Erythema dyschromicum perstans edp is a rare disorder characterized by asymptomatic, slowly progressive, ashgray macular pigmentation of the skin, which usually occurs from age 5 years through. Clinical syndrome of unknown origin characterized by a progressive bluish to ashgrey hyperpigmentation. It is a distinct and somewhat controversial cutaneous eruption that may be best regarded as a form of lichen planus or lichen planus actinicus and may persist for years. An association with bradykinin has been proposed in the case of hereditary angioedema. Clofazimine is a lipophilic rhimophenazine dye with both antimicrobial and antiinflammatory properties originally developed to treat tuberculosis. Many consider this condition to be a variant of lichen planus. I want to know why i have it and what is the treatment. Erythema dyschromicum perstans edp is a rare cutaneous eruption of unknown etiology that often resolves in prepubertal children but is generally thought to persist in adults. Erythema anulare centrifugum eac, is a descriptive term for a class of skin lesion presenting redness in a ring form anulare that spreads from a center centrifugum. Erythema dyschromicum perstans edp, otherwise known as ashy dermatosis, is a benign and chronic skin disorder characterized by hyperpigmented lesions of various sizes on the trunk, face and extremities. Erythema dyschromicum perstans ashy dermatosis is a distinct and somewhat controversial cutaneous eruption that may be best regarded as a form of lichen planus or lichen planus actinicus. The photos of erythema dyschromicum perstans below are not recommended for people with a weak psyche. The term erythema dyschromicum perstans is credited to marion b. About europe pmc funders joining europe pmc governance roadmap outreach.
Erythema dyschromicum perstans in prepubertal children. Learn the causes, symptoms, differential diagnosis and treatment. Erythema dyschromicum perstans edp is a rare disorder characterized by asymptomatic, slowly progressive, ashgray macular pigmentation of the skin which usually occurs from age 5 through adult life. There is a considerable similarity in histopathological findings between lpp and erythema dyschromicum perstans. Two patients with having characteristic asymptomatic and slowly progressive, slategrey macular lesions with distinct red borders involving the face, neck, upper trunk and limbs were. In adults, erythema dyschromicum perstans is more prevalent in the latin american and asian population.
Erythema dyschromicum perstans in prepubertal children erythema dyschromicum perstans in prepubertal children silverberg, nanette b herz, joshua. Erythema dyschromicum perstansashy dermatosis causes. The rim of erythema that appears during the inflammatory phase of the disorder distinguishes it from other disorders of hyperpigmentation. The world health organisation recommends that clofazimine is continued during pregnancy, as the symptoms of leprosy appear to worsen over this time. Erythema marginatum is a type of erythema redness of the skin or mucous membranes involving pink rings on the torso and inner surfaces of the limbs which come and go for as long as several months. Histologically, the epidermis is atrophic along with vacuolar degeneration of basal cell layer. Erythema dyschromicum perstans can be cosmetically distressing and socially embarrassing, especially if the lesion occurs in a visible area such as the face 1, 21. It is a form of acquired dermal macular hyperpigmentation. Erythema dyschromicum perstans ashy dermatosis huidziekten. Ashy dermatosis erythema dischromicum perstans article pdf available in medicina cutanea iberolatinoamericana 333. Dec 20, 20 history a young adult man presented with an asymptomatic wide spread macular slategrey hyperpigmentation involved mainly the torso of one year duration. Clinical trial with clofazimine for treating erythema dyschromicum perstans.
The aim of this study was to characterize the histopathology and immunopathology of edp. We wish you a cure and never get sick of this disease. Erythema dyschromicum perstans edp, also known as ashy dermatosis and dermatosis cenicienta, is a benign, uncommon skin condition that presents with generalized ashygray colored macules on the body. A 39yearold hispanic man presented with widespread, symmetric, hyperpigmented, browngrayblue, ovalto. Erythema dyschromicum perstans edp is a cosmetically distressing, acquired pigmentary disorder of unknown etiology for which few successful therapies exist.
The dermis exhibits incontinence of pigment with scattered melanophages and a sparse follicular or perivascular infiltrate. These changes were consistent with a diagnosis of erythema dyschromicum perstans edp. Some consider erythema dyschromicum perstans which is also known as ashy dermatosis as a macular variant of lichen planus. Acd az of skin erythema dyschromicum perstans and lichen. It was not associated with any cutaneous or systemic disease. Most of the patients i have diagnosed with erythema dyschromicum perstans are females between ages 20 and 40 but it can occur in men as well. Erythema dyschromicum perstans and lichen planus pigmentosus. We present a case of edp in an adult that cleared significantly after 6 years. Erythema dyschromicum perstans brook e tlougan md, mercedes e gonzalez md, rajni v mandal md, roopal v kundu md, debra l skopicki md dermatology online journal 16 11. In view of their beliefs that this disorder belonged to the erythema perstans group, they proposed the term erythema dyschromicum perstans, as originally suggested by dr. Refer to the related chapter on hyperpigmentation of the face and neck. Erythema dyschromicum perstans ashy dermatitis entered by. We have experienced two cases of edp in children aged 2 and 3, both exceptionally younger than the previously reported cases.
