A 28-year-old Black man presents with a 2-year history of dyspigmentation of his back and chest. The patient notes that the lesions initially had red borders but these have faded over the past several months. Physical examination reveals circular, ashy-gray macules distributed diffusely over his back and chest. The patient denies pain or pruritus and takes nomedication. He notes that he recently traveled to Mexico. He has been relatively unconcerned about the lesions and only sought medical care to rule out cancer.
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Erythema dyschromicum perstans (EDP) was first presented in 1957 by Ramirez,1 who reported on a large number of patients in El Salvador who developed progressive, ash-colored lesions.2,3 EDP is a rare, chronic disorder of pigmentation that manifests as gray or blue-brown macules in individuals with darker white to brown skin types.2,4
The lesions appear suddenly and first present with erythematous and slightly raised margins that fade over several months.3 The lesion can be a single round or oval macule measuring approximately 3 mm in diameter or it can progress to become large confluent patches that affect the majority of the body’s skin surface.3,4 The trunk is the area most frequently affected, but symmetric areas of hyperpigmentation can appear on the extremities, neck, and face.2,4,5 The palms of the hands, soles of the feet, and mucous membranes are usually spared.4 Most patients experience no symptoms associated with EDP; however, a minimal number of patients have experienced pruritus.3,5
The etiology of EDP is unknown. It is postulated that an autoimmune response to dermal CD8-positive T lymphocytes and epidermal keratinocyte intracellular adhesion molecules may damage melanocytes and basal cell keratinocytes. Various genetic risk factors have been studied, and human leukocyte antigen (HLA)-DR mutations have been linked to this condition.2
EDP is generally considered an idiopathic disease, although exposure to certain chemicals (cobalt, ammonium nitrate, and dithiazide iodide contrast for radiographs), medical conditions (hypothyroidism), and infections (HIV, chronic hepatitis, and intestinal parasites) have been associated with the development of EDP.3,4
EDP was first described in Latin Americans and is more prevalent in people of Hispanic descent. The typical age of onset is approximately 30 years, but some cases develop in children.3,5 Affected children are usually white and have a more favorable prognosis than their adult counterparts, with approximately 50% achieving remission.4 Evidence is conflicting regarding sexual bias.3
EDP is classified as a macular pigmentation of uncertain etiology along with ashy dermatosis and lichen planus pigmentosus (LPP).6 Whether or not EDP is a separate diagnosis from LPP has been an topic of controversy; however, a global consensus was reached in 2014 that these are separate conditions.4,6 LPP predominantly involves the head and neck; thus, lesions involving the trunk or extremities are more likely to be EDP. Another key historical feature specific to EDP is the erythematous border associated with its early active phase.6,7 Ashy dermatitis and EDP can be synonymous if there is an unclear history of an erythematous border.6 It is also possible to have multiple disease processes occurring simultaneously. Patients have been reported to have both EDP and LPP. It is also possible to have other autoimmune skin diseases, such as vitiligo, occur concurrently with EDP.4
On histologic examination, LPP and EDP are identical.6 Common findings include epidermal thinning with widening of intracellular spaces, lymphocytic infiltrate in the papillary dermis, presence of dermal melanophages, and vacuolization of basal cells.2,3 Because histology and physical examination cannot be used to distinguish these conditions, history is crucial for determining the correct diagnosis.
Other hyperpigmented etiologies often included in the differential diagnosis are fixed-drug eruption, melanoma, Addison disease, melasma, lentigines, macular amyloidosis, and confluent papillomatosis.5,7 Differentiating these diseases from EDP can be achieved via a combination of history and physical, histologic, serologic, and biopsy findings.
As current therapies for EDP have minimal benefit, it is reasonable to simply offer reassurance that this is a benign condition.3 If a patient wants to pursue treatment, options include topical creams, oral agents, and laser therapy.5 Topical corticosteroids have been used with some success but have been associated with long-term side effects.8 Oral agents such as antibiotics, vitamins, antihistamines, chloroquine, clofazimine, dapsone, and isotretinoin are thought to work because of their anti-inflammatory agents, but they have been shown to have unreliable efficacy.5 Symptoms often recur once medications are stopped, so patients may need life-long treatment, which again comes with potential side effects.5
The patient in this case was given the diagnosis of EDP and educated on the benign nature of the condition. He elected not to undergo any treatment as the lesion did not bother him cosmetically.
Eleanor Johnson, BA, and Yelena Dokic, BSA, are both medical students at Baylor College of Medicine. Christopher Rizk, MD, is a certified dermatologist at Elite Dermatology in Houston, Texas.
- Ramirez CO. Los cenicientos: problema clinico, Presented at: Proceedings of the First Central American Congress of Dermatology; San Salvador, El Salvador; December 5-8, 1957.
- Leung N, Oliveira M, Selim MA, McKinley-Grant L, Lesesky E. Erythema dyschromicum perstans: a case report and systematic review of histologic presentation and treatment. Int J Womens Detmatol. 2018;4(4):216-222.
- Torrelo A, Zaballos P, Colmenero I, Mediero IG, De Prada I, Zambrano A. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol. 2005;19(4):422-426.
- Tamer F. Coexistence of erythema dyschromicum perstans and vitiligo: a case report and review of the literature. Acta Dermatovenerol Alp Pannonica Adriat. 2016;25(4):77-78.
- Wang F, Zhao YK, Wang Z, Liu JH, Luo DQ. Erythema dyschromicum perstans response to isotretinoin. JAMA Dermatol. 2016;152(7):841-842.
- Kumarasinghe SPW, Pandya A, Chandran V, et al. A global consensus statement on ashy dermatosis, erythema dyschromicum perstans, lichen planus pigmentosus, idiopathic eruptive macular pigmentation, and Riehl’s melanosis. Int J Dermatol. 2019;58(3):263-272.
- Chandran V, Kumarasinghe SP. Macular pigmentation of uncertain aetiology revisited: two case reports and a proposed algorithm for clinical classification. Australas J Dermatol. 2017;58(1):45-49.
- Mahajan V, Chauhan P, Mehta K, Sharma A. Erythema dyschromicum perstans: response to topical tacrolimus. Indian J Dermatol. 2015;60(5):525.