Since their initial description, there have been several other small studies and cases adding to our knowledge of this entity. Backgroundrecently, necrolytic acral erythema nae has been described as a. Proposed theories for the cause of necrolytic acral erythema describe alterations in some metabolic factor, many of which are seen in other necrolytic erythemas, including necrolytic migratory erythema, pellagra, essential fatty acid and biotin deficiency, and acrodermatitis enteropathica. Skin biopsy and histopathological study could not be done in this patient. Necrolytic acral erythema can be distinguished from these entities based on. Endocrine tumors of the gastroenteropancreatic system associated with specific skin manifestations are rare. Zincresponsive acral hyperkeratotic dermatosisa novel entity or a subset of some wellknown dermatosis. Initially there is a ringshaped red area that blisters, erodes and crusts over. More than 30 patients have been reported since 1996, all of whom had anti. Low prevalence of necrolytic acral erythema in patients. Necrolytic acral erythema 310 cutis the pathogenesis is unknown, but a relationship to metabolic alterations has been hypothesized.
The histology of the skin in nme is very similar to the biopsy findings in deficiency states like acquired zinc deficiency, essential fatty acid deficiency, biotin deficiency and pellagra, necrolytic acral erythema, a condition strongly associated with hepatitis c presenting with erythematous patches with erosions and blisters with a. Necrolytic acral erythema in the absence of hepatitis c. Necrolytic acral erythema is a rare skin disease associated with hepatitis c virus infection. Necrolytic acral erythema nae is a rare cutaneous sign of hepatitis c virus infection and has recently been linked to zinc deficiency. Necrolytic migratory erythema nme is a rare skin disorder which was first described by becker et al. Since then, most of the cases have reported concomitant hepatitis c virus hcv infection. Thirty necrolytic acral erythema cases were biopsied and detailed histological description was performed by three separate dermatopathologists who were blinded as to clinical presentation.
Necrolytic acral erythema nae is a member of the necrolytic erythemas, which include necrolytic migratory erythema nme, acrodermatitis enteropathica, and various dermopathies secondary to nutritional deficiencies. In this paper, the authors describe a patient with nme and other features of the glucagonoma syndrome. We report a case of a 31yearold woman with hepatitis c virus infection and decreased zinc serum level. Lack of classic histology should not prevent diagnosis of. She recalled the presence of a rash on both feet during this period. Necrolytic acral erythema nae is a rare condition that was first described in seven patients in 1996 in. A deficiency of biotin or essential fatty acids also may mimic nae. Necrolytic acral erythema without hepatitis c infection wu 2009.
Background recently, necrolytic acral erythema nae has been described as a cutaneous marker for hepatitis c virus hcv infection. Necrolytic acral erythema nae is a recently described, poorly. Image correspondence british journal of dermatology. Topical steroids, systemic antibiotics, antifungal agents and descaling measures produced some improvement. Acral hyperkeratosis, hyperkeratosis, necrolytic acral erythema, zinc how to cite this article. Nme is the presenting feature in 70% of patients with pancreatic glucagonoma. Itchy hyperkeratotic acral plaque in a patient with. To date only eight cases have been described in literature, a fact that makes full histological description and appreciation of the disease process inadequate. She had had a history of chronic calcific pancreatitis for which she had undergone rouxeny lateral pancreaticojejunostomy followed by the frey procedure some years later. Causes glucagonoma syndrome necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, angular stomatitis necrolytic migratory erythema may become infected roughly half of cases metastasize, often to liver.
Necrolytic acral erythema masquerading as cellulitis. In the late phase, there is onset of welldelimited plaques with erythema on their outer rim, lichenification, secondary hyperpigmentation and fine desquamation on the surface. Necrolytic acral erythema nae is a recently recognized dermatosis almost exclusively associated with hepatitis c virus hcv infection and closely related to. The clinical and histopathological findings were compatible with nae. Necrolytic migratory erythema nme has been associated with intestinal malabsorption disorders, hepatic cirrhosis, chronic pancreatitis, inflammatory bowel disease, and nonpancreatic malignancies, but may not always be associated with glucagonoma. Necrolytic acral erythema in the absence of hepatitis c virus. Histopathology was similar to necrolytic erythema family which includes necrolytic migratory erythema nme. Optimal therapy appears to be treatment of the underlying hcv infection using a combination of ribavirin and interferon alfa.
Handfoot syndrome is also rarely seen in sicklecell disease. The patient developed a pruritic eruption on his face, trunk, genitals, and extremities, and the eruption had a predilection for bony prominences. Perineum, distal extremities, lower abdomen, and face are the most commonly affected sites. Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. Necrolytic acral erythema nae is a rare condition, first described in 1996. Necrolytic migratory erythema nme is a red, blistering rash that spreads across the skin. Image correspondence bjd british journal of dermatology image gallery. We hereby describe a case of a 30yearold lady who was referred to us with treatment refractory bilaterally symmetrical plaques on the dorsum of hands and feet. Necrolytic acral erythema is a newly described entity characterized by sharply. Necrolytic acral erythema nae is thought to be a reliable diagnostic marker of infection by hepatitis c virus hcv. It presents as welldemarcated erythematous plaques in a sandallike distribution on the dorsal feet with psoriasiform epidermal hyperplasia on histology. A hallmark sign of this syndrome is a rash known as necrolytic migratory erythema nme.
