Thick hyperkeratosis is sometimes present. Active lesions often include flaccid blisters. Edema may or may not be present. [emedicine.medscape.com]

Characteristic findings on physical examination Clinical appearance depends on the stage of NAE at presentation. [dermatologyadvisor.com]

When comparing our results to review other cases in the literature, clinical presentation of our patient was typical. Our patient also presented with weight loss, diabetes, a high level of serum glucagon, pancreatic tumor, and NME. [dovepress.com]

All seven had the characteristic dermatologic manifestations, present from 1 to 6 years prior to diagnosis. [thedoctorsdoctor.com]

Nunley MD INTRODUCTION A 59-year-old African American male presented with a widespread pruritic skin eruption that had been present for 1 year. [jddonline.com]

• Pallor

  However, the focal nature of epidermal dyskeratosis and pallor, along with basal cell vacuolar degeneration can be useful in distinguishing NAE from other clinical mimickers, such as psoriasis. [dermatologyadvisor.com]

  Chronic lesions demonstrate parakeratosis, irregular acanthosis, papillomatosis, epidermal pallor, and a superficial and deep perivascular inflammatory infiltrate. [idoj.in]

  Inflammatory infiltrate - Pigment incontinence - Fully evolved stage - Psoriasiform epidermal hyperplasia, as follows: - Marked papillomatosis - Parakeratosis - Focal hypergranulosis - Pigment incontinence - Occasional subcorneal pustules - Epidermal pallor [slideshare.net]

  Skin biopsy specimens showed confluent parakeratosis, epidermal pallor, papillary edema, and a lymphohistiocytic infiltrate. Two patients had alcoholic liver disease and one had liver dysfunction as a result of hemochromatosis. [thedoctorsdoctor.com]

  The necrolysis and pallor seen in acrodermatitis enteropathica are absent. DIABETES MELLITUS Cutaneous manifestations are common in diabetics, occurring at some time in approximately 30% of all people who have the disease. [plasticsurgerykey.com]

• Pathologist

  Editor-in-Chief, Journal of Cutaneous Pathology Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists [emedicine.medscape.com]

• Surgical Procedure

  Treatment options for necrotic acral erythema Medical Treatment Surgical Procedures Physical Modalities Treatment of Underlying Hepatitis C with Interferon Alpha and Ribavirin N/A N/A Oral zinc supplementation (440mg/day divided twice daily) Oral amino [dermatologyadvisor.com]

• Hypertension

  Williams [23] reported a case of necrolytic acral erythema in an adolescent boy with a history of infection with hepatitis C virus; he had hepatic fibrosis, hypertension, and thrombocytopenia. [emedicine.medscape.com]

  Williams8 reported a case of necrolytic acral erythema in an adolescent boy with a history of infection with hepatitis C virus; he had hepatic fibrosis, hypertension, and thrombocytopenia. 2 / 13 3. [slideshare.net]

• Liver Fibrosis

  Serum and hepatic zinc is decreased in chronic liver disease patients, and zinc depletion has been suggested to accelerate liver fibrosis. [readbyqxmd.com]

• Necrolytic Migratory Erythema

  Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome. [thedoctorsdoctor.com]

  Figure 1 Necrolytic migratory erythema with erosion and crust formation. Notes: ( A ) Face; ( B ) legs; ( C ) perianal region. Figure 2 Skin biopsy in necrolytic migratory erythema showing a large zone of necrolysis in the upper epidermis (arrow). [dovepress.com]

  Some authors believe that NAE is a distinct entity and others consider it as a variant of necrolytic migratory erythema (NME). METHODS: Five patients with clinical features consistent with NAE were included in this study. [ncbi.nlm.nih.gov]

  "Necrolytic acral erythema: A variant of necrolytic migratory erythema". Int J Dermatol. vol. 44. 2005. pp. 916-21. [dermatologyadvisor.com]

  Necrolytic migratory erythema: clinicopathologic study of 13 cases. Int J Dermatol 2004; 43: 12–18. CrossRef PubMed Google Scholar Technau K, Renkl A, Norgauer J et al. Necrolytic migratory erythema with myelodysplastic syndrome without glucagonoma. [link.springer.com]

• Hyperkeratosis

  Histopathology mostly showed hyperkeratosis, acanthosis, prominent granular layer, spongiosis and dermal infiltrate. After 6 weeks of follow up, all patients showed rapid and remarkable therapeutic response with zinc. [ingentaconnect.com]

  A lesional biopsy revealed hyperkeratosis, psoriasiform acanthosis, spongiosis, normal granular layer and minimal perivascular mononuclear infiltrate [Figure 3]. [ijdvl.com]

  […] incidence approaches 1.7% of patients with HCV. 1-3 Clinically, NAE presents with well-demarcated scaling erythematous papules and plaques with secondary lichenification and later hyperpigmentation. 3 Histologically, NAE is characterized by acanthosis, hyperkeratosis [jddonline.com]

  Palmoplantar filiform parakeratotic hyperkeratosis and digestive adenocarcinoma. Ann Dermatol Venereal 1997; 124:707–709. CAS Google Scholar Mehta RK, Mallet RB, Green C et al. [link.springer.com]

  This patient also had hyperkeratosis of the palms and soles and involvement of the face. [emedicine.medscape.com]

• Burning Sensation

  Skin lesions were associated with burning sensation. Cutaneous examination showed well-demarcated, erythematous to hyperpigmented, hyperkeratotic plaques involving both the dorsa of feet and index fingers [Figure 1]. [idoj.in]

