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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.87 no.1 Rio de Janeiro Jan./Feb. 2012
WHAT IS YOUR DIAGNOSIS?
Case for diagnosis*
Caso para diagnóstico
Ana Carolina Figueiredo Pereira CherobinI; Felipe Osta de OliveiraI; Isabela Guimarães Ribeiro BaetaI; Everton Carlos Siviero do ValeII
IPhysician and Resident of the Department of Dermatology, Hospital das Clinicas, Federal University of Minas Gerais (HC-UFMG), Belo Horizonte (MG), Brazil
IIMaster's Degree in Dermatology; Assistant Professor, Department of Internal Medicine, Federal University of Minas Gerais (UFMG); Medical Residency Preceptor of the Department of Dermatology, Hospital das Clinicas, Federal University of Minas Gerais (HC-UFMG), Belo Horizonte (MG), Brazil
Dyschromicum erythema perstans, or ashy dermatosis, is a rare chronic acquired skin disease characterized by gray hyperpigmented patches with erythematous borders. Its etiology is unknown and there is no specific treatment for the condition. We report a case of ashy dermatosis in a 41-year-old patient with extensive lesions on the trunk and limbs.
Keywords: Drug eruptions; Hyperpigmentation; Lichenoid eruptions; Melanins
Erythema dyschromicum perstans ou dermatose cinzenta é enfermidade cutânea adquirida, rara, de evolução crônica, caracterizada por máculas acinzentadas, com bordas eritematosas. A etiologia ainda é desconhecida, não havendo tratamento definido para a afecção. Apresenta-se um caso desta dermatose em paciente de 41 anos, com lesões disseminadas no tronco e membros.
Palavras-chave: Erupção por droga; Erupções liquenóides; Hiperpigmentação; Melaninas
Male patient, 41- years-old, born and living in Belo Horizonte (Minas Gerais), noticed nine months previously asymptomatic skin lesions on his face, trunk and limbs. Schizophrenia was a comorbid condition for which the patient has been treated with haloperidol 10mg/day for 15 years. No similar cases reported in patient's family. The dermatological examination revealed extensive and confluent grayish macules with erythematous raised borders affecting the face, neck, trunk and proximal parts of the limbs (Figures 1-3). Laboratory tests were normal. Histopathologic examination showed irregular atrophy of the epidermis and irregular foci of hydropic degeneration of the basal layer, as well as lymphohistiocytic perivascular inflammatory infiltrate and pigment incontinence (Figure 4).
Erythema perstans dyschromicum or ashy dermatosis is a rare chronic, benign disorder belonging to the group of acquired idiopathic hypermelanoses.13 Initially described by Ramirez in 1957 (in El Salvador), the condition tends to be more prevalent in Central and South America,1,2,4 primarily affecting young adults (under 20), with a slight preference for females and darker-skinned individuals. 1-3
The etiology remains elusive, but associations with endocrinopathies, nematode infestations, pesticide exposure, HIV infection, allergy to cobalt and radiological contrast material have been reported. 2,5 The presence of HLA-DR4 *0407 allelic subtype is a risk factor in Mexican mestizos. 3 It is believed that the immune system is involved due to the presence of interleukins and inflammatory mediators in the lesions. 6
The clinical picture is characterized by hyperchromic macules, blue-grayish, oval or polycyclic, with erythematous elevated borders with a diameter measuring from 3 mm to several centimeters. The lesions are asymptomatic, with centrifugal growth. The disorder tends to affect mainly the trunk and upper extremities, sparing the mucous membranes, palms, soles and scalp. 2
Histopathologic findings are nonspecific and include vacuolization of the basal layer, necrosis of basal keratinocytes, colloid bodies, exocytosis of lymphocytes, pigmentary incontinence and perivascular lymphocytic inflammatory infiltrate. 6
The differential diagnosis includes lichen planus pigmentosus, post-inflammatory hyperpigmentation, figurate erythemas, fixed drug eruption, Addison's disease and hemochromatosis. Among these, lichen planus pigmentosus is the most clinically and histologically similar to ashy dermatosis, which has resulted in it being thought to be a variant of lichen planus. At present, however, they are considered separate entities by most authors. 7
Evolution of the disorder is chronic and benign and spontaneous remission is rare in adults. In children clinical improvement can tend to occur within two to three years. 8,9 Many treatments have been proposed but there is no standard therapy and the results are in any case inconsistent. Reports exist of improvement with dapsone and clofazimine, as well as with other regimens such as systemic and topical corticosteroids, antibiotics, griseofulvin, isoniazid, antimalarials, keratolytic agents, phototherapy and psychotherapy. 1,8,10
1. López-Bárcenas A, Contreras-Ruíz J, Carrillo-Correa M, Hojyo-Tomoka MT, Arenas R, Dominguez-Soto L, et al. Dermatosis cenicienta (eritema discrómico perstans). Med Cutan Iber Lat Am. 2005;33:97-102. [ Links ]
2. Torrelo A, Zaballos P, Colmenero I, Mediero IG, Prada I, Zambrano A. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol. 2005;19:422-6. [ Links ]
3. Correa MC, Memije EV, Vargas-Alarcón G, Guzmán RA, Rosetti F, Acuña-Alonzo V, et al. HLA-DR association with the genetic susceptibility to develop ashy dermatosis in Mexican Mestizo patients. J Am Acad Dermatol. 2007;56:17-20. [ Links ]
4. Ramírez CO. Los cenicientos: Problema Clinica. Memoria del Primer Congresso Centroamericano de Dermatologica,. 1957;122-130. [ Links ]
5. Schwartz RA. Erythema dyschromicum perstans: the continuing enigma of Cinderella or ashy dermatosis. Int J Dermatol. 2004;43:230-2. [ Links ]
6. Vasquez-Ochoa LA, Isaza-Guzmán DM, Orozco-Mora B, Restrepo-Molina R, Trujillo-Perez J, Tapia FJ. Immunopathologic study of erythema dyschromicum perstans (ashy dermatosis). Int J Dermatol. 2006;45:937-41. [ Links ]
7. Metin A, Çalka Ö, Ugras S. Lichen planopilaris coexisting with erythema dyschromicum perstans. Br J Dermatol. 2001;145:522-3. [ Links ]
8. Silverberg NB, Herz J, Wagner A, Paller AS. Erythema dyschromicum perstans in prepubertal children. Pediatr Dermatol. 2003;20:398-403. [ Links ]
9. Sardana K, Rajpal M, Garg V, Mishra D. Periorbital hyperpigmentation mimicking fixed drug eruption: a rare presentation of erythema dyschromicum perstans in a paediatric patient. J Eur Acad Dermatol Venereol. 2006;20:1381-3. [ Links ]
10. Bahadir S, Çobanoglu Ü, Çimsit G, Yayli S, Alpay K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol. 2004;43:220-2. [ Links ]
Ana Carolina Figueiredo Pereira Cherobin
Alameda Álvaro Celso, 55 - Santa Efigênia
CEP: 30150-260 Belo Horizonte - MG, Brazil
Received on 18.03.2011.
Approved by the Advisory Board and accepted for publication on 25.04.2011.
Conflict of interest: None
Financial funding: None
* Study conducted at the Hospital das Clinicas, Federal University of Minas Gerais (HC-UFMG), Belo Horizonte (MG), Brazil.