Enteroviral meningitis was confirmed, but the skin lesions continued to evolve and the patient was ultimately diagnosed with erythema dyschromicum perstans. Piqueromartin j, perezalfonzo r, abrusci v, briceno l, gross a, mosca w, et al. It typically presents as asymptomatic, chronic, slowly progressive ashygray hyperpigmented macules on. 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. Therapeutic outcome with clofazimine, dapsone, oral antibiotics, vitamins, isoniazid, choloroquin, gresiofulvin, topical corticosteroids, chemical peels, sun protection, and psychotherapy has been variable and unsatisfactory. 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.
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. 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. History a young adult man presented with an asymptomatic wide spread macular slategrey hyperpigmentation involved mainly the torso of one year duration. It can slow down the growth and weakly kill mycobacterium leprae, the bacteria that cause leprosy. Erythema dyschromicum perstans in early childhood md. Erythema dyschromicum perstans is also called ashy dermatosis of ramirez. Jan 24, 2008 my doctor says i have erythema dyschromicum perstans. 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.
Erythema dyschromicum perstans edp or ashy dermatosis ad is a pigmentary, asymptomatic disease of unknown etiology that appears in healthy individuals. Ashy dermatosis definition of ashy dermatosis by medical. Erythema dyschromicum perstans edp is an acquired, generalized dermal hypermelanosis of unknown etiology. Erythema dyschromicum perstans american osteopathic. Erythema dyschromicum perstans edp is a cosmetically distressing, acquired pigmentary disorder of unknown etiology for which few successful therapies exist. 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.
Dec 12, 2006 a 25yearold man presented with a 2year history of developing a slowly progressing symmetrical eruption comprised of multiple hyperpigmented slate graycolored irregular patches predominantly involving the neck, upper torso and the proximal extremities. Dear colleagues, i would like to present this case of erythema dyschromicum perstans to contiue our valuable discussion for the case a lady with a violaceous lesions. Erythema dyschromicum perstans edp is a disorder of pigmentation that is characterized by gray or bluebrown macules or patches in individuals with fitzpatrick skin types iiiv chang et al. I want to know why i have it and what is the treatment.
Ashy dermatosis or erythema dyschromicum perstans edp is a typically asymptomatic disease of unknown origin that causes symmetrical gray spots on the trunk and extremities 1. Key points rare form of necrotizing vasculitis which usually affects the skin over joints and other areas exact cause is unknown, but may be associated with various underlying disorders consists of papules andor nodules which may coalesce into plaques and are red, brown, yellow, or purple. Some consider erythema dyschromicum perstans which is also known as ashy dermatosis as a macular variant of lichen planus. Rooks textbook of dermatology is the most comprehensive work of reference available to the dermatologist. Erythema dyschromicum perstans is an asymptomatic eruption of oval, polycyclic, or irregularly shaped, grayblue hyperpigmented macules on the trunk, the arms, the face, and the neck. Disease bioinformatics research of erythema dyschromicum perstans has been linked to erythema, pigmentation disorders, dermatologic disorders, hyperpigmentation, lichen planus. Erythema dyschromicum perstans in a child following an enteroviral. The list of acronyms and abbreviations related to edp erythema dyschromicum perstans. Aliaga, ashy dermatosis in an hiv antibodypositive patient, acta dermatovenereologica, vol. Improvement of erythema dyschromicum perstans using a. Clinically it presents as asymptomatic, ashengrayblue macules of varying sizes, most commonly on the trunk and proximal extremities. Chlorothalonil, a possible cause of erythema dyschromicum perstans ashy dermatitis. Abstract ashy dermatosis ad, lichen planus pigmentosus lpp, erythema dyschromicum perstans edp, and idiopathic eruptive macular.
Erythema dyschromicum perstans article about erythema. My doctor says i have erythema dyschromicum perstans. Histopathologic features showed an interface dermatitis, thin lichenoid and superficial perivascular infiltrates, and dermal melanophages. A global consensus statement on ashy dermatosis, erythema. A report of three cases, international journal of dermatology, vol. Erythema dyschromicum perstans showing resolution in an adult. Erythema dyschromicum perstans edp is a rare disorder of pigmentation that is most common in hispanic patients. 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 syndrome of unknown origin characterized by a progressive bluish to ashgrey hyperpigmentation. Erythema dyschromicum perstans is uncommon dermatosis of significant cosmetic morbidity. Lichen planus is a welldescribed entity of unknown cause and various presentations. Ashy dermatosis erythema dischromicum perstans article pdf available in medicina cutanea iberolatinoamericana 333. Erythema dyschromicum perstans altmeyers enzyklopadie. Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. Many therapeutic options are available for erythema dyschromicum perstans edp, but few have been effective, except for clofazimine. This computergenerated list may be inaccurate or incomplete. The nosologic identity of edp has been challenged repeatedly as the number of patients with concurrent edplike eruptions and lichenoid disorders has increased. Mar 18, 2020 erythema dyschromicum perstans is an asymptomatic eruption of oval, polycyclic, or irregularly shaped, grayblue hyperpigmented macules on the trunk, the arms, the face, and the neck. It affects both males and females, and is seen most commonly before the age of 40. 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. This patient is a 35 year old saudi lady that has a grayblue ill defined discoloration on neck, face and proximal arms for 7 years.