Conditions to consider include other forms of annular erythema erythema perstans a paraneoplastic eruption associated with underlying malignancy in which there are concentric and whirling rings. Herein, we present the successful use of nonablative fractional photothermolysis in combination with topical tacrolimus ointment. Dermatopathology reference describes erythema dyschromicum perstans ashy dermatosis histopathology including histologic features and. Erythema dyschromicum perstans edp is a rare disorder of pigmentation that is most common in hispanic patients. It typically presents as asymptomatic, chronic, slowly progressive ashygray hyperpigmented macules on the trunk and proximal extremities. Proton pump inhibitorinduced erythema dyschromicum perstans. My doctor says i have erythema dyschromicum perstans. Improvement of erythema dyschromicum perstans using a. Our dermatology online original article ashy dermatosis. It affects both males and females, and is seen most commonly before the age of 40. Erythema dyschromicum perstans american osteopathic. Clinically it presents as asymptomatic, ashengrayblue macules of varying sizes, most commonly on the trunk and proximal extremities. This computergenerated list may be inaccurate or incomplete. However, the border can be difficult to discern in darker skinned populations 12.
Therapeutic outcome with clofazimine, dapsone, oral antibiotics, vitamins, isoniazid, choloroquin, gresiofulvin, topical corticosteroids, chemical peels, sun protection, and psychotherapy has been variable and unsatisfactory. Lichen planus is a welldescribed entity of unknown cause and various presentations. Lee, ashy dermatosis erythema dyschromicum perstans induced by omeprazole. Macules in varying shades of grey with a slightly raised erythematous margin occur mainly on the face, trunk, and upper limbs. Later, erythema dyschromicum perstans was called dermatosis ceniciento, meaning ashy dermatosis, because of its ashy bluish gray color. Clofazimine crosses the placenta, and skin discolouration has been seen in neonates. Its etiology is obscure and current treatment modalities remain ineffective. Erythema elevatum diutinum is a rare form of necrotizing vasculitis which affects the skin covering joints fingers, toes, knees, elbows, wrists, ankles and other areas in some cases. Erythema dyschromicum perstans bioinformatics tool laverne is a handy bioinformatics tool to help facilitate scientific exploration of related genes, diseases and pathways based on cocitations. Ashy dermatosis definition of ashy dermatosis by medical. Erythema dyschromicum perstans and lichen planus jama. Erythema dyschromicum perstans in early childhood md. Piqueromartin j, perezalfonzo r, abrusci v, briceno l, gross a, mosca w, et al. Is there is treatment for erythema dyschromicum perstans.
Dec 12, 2006 in 1961, convit et al described five patients with similar findings in venezuela. Erythema dyschromicum perstans edp was first described in 1957, and electron microscopic studies were reported in 1969. Eritema discromico perstans, dermatitis cenicienta. Actinic lichen planus erythema dyschromicum perstans. Erythema dyschromicum perstans american osteopathic college. Erythema dyschromicum perstans and ashy dermatosis the only significant difference between erythema dyschromicum perstans and ashy dermatosis has been the presence of a palpable erythematous border measuring 12 mm in width in the early stages of the former 12,19. Dermis erythema dyschromicum perstans information on the. Some authors include erythema dyschromicum perstans as a variant of lichen planus because of the many clinical and histopathologic similarities between the diseases,z3 we describe a patient with erythema dyschromicum perstans preceded by lichen planus and.
Erythema dyschromicum perstans is a form of acquired dermal macular hyperpigmentation characterised by well circumscribed round to oval or irregular patches on the face, neck and trunk that are grey in colour. The etiology and clinical course in children is poorly defined. They later merge to form large hyperpigmented patches. Abstract erythema dyschromicum perstans edp or ashy dermatosis ad is a pigmentary, asymptomatic disease of unknown etiology that appears in healthy individuals. Erythema dyschromicum perstans, a rare dermatosis of obscure etiopathogenesis and significant cosmetic morbidity, have no satisfactory treatment. Many therapeutic options are available for erythema dyschromicum perstans edp, but few have been effective, except for clofazimine. What is erythema dyschromicum perstans erythema dyschromicum perstans is a form of acquired dermal macular hyperpigmentation characterised by wellcircumscribed round to oval or irregular patches on the face, neck and trunk that are grey in colour. It initially consists of the formation of firm papules andor nodules on the back of the hands which are yellow or pink in color. Cutaneous pigmentation mimicking ashy dermatosis aderythema dyschromicum perstans edp has been described with both omeprazole. Erythema dyschromicum perstans is also called ashy dermatosis of ramirez.
