Necrolytic acral erythema | |
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Other names | Zinc-responsive necrolytic acral erythema [1] |
Specialty | Dermatology |
Necrolytic acral erythema is a cutaneous condition that is a manifestation of hepatitis C viral infection or zinc deficiency. [2] In the early stages, bullae, erosions, and erythematous or violaceous papules are its defining characteristics. Well-defined plaques with erythema on the outer rim, lichenification, secondary hyperpigmentation, and fine desquamation on the surface begin to appear in the late phase. [3]
Traditionally, necrolytic acral erythema manifests as distinct, dark red hyperkeratotic plaques with a keratotic border that are usually found on the dorsum of the foot and toes. Ankles, legs, and knees may also be affected in some cases. [4] On occasion, lesions may appear on the buttocks, genitalia, hands, and elbows. It has been documented that the nails, palms, and soles, features typically thought of as distinguishing characteristics against necrolytic acral erythema, are affected. [5] Necrolytic acral erythema can show clinically as either acute or chronic. Flaccid blisters, erosions at the margins, and noticeable erythema are the symptoms of acute lesions. The hyperkeratotic surface, moderate erythema, and dark red border are seen in the chronic lesions. Edema may be present in conjunction. [4] The illness may have been present for two to one hundred and sixty months at the time of presentation. [6]
Three stages characterize the evolution of necrolytic acral erythema lesions: early, well-developed, and late. Scaly, erythematous papules or plaques with a distinctively dark or worn center first emerge. When the lesions reach a well-developed stage, they combine to create a thick, hyperpigmented plaque that is clearly defined and has adhering scales. Sometimes there might be pustules. [4] In the later stages, the lesions become thinner, more confined, and more pigmented. [7] Lesions usually show a spontaneous remission and relapse pattern throughout time. [5]
As of yet, the precise etiology of necrolytic acral erythema remains unknown. Numerous causes, including hepatic dysfunction, hypoglucagonemia, hypoalbimunemia, hypoaminoacidemia, zinc deficiency, and diabetes with or without an underlying hepatitis C viral infection, have been postulated as part of the multifactorial pathophysiology of necrolytic acral erythema. [4]
The biopsy site and illness stage are reflected in the histological characteristics. Early lesions from the margin display upper epidermal necrosis that has detached from the surrounding tissue in the form of blisters. Acanthosis, spongiosis, and a superficial dermal infiltration mimicking nummular dermatitis are observed in the epidermis. The well-developed lesions exhibit necrotic keratinocytes, psoriasiform hyperplasia, subcorneal pustules, significant papillomatosis, and parakeratosis. A fissure may occur in the top epidermis as a result of necrotic keratinocytes converging. [4]
The most successful course of treatment to date has been oral zinc therapy. [4]
Both sexes are equally affected by the illness; 46.2% of males and 53.8% of females are affected, respectively. Patients with necrolytic acral erythema fall into the 19–58 year age range, with a mean age of 44±11.3 and a median age of 50. [6]
Bromoderma is a skin condition characterized by an eruption of papules and pustules on the skin. It is caused by hypersensitivity to bromides, such as those found in certain drugs. There is at least one reported case of bromoderma caused by excessive consumption of a soft drink containing brominated vegetable oil.
Necrolytic migratory erythema is a red, blistering rash that spreads across the skin. It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.
Parakeratosis is a mode of keratinization characterized by the retention of nuclei in the stratum corneum. In mucous membranes, parakeratosis is normal. In the skin, this process leads to the abnormal replacement of annular squames with nucleated cells. Parakeratosis is associated with the thinning or loss of the granular layer and is usually seen in diseases of increased cell turnover, whether inflammatory or neoplastic. Parakeratosis is seen in the plaques of psoriasis and in dandruff.
Palisaded neutrophilic and granulomatous dermaititis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows.
Eccrine angiomatous hamartoma (EAH), first described by Lotzbeck in 1859, is a rare benign vascular hamartoma characterized histologically by a proliferation of eccrine and vascular components. EAH exists on a spectrum of cutaneous tumors that include eccrine nevus, mucinous eccrine nevus and EAH. Each diagnostic subtype is characterized by an increase in the number as well as size of mature eccrine glands or ducts, with EAH being distinguished by the added vascular component.