Necrolytic acral erythema is a rare, cutaneous manifestation of hepatitis c virus infection that is characterized by erythematous, violaceous or dusky papules, blisters, andor erosions in the. A 44yearold man with a history of hepatitis c infection presents with thick, dry plaques on his fingers and hands. Necrolytic migratory erythema primary care dermatology. Zincresponsive acral hyperkeratotic dermatosisa novel. The code is valid for the year 2020 for the submission of hipaacovered transactions. Necrolytic acral erythema presents with discoloration at acral sites especially the feetlower legs associated with scale hyperkeratosis, erosions, or even vesicles. When it is obtained, the histopathology associated with nae typically shows psoriasiform, epidermal hyperplasia with a dermal inflammatory infiltrate and necrosis of epidermal cells, however there are no pathognomonic findings. Necrolytic acral erythema also may be confused with other necrolytic erythemas, including necrolytic migratory erythema, acrodermatitis enteropathica, and pellagra.
Histopathology showed diffuse parakeratosis with a neutrophil. Necrolytic acral erythema is a cutaneous condition that is a manifestation of hepatitis c viral infection or zinc deficiency it is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma and nutritional deficiency syndromes. Necrolytic migratory erythema may affect any site but it most often affects the genital and anal region, the buttocks, groin and lower legs. Necrolytic acral erythema nae is a newly described entity, seen in. Necrolytic migratory erythema associated with glucagonoma. Nae is distinct from the other necrolytic erythemas by virtue of its consistent association with hepatitis c hcv together with the acral distribution of its lesions, in. Reviewed by brunilda nazario on september 2, 2019 how is lichen planus linked to hepatitis c.
Necrolytic acral erythema necrolytic acral erythema nae was first described in 1996 by physicians in egypt, m. Erythematous scaly lesions with centrifugal growth characterize the clinical appearance of the disease. Necrolytic acral erythema associated with hepatitis c. An 18yearold woman developed a rash similar to recurrent seborrheic dermatitis, with welldemarcated erythema, pustules, and hair loss over the scalp. Zalewski pdforbes ijgiannakis c physiological role for zinc in prevention of. Necrolytic migratory erythema nme was first described by becker and colleagues in 1942 in a woman with an alphacell tumor of the pancreas with an.
We report a 53yearold female who presented with migratory annular and arcuate ulcers on her limbs. Necrolytic migratory erythema as the first manifestation. Critical inflammatory dermatoses diagnostic histopathology. Necrolytic acral erythema nae is a recently recognized dermatosis almost exclusively associated with hepatitis c virus hcv infection and closely related to a group of necrolytic. They may present with paraneoplastic phenomena known together as the glucagonoma syndrome. These changes can mimic nutritional deficiencies including necro. Necrolytic migratory erythema necrolytic migratory erythema nme is a classic example where recognition of a dermatological condition can lead to the timely diagnosis of a potentially fatal internal malignancy. Oral zinc treatment 45 mcg orally 3 times daily alleviates the severe signs and symptoms, in which cessation of supplementation leads to rapid recurrence. Physical examination revealed scaly, lichenified plaques, welldemarcated with an erythematous peripheral rim located on the lower limbs. The authors considered the case reported by them as an addition to the list of these few cases. It is a cutaneous manifestation of hepatitis c viral infection considered an early cutaneous marker of hepatitis c with onset typically between 35. Necrolytic migratory ulceration pakran j, riyaz n indian. The cause of necrolytic acral erythema is multifactorial with liver dysfunction possibly playing a role in the development of the skin disease. Like nme, ae follows a periorificial and acral distribution, and is commonly associated with diarrhea, angular cheilitis, glossitis, and stomatitis.
Histology revealed hyperkeratosis, parakeratosis, acanthosis and perivascular mononuclear cell infiltrate. Etiology and pathogenesis of necrolytic migratory erythema. Necrolytic migratory erythema is often one of the first presenting symptoms. Arun c inamadar, 1 ragunatha shivanna, 2 balachandra s ankad 3 1 department of dermatology, venereology and leprosy, sri bm patil medical college, blde university, vijayapura 586103, karnataka, india. Chemotherapyinduced acral erythema is reddening, swelling, numbness and desquamation skin sloughing or peeling on palms of the hands and soles of the feet and, occasionally, on the knees, elbows, and elsewhere that can occur after chemotherapy in patients with cancer.
An unusual rash during evaluation for chronic pancreatitis. A woman in her 40s complained of itchy, thick plaque of 5 years duration on her left foot. Necrolytic acral erythema sharp journal of cutaneous pathology. Given the patients positive hepatitis c status and clinicopathological correlation, a diagnosis of necrolytic acral erythema was rendered. How is necrolytic acral erythema linked to hepatitis c. The pathophysiology of necrolytic acral erythema nae is uncertain. Necrolytic acral erythema nae is one of the cutaneous manifestations of hepatitis c virus hcv infection.
Necrolytic migratory erythema is a red, blistering rash that spreads across the skin. Necrolytic acral erythema in seronegative hepatitis c fulltext. It particularly affects the skin around the mouth and distal extremities. Necrolytic migratory erythema nme is, in most cases, associated with a glucagonoma, which is a slow growing malignant tumour predominantly arising in the cells of the pancreas. Hyperkeratotic lesions in a patient with hepatitis c virus. Necrolytic acral erythema without hepatitis c infection. Glucagonoma syndrome is a rare disease that is usually associated with an underlying neuroendocrine tumor. Glucagonoma syndrome is a rare paraneoplastic phenomenon. There is a low prevalence of necrolytic acral erythema in patients given the diagnosis of chronic hepatitis c in the philadelphia area. Necrolytic acral erythema nae is a distinctive skin disorder. Necrolytic migratory erythema has been reported in a heroin smoker 36 a. Most of the cases of necrolytic acral erythema nae are associated with hepatitis c virus infection, and only a few cases have so far been reported without associated hepatitis c infection. Though initial ct scans were normal, follow up scans revealed multiple mass lesions in.
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