucagonoma, its early recognition may lead to further computerized tomography scanning and establishing a diagnosis [7]. In this work, we report a case of pseudo-glucagonoma syndrome in a patient with malnutrition. It is extremely rare; data on incidence, prevalence, and pathogenesis are not currently established for this syndrome. NME is a distinctive, necrotizing cutaneous eruption is usually associated with glucagonoma. However, it can also occur without a pancreatic glucagonoma, as in chronic hepatic diseases, malabsorption with villous atrophy, chronic pancreatitis, celiac sprue, and hypoproteinemia. The majority of cases that were reported by the literature were NME in pancreatic glucagonoma [8]. There was a case report of NME in patients with liver disease. They found that hepatocellular dysfunction and hypoalbuminemia appeared to be the most common factors associated with NME [9]. NME was also reported in a heroin-dependent patient [10], iatrogenic case [11], the associated with zinc deficiency [12], and secondary to pancreatitis [13]. We found a reported case that showed pseudo-glucagonoma syndrome in a malnourished patient after a large resection of the colon and pancreaticojejunostomy [14]. The present result was compatible with that of Wei et al. [3], who reported that the physical examination of the patient showed skin lesions that were erythematous macules with erosions, and laboratory tests showed elevated blood glucose, anemia, and hypoproteinemia. The skin lesions were partially relieved following treatment with octreotide and intravenous amino acid infusion. The patient gradually recovered following surgery. In the case of malnutrition, total parenteral nutrition with amino acid and caloric supplementation may be used to counteract the catabolic effects of high glucagon and reduce perioperative complications [5]. Routine investigations of the current study showed microcytic anemia, low albumin level, and high RBS. High levels of glucagon serum have appeared as a feature of NME that occurred in the context of glucagonoma. On the other hand, NME has been reported with normal levels of glucagon in celiac sprue and pancreatitis. This might be attributed and explained by enteroglucagon which is a substance that is produced by the crypt cells of the small intestine in the malabsorptive state. Unabsorbed nutrients in the lumen are potent stimulators of enteroglucagon, which can cause NME by an undetermined mechanism [9]. In the beginning, our differential diagnosis was that of acquired acrodermatitis enteropathica, and we excluded it because the patient started to improve before the zinc treatment trail. This was suspected because the patient had an acral and periorificial rash which appeared to be vesiculobullous. Our other differential diagnosis was pellagra, but the lesions were not in a photosensitive distribution. Acrokeratosis paraneoplastica (Bazex syndrome) was also ruled out by a normal endoscopy which showed no esophageal cancer. Finally, our patient’s clinical and pathologic findings lead us to the diagnosis of pseudoglucagonoma syndrome secondary to malnutrition, which became more evident after his skin lesions starting resolving after improved nutrition.

Figure 3. Clinical images showed marked improvement (after 2 weeks) with residual post-inflammatory hyperpigmentation and desquamation.


Conclusion

NME associated with glucagonomas has a good prognosis with rapid resolution of the skin rash following surgical resection of the tumor. Pseudoglucagonoma syndrome has an even better prognosis if the underlying condition is detected and treated early. In our case, the skin biopsy showed full-thickness epidermal necrosis with underlying focal keratinocyte ballooning. The diagnosis was consistent with NME. The patient was treated with topical antibiotics (Mupirocin) and high potency topical corticosteroids. The lesions rapidly regressed with moderately improved nutrition.


List of Abbreviations

NME Necrolytic migratory erythema
RBS Random blood sugar

Conflict of Interest

The authors declare that there is no conflict of interest regarding the publication of this article.


Funding

None.


Consent for Publication

Informed consent was obtained from all the participants.


Ethical Approval

Ethical approval is not required at our institution to publish an anonymous case report.


Author details

Jehad O. Hariri, Mohammed H. Abduljabbar, Shahad Khalid M. Alshammari, Manal Jobran N. Alrogi, Asma Abdulwahed H. Alzahrani, Shadah Abdullah A. Babakr

King Abdulaziz University Hospital, Jeddah, Saudi Arabia


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How to Cite this Article
Pubmed Style

Hariri JO, Abduljabbar MH, Alshammari SKM, Alrogi MJN, Alzahrani AAH, Babakr SAA. Pseudogluoconoma: a case report. IJMDC. 2020; 4(3): 759-762. doi:10.24911/IJMDC.51-1578320583


Web Style

Hariri JO, Abduljabbar MH, Alshammari SKM, Alrogi MJN, Alzahrani AAH, Babakr SAA. Pseudogluoconoma: a case report. http://www.ijmdc.com/?mno=80847 [Access: March 29, 2020]. doi:10.24911/IJMDC.51-1578320583


AMA (American Medical Association) Style

Hariri JO, Abduljabbar MH, Alshammari SKM, Alrogi MJN, Alzahrani AAH, Babakr SAA. Pseudogluoconoma: a case report. IJMDC. 2020; 4(3): 759-762. doi:10.24911/IJMDC.51-1578320583



Vancouver/ICMJE Style

Hariri JO, Abduljabbar MH, Alshammari SKM, Alrogi MJN, Alzahrani AAH, Babakr SAA. Pseudogluoconoma: a case report. IJMDC. (2020), [cited March 29, 2020]; 4(3): 759-762. doi:10.24911/IJMDC.51-1578320583



Harvard Style

Hariri, J. O., Abduljabbar, . M. H., Alshammari, . S. K. M., Alrogi, . M. J. N., Alzahrani, . A. A. H. & Babakr, . S. A. A. (2020) Pseudogluoconoma: a case report. IJMDC, 4 (3), 759-762. doi:10.24911/IJMDC.51-1578320583



Turabian Style

Hariri, Jehad O., Mohammed H. Abduljabbar, Shahad Khalid M. Alshammari, Manal Jobran N. Alrogi, Asma Abdulwahed H. Alzahrani, and Shadah Abdullah A. Babakr. 2020. Pseudogluoconoma: a case report. International Journal of Medicine in Developing Countries, 4 (3), 759-762. doi:10.24911/IJMDC.51-1578320583



Chicago Style

Hariri, Jehad O., Mohammed H. Abduljabbar, Shahad Khalid M. Alshammari, Manal Jobran N. Alrogi, Asma Abdulwahed H. Alzahrani, and Shadah Abdullah A. Babakr. "Pseudogluoconoma: a case report." International Journal of Medicine in Developing Countries 4 (2020), 759-762. doi:10.24911/IJMDC.51-1578320583



MLA (The Modern Language Association) Style

Hariri, Jehad O., Mohammed H. Abduljabbar, Shahad Khalid M. Alshammari, Manal Jobran N. Alrogi, Asma Abdulwahed H. Alzahrani, and Shadah Abdullah A. Babakr. "Pseudogluoconoma: a case report." International Journal of Medicine in Developing Countries 4.3 (2020), 759-762. Print. doi:10.24911/IJMDC.51-1578320583



APA (American Psychological Association) Style

Hariri, J. O., Abduljabbar, . M. H., Alshammari, . S. K. M., Alrogi, . M. J. N., Alzahrani, . A. A. H. & Babakr, . S. A. A. (2020) Pseudogluoconoma: a case report. International Journal of Medicine in Developing Countries, 4 (3), 759-762. doi:10.24911/IJMDC.51-1578320583