Erythema dyschromicum perstans
Published Web Locationhttps://doi.org/10.5070/D39218v6bn
Erythema dyschromicum perstansDepartment of Dermatology, New York University, New York, New York
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): 17
A 39-year-old Hispanic man presented with widespread, symmetric, hyperpigmented, brown-gray-blue, oval-to-circular, discrete and coalescing patches on the upper extremities, trunk, neck and face, many with an associated annular erythematous border. Histopathologic features showed an interface dermatitis, thin lichenoid and superficial perivascular infiltrates, and dermal melanophages. These changes were consistent with a diagnosis of erythema dyschromicum perstans (EDP). The etiology of EDP is unknown, but many associations have been described. The rim of erythema that appears during the inflammatory phase of the disorder distinguishes it from other disorders of hyperpigmentation. There is no consistently effective treatment for EDP. We highlight the use of narrow-band UV B phototherapy in the treatment of this disorder, which has shown good success in our patient thus far.
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A 39-year-old Hispanic man presented to the Dermatology Clinic at the Veterans’ Affairs New York Harbor Healthcare System in May, 2009, for evaluation of discolored areas on the arms that initially were associated with a peripheral red ring that surrounded several of the dark spots. Although the eruption had been present for many years, it would wax and wane spontaneously and leave behind the areas of hyperpigmentation. Over the six weeks prior to presentation, the patient noted that the active lesions had returned and were slowly progressing, especially on his arms, wrists, abdomen, neck, and face. There was minimal pruritus. The patient denied any associated systemic symptoms.
Additionally, he noted that his mother had experienced a similar eruption intermittently throughout her life. There was no personal or family history of autoimmune conditions or thyroid disease. There were no new medications or history of exposure to toxic chemicals. He did not take any medications regularly. There was no history of human immunodeficiency virus infection, herpes simplex virus infection, or other sexually transmitted infections.
Of note, the patient stated that he often saw improvement in the lesions when he would go on a vacation and get a tan. He has shown moderate improvement with the use of ultraviolet B (UVB) phototherapy. After a two-week vacation in the Caribbean, with controlled sun exposure, the eruption had improved and he continues to be maintained on narrow-band UVB phototherapy.
On the proximal and distal aspects of the arms, neck, abdomen, and face, in an essentially symmetric distribution, there were numerous, 5-to-3 cm, slate-gray-to-blue-brown, oval-to-circular, gyrate, discrete, and coalescing patches. Many of the patches were associated with an erythematous, slightly raised, annular border. The lesions had no scale and were non-tender. There were no lesions on the mucous membranes, palms, or soles.
A complete blood count and comprehensive metabolic panel were normal. A human immunodeficiency virus test and rapid plasma reagin test were negative.
There is a sparse, superficial, perivascular, lymphocytic infiltrate with focal vacuolar changes and melanophages within the dermis.
Erythema dyschromicum perstans (EDP) was first described in El Salvador by Ramirez in 1957 and was termed at that time dermatosis cenicienta or ashy dermatosis . The name erythema dyschromicum perstans did not emerge until four years later, in 1961, when Dr. Marion Sulzberger coined the term after describing a patient in Venezuela . Persons of Latin American descent continue to be the most frequently affected by this condition [3, 4].
The etiology of EDP remains unknown. Occasionally, the condition is associated with infections (whipworm, human immunodeficiency virus), the ingestion of toxins (ammonium nitrate, cobalt, radio contrast media, pesticides, fungicides), medications (ethambutol, penicillins, benzodiazepines), and endocrinopathies (thyroid disease) [3, 4, 5]. In the vast majority of patients, an underlying etiology is never found. In Mexican patients, the presence of the HLA-DR4 allele may be associated with EDP .
Classically, patients present with oval-to-circular, gray-to-blue-brown macules and patches, which are symmetrically distributed on the trunk, proximal aspects of the extremities, neck, and face. In the acute phase, the lesions also may possess a thin, erythematous, papular border, which is a unique characteristic of the eruption . It is usually asymptomatic, but mild pruritus may occur . The differential diagnosis of EDP includes ashy dermatosis/dermatitis, lichenoid drug reaction, generalized fixed-drug reaction, lichen planus pigmentosus, idiopathic eruptive macular pigmentation, macular urticaria pigmentosa, and postinflammatory hyperpigmentation [3, 4, 7, 8]. Treponemal eruptions, such as secondary syphilis and pinta, also may resemble EDP; therefore, serologic testing for treponemes is appropriate when encountering this type of outbreak. Some authors differentiate ashy dermatosis from EDP, with the salient feature of EDP being the inflammatory annular border, whereas others believe that EDP and ashy dermatosis represent the same entity [7, 9].