We wish you a cure and never get sick of this disease. Apr 30, 2018 erythema dyschromicum perstans edp also known as ashy dermatosis refers to a persistent local redness of the skin. It clinically pre sents with oval to round, gray, blue. While agents such as certain medications, radiographic contrast. The name erythema dyschromicum perstans did not emerge until four years later, in 1961, when dr. In adults, edp has a slow onset and is unlikely to resolve. The term erythema dyschromicum perstans is credited to marion b. Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. Refer to the related chapter on hyperpigmentation of the face and neck. Erythema dyschromicum perstans edp is a rare, chronic, pig mentary disorder with unknown etiology 1. 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. See the analysis of the prevalence of 4 causes of erythema dyschromicum perstans conditions listing medical symptoms. 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. Ashy dermatosis and erythema dyschromicum perstans rooks.
Key points rare form of necrotizing vasculitis which usually affects the skin over joints and other areas exact cause is unknown, but may be associated with various underlying disorders. It is a form of acquired dermal macular hyperpigmentation. However, the border can be difficult to discern in darker skinned populations 12. 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 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. Erythema dyschromicum perstans northwestern scholars. Ashy dermatosis in the setting of a positive patch test. In particular, it is used to treat erythema nodosum leprosum, the red lumps and thickened patches often seen in lepromatous leprosy. Edp is characterized by hyperpigmented macules that are ashgrey in color and may vary in size and shape. Erythema dyschromicum perstans, lichen planus pigmentosus, and pigmented contact dermatitis. Jul 31, 20 dermatopathology reference describes erythema dyschromicum perstans ashy dermatosis histopathology including histologic features and provides links to additional medical references.
The etiology of edp is unknown, but many associations have been described. Herein, we present the successful use of nonablative fractional photothermolysis in combination with topical tacrolimus ointment. Erythema dyschromicum perstans is a dermal pigmentation seen on the trunk and proximal extremities, most commonly presenting in darkskinned hispanics. The following list of conditions have erythema dyschromicum perstans or similar listed as a symptom in our database. It is used in combination with rifampicin and dapsone for the treatment of the many forms of leprosy. May 24, 2008 dear colleagues, i would like to present this case of erythema dyschromicum perstans to contiue our valuable discussion for the case a lady with a violaceous lesions. Marion sulzberger coined the term after describing a patient in venezuela 2. The study of erythema dyschromicum perstans has been mentioned in research publications which can be found using our bioinformatics tool. 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. Our dermatology online original article ashy dermatosis. Sulzberger, who suggested it when examining convits 2 patients in caracas. In adults, edp has a slow onset and is unlikely to resolve spontaneously. Erythema dyschromicum perstans ashy dermatosis d e r m l. Coexistence of erythema dyschromicum perstans and vitiligo acta.
Erythema dyschromicum perstans is also called ashy dermatosis of ramirez, because of. Erythema dyschromicum perstans also known as ashy dermatosis, and dermatosis cinecienta is a skin condition with age of onset almost always before 40 years old, characterized by skin lesions that are usually symmetrical and generalized. Dermis erythema dyschromicum perstans information on. 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 dyschromicum perstans in prepubertal children erythema dyschromicum perstans in prepubertal children silverberg, nanette b herz, joshua. These changes were consistent with a diagnosis of erythema dyschromicum perstans edp. Erythema dyschromicum perstans ashy dermatosis d e r m.
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. 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. We present a case of edp in an adult that cleared significantly after 6 years. Many consider this condition to be a variant of lichen planus. In other reports lichen planus actinicus or subtropi cum, and lichen pigmentosus, have been men tioned as variants of lichen planus that closely resemble erythema dyschromicum perstans,z 3.
It begins as ashcolored macules, sometimes with an erythematous or elevated border see the image below. Erythema dyschromicum perstans and lichen planus pigmentosus. It is a chronic form of erythema multiforme in which the relapses recur so persistently that the eruption is almost permanent. The etiology and clinical course in children is poorly defined. Erythema dyschromicum perstans in prepubertal children. Later, erythema dyschromicum perstans was called dermatosis ceniciento, meaning ashy dermatosis, because of its ashy bluish gray color. It was not associated with any cutaneous or systemic disease. Macules in varying shades of grey with a slightly raised erythematous margin occur mainly on the face, trunk, and upper limbs.
Erythema dyschromicum perstans clinical presentation. Variable components include erythema and papulation. Erythema dyschromicum perstans and lichen planus jama. In adults, erythema dyschromicum perstans is more prevalent in the latin american and asian population. Erythema dyschromicum perstans edp also known as ashy dermatosis refers to a persistent local redness of the skin. Mar 18, 2020 many therapeutic options are available for erythema dyschromicum perstans edp, but few have been effective, except for clofazimine. It typically presents as asymptomatic, chronic, slowly progressive ashygray hyperpigmented macules on the trunk and proximal extremities. Dermis erythema dyschromicum perstans information on the. Erythema dyschromicum perstansashy dermatosis causes. Erythema dyschromicum perstans sri lanka journal of child health. Penagos h, jimenez v, fallas v, omalley m, maibach hi. We have experienced two cases of edp in children aged 2 and 3, both exceptionally younger than the previously reported cases. Erythema dyschromicum perstans edp is a cutaneous pigmentary disturbance originally considered to be a variant of erythema perstans. Erythema dyschromicum perstans definition of erythema.
Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. 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. Druginduced and postinflammatory hyperpigmentation may last for many months after the offending drug or dermatitis has been eliminated. Erythema dyschromicum perstans edp, or ashy dermatosis, is a relatively rare condition clinically characterized by slate gray to bluebrown macules often with some degree of initial inflammation. The lesions are usually distributed symmetrically on both sun and nonsunexposed areas including the trunk 69. Sulzbergers comment, in discussion of another paper, is as follows. Erythema dyschromicum perstans edp can be difficult to diagnose and treat. Acd az of skin erythema dyschromicum perstans and lichen. A 39yearold hispanic man presented with widespread, symmetric, hyperpigmented, browngrayblue, ovaltocircular, discrete and.