PEDIATRIC DERMATOLOGY PHOTOQUIZ Editor: Antonio Torrelo, M.D.
ASYMPTOMATIC HYPERPIGMENTED MACULES Ossama Abbas, M.D. Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon
Case Report A 15-year-old girl presented with 1-year history of asymptomatic dark lesions over the neck, trunk, and proximal extremities. These were not preceded by any redness. She was otherwise healthy and not taking any drug therapy. There was no family history of similar lesions. Physical examination revealed several hyperpigmented nonscaly macules on the neck, trunk, and proximal extremities ranging in size from 0.5 to 2.5 mm in diameter (Figs. 1 and 2). Scratching several lesions did not produce any swelling or erythema. Hair, nails, and mucous membranes were normal. Skin biopsy was performed and the specimen was sent for histopathologic examination (Fig. 3).
What is the diagnosis?
Figure 2. Close-up view of the hyperpigmented macules.
Figure 1. Multiple hyperpigmented nonscaly macules on the back.
© 2015 Wiley Periodicals, Inc.
Figure 3. Increased pigmentation of the basal cell layer and a mild superficial perivascular lymphocytic infiltrate.
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734 Pediatric Dermatology Vol. 32 No. 5 September/October 2015
Diagnosis: Idiopathic eruptive macular hyperpigmentation
Microscopic Findings Histopathologic examination revealed increased pigmentation of the basal cell layer and a mild superficial perivascular lymphocytic infiltrate with scattered melanophages. There was no basal cell vacuolization, and mast cell numbers were within normal range.
Discussion Idiopathic eruptive macular pigmentation (IEMP) is a rare condition that Degos et al (1) first described in 1978. It is characterized by the presence of asymptomatic pigmented macules involving the neck, trunk, and proximal extremities. Age at onset ranges from 1 to 20 years, and the course of the disease lasts from 2 months to 6 years. The lesions persisted for longer than 20 years in only a single reported case (2). Galdeano et al (3) suggested five diagnostic criteria for IEMP: eruption of brownish, nonconfluent macules located on the trunk, neck, and proximal extremities in children and young adults; absence of preceding inflammatory skin disease; no previous medication intake; basal cell layer hyperpigmentation and occasional dermal melanophages without visible damage of the basal layer or lichenoid inflammatory infiltrate; and normal mast cell counts. Our patient met all the above criteria, therefore we diagnosed IEMP. IEMP is of unknown etiology. Although hereditary factors seem to be insignificant, hormonal causes may be implicated because IEMP occurs mostly during childhood and adolescence (4). IEMP has been reported during pregnancy in a woman with a history of thyroid adenoma, autoimmune Hashimoto’s thyroiditis, and alopecia areata, which might indicate a role of hormonal changes and autoimmunity in the pathogenesis of IEMP. On the other hand, sunlight does not seem to be involved since lesions occur mainly on unexposed areas and are not affected by sun exposure (5). The main differential diagnosis is erythema dyschromicum perstans (EDP), an acquired dermatosis that is more prevalent in adults, unlike IEMP, which affects adolescents, of Latin American populations. Although the
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distribution of the hyperpigmented macules may be similar in EPD and IEMP, macules in EDP are grey with an erythematous border and can be confluent, whereas in IEMP the macules are brownish and nonconfluent. Microscopically, vacuolization of the basal layer is seen only in EDP, and lichenoid changes may occasionally be observed (6). Nevertheless, these differences between IEMP and EDP are subtle, and some consider them to be a single entity. Other diseases should be considered in the differential diagnosis, including urticaria pigmentosa, pityriasis versicolor, multiple cafe au lait spots, Gougerot–Carteaud syndrome, and postinflammatory hyperpigmentation. These can be easily differentiated when the appropriate clinicopathologic correlation is undertaken. As for treatment, IEMP resolves gradually and spontaneously over months to years (3,4). Neodymiumdoped yttrium aluminum garnet laser and hydroquinone preparations have been used previously with no major benefit (2). Recognizing this disease is important because it allows us to avoid misdiagnosis and thus needless, nonspecific, unnecessarily expensive and time-consuming therapeutic approaches.
References 1. Degos R, Civatte J, Belaich S. La pigmentation maculeuse eruptive idiopathique. Ann Dermatol Venereol 1978;105:177–182. 2. Mehta S, Aasi S, Cole R et al. Idiopathic eruptive macular pigmentation: a case of 21 years’ duration. J Am Acad Dermatol 2003;49:S280–S282. aute -Labre ze C, Bioula-Sage P et al. 3. Sanz de Galdeano C, Le Idiopathic eruptive macular pigmentation: report of five patients. Pediatr Dermatol 1996;13:274–277. 4. Jang KJ, Choi JH, Sung KJ et al. Idiopathic eruptive macular pigmentation: report of ten cases. J Am Acad Dermatol 2001;44:351–353. 5. Milobratovic D, Djordjevic S, Vukicevic J et al. Idiopathic eruptive macular pigmentation associated with pregnancy and Hashimoto thyroiditis. J Am Acad Dermatol 2005;52:919–920. 6. Torrelo A, Zaballos P, Colmenero I et al. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol 2005;19:422–426.
Address correspondence to Ossama Abbas, M.D., Associate Professor, Department of Dermatology, American University of Beirut Medical Center, P.O. Box 11-0236, Riad El Solh/Beirut 1107 2020, Beirut, Lebanon, or e-mail: [email protected].