Acrokeratoelastoidosis of Costa or Acrokeratoelastoidosis is a hereditary form of marginal keratoderma, and can be defined as a palmoplantar keratoderma. It is distinguished by tiny, firm pearly or warty papules on the sides of the hands and, occasionally, the feet. It is less common than the hereditary type of marginal keratoderma, keratoelastoidosis marginalis.
Psoriatic erythroderma represents a form of psoriasis that affects all body sites, including the face, hands, feet, nails, trunk, and extremities. This specific form of psoriasis affects 3 percent of persons diagnosed with psoriasis. First-line treatments for psoriatic erythroderma include immunosuppressive medications such as methotrexate, acitretin, or ciclosporin.
Generalized bullous fixed drug eruption (GBFDE) most commonly refers to a drug reaction in the erythema multiforme group. These are uncommon reactions to medications, with an incidence of 0.4 to 1.2 per million person-years for toxic epidermal necrolysis and 1.2 to 6.0 per million person-years for Stevens–Johnson syndrome. The primary skin lesions are large erythemas, most often irregularly distributed and of a characteristic purplish-livid color, at times with flaccid blisters.
Annular erythema of infancy(AEI) consists of self-limited eruptions of erythematous, annular to polycyclic patches and plaques. It is an idiopathic figurate erythema. Over several days, a single lesion disappears without leaving behind any scale or hyperpigmentation. Mostly affecting the trunk, face, and extremities, this rash has no symptoms. The diagnosis of AEI is made through a combination of histopathologic and clinical examinations. The disease first manifests in infancy, and if treatment is not received, the periodic eruptions usually stop after the first year of life.
Neutrophilic dermatosis of the dorsal hands (NDDH) is a skin condition that presents with edematous pustular or ulcerative nodules or plaques localized to the dorsal hands.
Adrenergic urticaria is a skin condition characterized by an eruption consisting of small (1-5mm) red macules and papules with a pale halo, appearing within 10 to 15 min after emotional upset. There have been 10 cases described in medical literature, and involve a trigger followed by a rise in catecholamine and IgE. Treatment involves propranolol and trigger avoidance.
Acral persistent papular mucinosis (APPM) is a rare form of lichen myxedematosus. It is characterized by small papules on the backs of the hands, wrists, and extensor aspects of the distal forearms, with no further clinical or laboratory indications. Lesions tend to persist and may grow in number gradually. Because there are no symptoms, treatment is rarely required.
Self-healing juvenile cutaneous mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, and is characterized by the sudden onset of skin lesions and polyarthritis.
Reticular erythematous mucinosis (REM) is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.
Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. The most common dermatophyte is called Trichophyton rubrum.
Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.
Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.
Congenital erosive and vesicular dermatosis is a cutaneous condition characterized by generalized erosions, vesicles, crusting and ‘scalded skin-like’ erythematous areas affecting up to 75% of the body surface area.
Palpable purpura is characterized by firm, elevated hemorrhagic plaques or papules that can measure several centimeters in diameter. These are typically found on dependent surfaces, like the back of a recumbent patient or the lower legs. The center of a lesion may become ulcerative, pustular, vesicular, necrotic, or nodular. They tend to be asymptomatic, but when nodular or ulcerative, they can become tender. Palpable purpura is the most common cutaneous lesion among individuals with inflammatory vascular injury, whereas nonpalpable purpura typically indicates bleeding caused by a platelet or coagulation disorder.
Cutaneous manifestations of COVID-19 are characteristic signs or symptoms of the Coronavirus disease 2019 that occur in the skin. The American Academy of Dermatology reports that skin lesions such as morbilliform, pernio, urticaria, macular erythema, vesicular purpura, papulosquamous purpura and retiform purpura are seen in people with COVID-19. Pernio-like lesions were more common in mild disease while retiform purpura was seen only in critically ill patients. The major dermatologic patterns identified in individuals with COVID-19 are urticarial rash, confluent erythematous/morbilliform rash, papulovesicular exanthem, chilbain-like acral pattern, livedo reticularis and purpuric "vasculitic" pattern. Chilblains and Multisystem inflammatory syndrome in children are also cutaneous manifestations of COVID-19.
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