  Patients may report a burning sensation, especially when walking or standing. Sometimes, necrolytic acral erythema lesions are pruritic. The most common area of origination of the rash is the dorsal aspect of the great toe. [emedicine.medscape.com]

  The presence of liver disease appears to correlate with specific mutations. 525 It generally becomes manifest in early childhood with episodes of acute photosensitivity accompanied by a painful, burning sensation. 516 Often there is edema and erythema [plasticsurgerykey.com]

Significant updates on bariatric surgery, Barrett's esophagus, endoscopic ultrasound, endosonography, treatment of liver disease, and much more keep you current on the latest advances. [books.google.de]

Treatment Options Treatment options are summarized in Table I. Table I. [dermatologyadvisor.com]

Treatment of NAE includes supplementation of oral zinc. [idoj.in]

Efficacy of weekly oral doramectin treatment in canine demodicosis. Murayama N, Shibata K, Negata M. Vet Rec 167:63-64, 2010. 3. [cliniciansbrief.com]

Medical Care The optimal treatment for necrolytic acral erythema (NAE) is the optimal treatment of hepatitis C—combination therapy with interferon and ribavirin. [slideshare.net]

Prognosis - Gianotti-Crosti syndrome The rash fades in 2-8 weeks with mild scaling. If hepatitis B is present, the liver takes between 6 months and 4 years to recover fully. Sometimes there is persistent hepatitis and long term viral carriage. [checkorphan.org]

However, NME presents as a hallmark clinical sign of glucagonoma syndrome. 2 Its early recognition allows for early diagnosis of the tumor and leads to a better prognosis. [dovepress.com]

HLA-DRB1, complement factors, and both antismooth muscle and antinuclear antibodies. 4 There have also been data to support a correlation between viral load and specific viral genotypes and NAE. 5 It is unclear if the presence of NAE relates to HCV prognosis [jddonline.com]

Prognosis With therapy, prognosis is fair. Some patients have a relapsing course. Necrolytic acral erythema (NAE) has no known directly associated morbidly or mortality. [emedicine.medscape.com]

Etiology The cause of NAE is unknown, though the metabolic derangements from an HCV-infected liver are believed to play a role. [dermatologyadvisor.com]

[…] fingers, genetics, deficiency, cell differentiation, methods, analysis, pathology, drug therapy, zinc oxide, therapeutic use, schiff bases, metabolism, cytology, transcription factors, biosynthesis, saccharomyces cerevisiae proteins, copper, immunology, etiology [rails-staging2.nubic.northwestern.edu]

Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. Med Gen Med 2004; 6:4. Google Scholar Anhalt GJ, Kim SC, Stanley JR et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. [link.springer.com]

Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed. 2004;6(3):4. 8. Peterson LL, Shaw JC, Acott KM, Mueggler PA, Parker F. [dovepress.com]

Etiology The cause of necrolytic acral erythema (NAE) is uncertain, but a metabolic alteration due to hepatocellular degeneration from hepatitis C infection is proposed. [emedicine.medscape.com]

Global epidemiology of hepatitis C virus infection. Lancet Infect Dis 2005;5:558-67. 14. El-Ghandour TM, Sakr MA, El-Sebai H, El-Gammal TF, El-Sayed MH. Necrolytic acral erythema in Egyptian patients with hepatitis C virus infection. [idoj.in]

Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae. J Am Acad Dermatol 2002; 47:47–52. CrossRef PubMed Google Scholar Schwartz RA. Sign of Leser-Trelat. J Am Acad Dermatol 1996; 35:88–95. [link.springer.com]

Epidemiology Frequency United States Only two cases of necrolytic acral erythema (NAE) have been described in the United States to date, but the condition is likely more common than the case reports suggest. [emedicine.medscape.com]

The pathophysiology of NAE with respect to HCV is unclear. [jddonline.com]

Several theories have been proposed regarding the etiology and pathophysiology of NAE including hypoaminoacidemia, hyperglucagonemia, hypoalbuminemia, and low serum zinc levels. [dermatologyadvisor.com]

It has been speculated that viral load and viral genotype might play a role in NAE.6 Pathophysiology The pathophysiology of this condition is uncertain. [slideshare.net]

They are related to (1) chronic inflammation, (2) immune complex deposition, and (3) immunoproliferative diseases. [16] Pathophysiology The pathophysiology of necrolytic acral erythema (NAE) is uncertain. [emedicine.medscape.com]

[…] fever Causes - Gianotti-Crosti syndrome The specific viruses causing Gianotti-Crosti syndrome include: * Hepatitis B infection * Epstein Barr virus (the cause of glandular fever) * Enterovirus infections * Echo viruses * Respiratory syncytial virus Prevention [checkorphan.org]

The patient should be told to monitor for the stigmata of liver disease in order to prevent its progression. The patient’s skin must be monitored for secondary infection of skin lesions. [dermatologyadvisor.com]

Solar Dermatitis 185 2 Stud Tail (Feline Tail Gland Hyperplasia) 187 2 Superficial Necrolytic Dermatitis (SND, Hepatocutaneous Syndrome, Necrolytic Migratory Erythema) 189 2 Allergy Injections (Desensitization Shots, Allergy Shots) 193 2 Flea Treatment-Prevention [isbn.nu]

Shingles Prevention Study Group. A vaccine to prevent herpes zoster and postherpetic neuralgia in older adults. N Engl J Med. 2005; 352:2271–84 CAS PubMed Google Scholar Ozdemir M, Tüzün Y. Herpes zoster and pruritus. [link.springer.com]

Perhaps, concomitant immunosuppression may prevent development of overt skin manifestations or perhaps zinc deficiency in these patients is never severe enough to produce symptoms. [bioline.org.br]