Erythema dyschromicum perstans is also called ashy dermatosis of ramirez, because of. The terms lichen planus pigmentosus lpp, erythema dyschromicum perstans edp and idiopathic eruptive macular pigmentation iemp are names given to pigmentary disorders and often used interchangeably. Erythema dyschromicum perstans ashy dermatosis is een zeldzame verkregen hypermelanose met onbekende etiologie. In south america, another name, erythema chronicum figuratum melanodermicum, also is used. Mar 18, 2020 erythema dyschromicum perstans ashy dermatosis is a distinct and somewhat controversial cutaneous eruption that may be best regarded as a form of lichen planus or lichen planus actinicus. The nosologic identity of edp has been challenged repeatedly as the number of patients with concurrent edplike eruptions and lichenoid disorders has increased. Erythema annulare centrifugum secondary to treatment with finasteride. Ashy dermatosis of ramirez, erythema dyschromicum perstans. Pdf objective erythema dyschromicum perstans edp can be difficult to diagnose and treat.
Herein, we describe five cases and compare light and electron microscopic findings, direct and indirect immunofluorescence, and dopapositive melanocyte counts between normal and affected skin. Dermis erythema dyschromicum perstans information on. History a young adult man presented with an asymptomatic wide spread macular slategrey hyperpigmentation involved mainly the torso of one year duration. Variable components include erythema and papulation. Erythema dyschromicum perstans edp is a cutaneous pigmentary disturbance originally considered to be a variant of erythema perstans. Erythema dyschromicum perstans edp is a rare disorder characterized by asymptomatic, slowly progressive, ashgray macular pigmentation of the skin. A report of three cases, international journal of dermatology, vol. For more information on how to use laverne, please read the how to guide. Druginduced and postinflammatory hyperpigmentation may last for many months after the offending drug or dermatitis has been eliminated. In adults, edp has a slow onset and is unlikely to resolve spontaneously.
Erythema annulare centrifugum, medscape, sep 2014 al hammadi a, asai y, patt ml, et al. Cutaneous mastocytosis must be distinguished from ashy dermatitis or erythema dyschromicum perstans, nodular scabies, neurofibromatosis, juvenile english pro pts in pair. Erythema dyschromicum perstans edp is an acquired, generalized dermal hypermelanosis of unknown etiology. Explore more on erythema dyschromicum perstans below. Erythema dyschromicum perstans also known as ashy dermatosis, and dermatosis cinecienta is an uncommon skin condition with peak age of onset being young adults, but it may also be seen in children or adults of any age. It is a chronic form of erythema multiforme in which the relapses recur so persistently that the eruption is almost permanent. Many different terms have been used to classify these types of lesions and it is still controversial on what exactly defines eac. In one series of 8 patients, 7 had a good or excellent response to clofazimine administered either 100 mg every other day to patients weighing less than 40 kg or 100 mg every day to patients weighing more than 40 kg. The following list of conditions have erythema dyschromicum perstans or similar listed as a symptom in our database. Erythema dyschromicum perstans edp and ashy dermatosis ad are pigmentary disorders of unknown etiology. Automatic update in citescore measures average citations received per document published.
Erythema dyschromicum perstans is a dermal pigmentation seen on the trunk and proximal extremities, most commonly presenting in darkskinned hispanics. Erythema dyschromicum perstans northwestern scholars. Clofazimine may be used in pregnant women with leprosy if the potential benefits justify the risk to the fetus. Erythema dyschromicum perstans is also called ashy. See the analysis of the prevalence of 4 causes of erythema dyschromicum perstans conditions listing medical symptoms.
Erythema dyschromicum perstans is uncommon dermatosis of significant cosmetic morbidity. Jul 31, 20 dermatopathology reference describes erythema dyschromicum perstans ashy dermatosis histopathology including histologic features and provides links to additional medical references. Pigmented skin lesions, erythema dyschromicum perstans, ashy dermatosis. Erythema dyschromicum perstans edp can be difficult to diagnose and treat. Participation is free and the site has a strict confidentiality policy. Erythema dyschromicum perstans edp also known as ashy dermatosis refers to a persistent local redness of the skin. Erythema dyschromicum perstans showing resolution in an adult.
Sulzberger, who suggested it when examining convits 2 patients in caracas. Mar 18, 2020 many therapeutic options are available for erythema dyschromicum perstans edp, but few have been effective, except for clofazimine. Erythema dyschromicum perstans edp is a pigmentary disease of unknown etiology in which damage to basal cells is thought to be mediated by adhesion molecules. The etiology of edp is unknown, but many associations have been described. Apr 30, 2018 erythema dyschromicum perstans a local lesion, a disease of the dermis with unknown cause, a complication without proper treatment.
Sulzbergers comment, in discussion of another paper, is as follows. Aliaga, ashy dermatosis in an hiv antibodypositive patient, acta dermatovenereologica, vol. The only medication which seems to be helpful for edp ash dermatosis is clofazimine. Erythema dyschromicum perstans article about erythema.
Pdf immunopathologic study of erythema dyschromicum. Erythema dyschromicum perstans, lichen planus, lichen planus pigmentosus. Erythema dyschromican perstans is a relatively uncommon form of lichen planus, however i do encounter it rather frequently in both my the woodlands dermatology and conroe dermatology offices. Erythema dyschromicum perstans definition of erythema. What is the differential diagnosis for erythema annulare centrifugum several skin conditions should be considered in the differential diagnosis of erythema annulare centrifugum. Edp is characterized by hyperpigmented macules that are ashgrey in color and may vary in size and shape.
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