© 2015 Wiley Periodicals, Inc.
ASYMPTOMATIC HYPERPIGMENTED MACULES Ossama Abbas, M.D. Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon
Case Report A 15-year-old girl presented with 1-year history of asymptomatic dark lesions over the neck, trunk, and proximal extremities. These were not preceded by any redness. She was otherwise healthy and not taking any drug therapy. There was no family history of similar lesions. Physical examination revealed several hyperpigmented nonscaly macules on the neck, trunk, and proximal extremities ranging in size from 0.5 to 2.5 mm in diameter (Figs. 1 and 2). Scratching several lesions did not produce any swelling or erythema. Hair, nails, and mucous membranes were normal. Skin biopsy was performed and the specimen was sent for histopathologic examination (Fig. 3).
What is the diagnosis?
Figure 2. Close-up view of the hyperpigmented macules.
Figure 1. Multiple hyperpigmented nonscaly macules on the back.
© 2015 Wiley Periodicals, Inc.
Figure 3. Increased pigmentation of the basal cell layer and a mild superficial perivascular lymphocytic infiltrate.
733
734 Pediatric Dermatology Vol. 32 No. 5 September/October 2015
Diagnosis: Idiopathic eruptive macular hyperpigmentation
Microscopic Findings Histopathologic examination revealed increased pigmentation of the basal cell layer and a mild superficial perivascular lymphocytic infiltrate with scattered melanophages. There was no basal cell vacuolization, and mast cell numbers were within normal range.
Discussion Idiopathic eruptive macular pigmentation (IEMP) is a rare condition that Degos et al (1) first described in 1978. It is characterized by the presence of asymptomatic pigmented macules involving the neck, trunk, and proximal extremities. Age at onset ranges from 1 to 20 years, and the course of the disease lasts from 2 months to 6 years. The lesions persisted for longer than 20 years in only a single reported case (2). Galdeano et al (3) suggested five diagnostic criteria for IEMP: eruption of brownish, nonconfluent macules located on the trunk, neck, and proximal extremities in children and young adults; absence of preceding inflammatory skin disease; no previous medication intake; basal cell layer hyperpigmentation and occasional dermal melanophages without visible damage of the basal layer or lichenoid inflammatory infiltrate; and normal mast cell counts. Our patient met all the above criteria, therefore we diagnosed IEMP. IEMP is of unknown etiology. Although hereditary factors seem to be insignificant, hormonal causes may be implicated because IEMP occurs mostly during childhood and adolescence (4). IEMP has been reported during pregnancy in a woman with a history of thyroid adenoma, autoimmune Hashimoto’s thyroiditis, and alopecia areata, which might indicate a role of hormonal changes and autoimmunity in the pathogenesis of IEMP. On the other hand, sunlight does not seem to be involved since lesions occur mainly on unexposed areas and are not affected by sun exposure (5). The main differential diagnosis is erythema dyschromicum perstans (EDP), an acquired dermatosis that is more prevalent in adults, unlike IEMP, which affects adolescents, of Latin American populations. Although the
734
distribution of the hyperpigmented macules may be similar in EPD and IEMP, macules in EDP are grey with an erythematous border and can be confluent, whereas in IEMP the macules are brownish and nonconfluent. Microscopically, vacuolization of the basal layer is seen only in EDP, and lichenoid changes may occasionally be observed (6). Nevertheless, these differences between IEMP and EDP are subtle, and some consider them to be a single entity. Other diseases should be considered in the differential diagnosis, including urticaria pigmentosa, pityriasis versicolor, multiple cafe au lait spots, Gougerot–Carteaud syndrome, and postinflammatory hyperpigmentation. These can be easily differentiated when the appropriate clinicopathologic correlation is undertaken. As for treatment, IEMP resolves gradually and spontaneously over months to years (3,4). Neodymiumdoped yttrium aluminum garnet laser and hydroquinone preparations have been used previously with no major benefit (2). Recognizing this disease is important because it allows us to avoid misdiagnosis and thus needless, nonspecific, unnecessarily expensive and time-consuming therapeutic approaches.
References 1. Degos R, Civatte J, Belaich S. La pigmentation maculeuse eruptive idiopathique. Ann Dermatol Venereol 1978;105:177–182. 2. Mehta S, Aasi S, Cole R et al. Idiopathic eruptive macular pigmentation: a case of 21 years’ duration. J Am Acad Dermatol 2003;49:S280–S282. aute -Labre ze C, Bioula-Sage P et al. 3. Sanz de Galdeano C, Le Idiopathic eruptive macular pigmentation: report of five patients. Pediatr Dermatol 1996;13:274–277. 4. Jang KJ, Choi JH, Sung KJ et al. Idiopathic eruptive macular pigmentation: report of ten cases. J Am Acad Dermatol 2001;44:351–353. 5. Milobratovic D, Djordjevic S, Vukicevic J et al. Idiopathic eruptive macular pigmentation associated with pregnancy and Hashimoto thyroiditis. J Am Acad Dermatol 2005;52:919–920. 6. Torrelo A, Zaballos P, Colmenero I et al. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol 2005;19:422–426.
Address correspondence to Ossama Abbas, M.D., Associate Professor, Department of Dermatology, American University of Beirut Medical Center, P.O. Box 11-0236, Riad El Solh/Beirut 1107 2020, Beirut, Lebanon, or e-mail: [email protected].
© 2015 Wiley Periodicals, Inc.