There are no prospective, randomized, controlled studies to guide the treatment of EDP. There is no consistently effective treatment for this condition. Spontaneous resolution has been reported in some patients, especially prepubertal children, but many patients with EDP are affected for many years . Clofazimine, 100 mg daily for three months, has been examined in two small studies, with a response rate of 66 to 87 percent. Side effects include discoloration of the skin, cornea, and body fluids as well as gastrointestinal intolerance in >1 percent of patients [11, 12]. The use of dapsone, 100 mg daily for three months and oral glucocorticoid therapy also have been suggested anecdotally as beneficial therapies [13, 14]. Camouflage creams and make-ups also may be suggested for cosmetic purposes.
Ultraviolet (UV) phototherapy likely helps in the treatment of this eruption in many ways. The photo-immunologic effects of UV phototherapy by the suppression of immune function and reduction in pro-inflammatory cytokines assist by exerting potent anti-inflammatory effects. UV phototherapy provides camouflage, which hides the dermal pigmentation by stimulating pigment production. In addition to hyperpigmentation, UV phototherapy also induces thickening of the stratum corneum and apoptosis of T lymphocytes, which causes a decrease in the lichenoid inflammatory infiltrate that often is observed in active areas of EDP . Narrow-band UVB phototherapy has been used recently in the treatment of other lichenoid pigmentary disorders with success . We conclude that UV light is an excellent and viable treatment option for patients with this difficult-to-treat disorder of pigmentation.
References1. Ramirez CO. Los cenicientos: problema clinica. In: Memoria del Primer Congresso Centroamericano de Dermatologica, December 5-8, 1957, San Salvador, El Salvador 1957: 122
2. Convit J, et al. Erythema dyschromicum perstans: a hitherto undescribed skin disease. J Invest Dermatol 1961; 36: 457
3. Chang MW. Disorders of Hyperpigmentation. In: Bolognia JL, et al eds. Dermatology, 2nd ed. Mosby, Elsevier Limited, 2008: 939
4. Schwartz RA. Erythema dyschromicum perstans: the continuing enigma of Cinderella or ashy dermatosis. Int J Dermatol 2004; 43: 230 [PubMed]
5. Srivastava N, et al. Ashy dermatosis-like pigmentation due to ethambutol. Indian J Dermatol Venereol Leprol 2008; 74; 281 [PubMed]
6. Correa MC, et al. HLA-DR association with the genetic susceptibility to develop ashy dermatosis in Mexican Mestizo patients. J Am Acad Dermatol 2007; 56: 617 [PubMed]
7. Zaynoun S, et al. Ashy dermatoses — a critical review of the literature and a proposed simplified clinical classification. Int J Dermatol 2008; 47: 542 [PubMed]
8. Sanz de Galdeano C, et al. Idiopathic eruptive macular pigmentation: a report of five patients. Pediatr Dermatol 1996; 13: 274 [PubMed]
9. Combemale P, et al. Erythema dyschromicum perstans: report of a new case and critical review of the literature. J Dermatol 1998; 25: 747 [PubMed]
10. Silverberg NB, et al. Erythema dyschromicum perstans in prepubertal children. Pediatr Dermatol 2003; 20: 398 [PubMed]
11. Piquero-Martin J, et al. Clinical trial with clofazimine for treating erythema dyschromicum perstans: evaluation of cell-mediated immunity. Int J Dermatol 1989; 28: 198 [PubMed]
12. Baranda L, et al. Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis): the effect of clofazimine therapy. Arch Dermatol 1997; 133: 325 [PubMed]
13. Kontochristopoulos G, et al. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol 1998; 37: 796 [PubMed]
14. Osswald SS, et al. Erythema dyschromicum perstans: a case report and review. Cutis 2001; 68: 25 [PubMed]
15. Honigsmann H, Schwarz T. Ultraviolet Therapy. In: Bolognia JL, et al eds. Dermatology, 2nd ed. Mosby, Elsevier Limited, 2008: 2053
16. Kocaturk E, et al. Narrowband UVB treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum). Photodermatol Photoimmunol Photomed 2009; 25: 55 [PubMed]
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