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CASE REPORT |
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Year : 2014 | Volume
: 15
| Issue : 3 | Page : 114-116 |
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Idiopathic eruptive macular pigmentation
Asiya Begum, BV Ramachandra, Ajay Kumar
Department of DVL, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India
Date of Web Publication | 30-Oct-2014 |
Correspondence Address: Asiya Begum 22/2, II Main, Pillanna Garden 1st Stage, Bengaluru - 560 084, Karnataka India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2319-7250.143661
Idiopathic eruptive macular pigmentation (IEMP) is an uncommon, rather under-reported disease entity of the pediatric age characterized by asymptomatic, brownish hyperpigmented macules involving the neck and trunk with no preceding inflammation or exposure to drug. Here we report this case to increase awareness of this entity among dermatologists, dermatopathologists and pediatricians. A 11-year-old healthy boy presented with brownish-gray to dark, discrete, asymptomatic macules on the face, trunk, neck and limbs of insidious onset. Histopathologic examination showed melanophages, mild perivascular lymphohistiocytic infiltrate in the papillary dermis and papillomatosis. The natural course of the disease is spontaneous remission without treatment, which was so even in our patient. IEMP is a benign entity with an excellent prognosis as it exhibits spontaneous resolution. It falls into the differential diagnoses of asymptomatic hyperpigmentatory disorders in pediatric population. Awareness of the entity leads to avoidance of unnecessary aggressive damaging treatment. Keywords: Idiopathic eruptive macular pigmentation, papillomatosis, children
How to cite this article: Begum A, Ramachandra B V, Kumar A. Idiopathic eruptive macular pigmentation. Indian J Paediatr Dermatol 2014;15:114-6 |
Introduction | |  |
Idiopathic eruptive macular pigmentation (IEMP) is a pigmentary disorder of unknown etiology. It was first reported by Degos et al., [1] in 1978. Knowledge and familiarity of this entity is minimal. Less than 30 cases of IEMP have been reported in the literature so far, reflecting unfamiliarity with the entity. [2] We report one such case to highlight its existence and self-resolving course.
Case report | |  |
This is a case report of a 11-year-old healthy boy who presented with asymptomatic brown to dark lesions over the face, neck, trunk and proximal extremities of 3 months' duration. They appeared spontaneously without any preceding lesions or topical therapy. The lesions started insidiously and gradually progressed over a duration of 1 month. There was no history of prior drug intake. The general physical and systemic examination was unremarkable. Cutaneous examination revealed multiple brownish gray to dark, discrete, round to oval macules [Figure 1] and [Figure 2]. The lesions on the lower abdomen had a velvety texture [Figure 3] and [Figure 4]. Palms and soles were spared. The size varied from 0.5 cm to 3 cm. Darier's sign was negative. The mucosae, hair and nails were normal. Hematological investigations were normal. Biopsy showed moderate irregular acanthosis, slight papillomatosis [Figure 5] and [Figure 6] and basal layer hyperpigmentation [Figure 7] and [Figure 8]. The upper dermis showed sparse superficial lymphohistiocytic infiltrate. Few melanophages were seen in the papillary dermis. The mast cell number was normal. The final diagnosis was IEMP. No specific treatment was given. | Figure 1: Generalized hyperpigmented discrete oval macules present on the trunk, neck and upper extremity
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 | Figure 2: Generalized hyperpigmented discrete oval macules present on the back
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 | Figure 3: Close up of the lesion shows the velvety texture of the lesions
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 | Figure 4: Hyperpigmented macules on the forehead, cheeks, neck and upper chest
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 | Figure 5: Histopathology with H and E staining under ×4 shows moderate irregular acanthosis, papillomatosis and sparse superficial lymphohistiocytic infiltrate in the upper dermis
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 | Figure 6: H and E staining under ×10. Shows acanthosis , papillomatosis and sparse superficial lymphohistiocytic infiltrate in the upper dermis with increased basal cell pigmentation
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 | Figure 7: A H and E ×40 view showing basal layer hyperpigmentation. The upper dermis showed sparse superficial lymphohistiocytic infiltrate
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 | Figure 8: H and E staining under ×40 .Shows acanthosis, papillomatosis and basal layer hyperpigmentation with sparse superficial lymphohistiocytic infiltrate in the upper dermis
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An unusual case of a 24-year-old woman lasting 21 years was characterized by several periods of spontaneous resolution followed by recurrences. [3]
This case is reported for its rarity with an objective to increase its awareness among dermatologists, pathologists and pediatricians. A misdiagnosis can lead to unnecessary, sometimes dangerous management of a clinical entity which otherwise regresses spontaneously.
Discussion | |  |
IEMP is an under diagnosed condition. Although it clinically resembles lichen planus pigmentosus, erythema dyschromicum perstans, fixed drug eruption and mastocytosis. Therefore the only way to differentiate is by histopathological examination. The youngest and oldest case reported in the literature is that of a 1-year-old and a 50-year-old. [4],[5] Though most cases have been reported in the young; it has also been reported in a 31-year-old female. [6] A study by Sanz de Galdeano et al.[7] gave the criteria for diagnosis in 1996, namely: (a) Eruption of brownish, non-confluent, asymptomatic macules involving the trunk, neck and proximal extremities in children and adolescents (b) absence of preceding inflammatory lesions (c) no previous drug exposure (d) basal layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible basal layer damage or lichenoid inflammatory infiltrate (e) normal mast cell count. The present case fulfilled all the above mentioned criteria and very few cases of similar nature have been reported [2],[8] among Indians.
The histopathological finding,`pigmented papillomatosis` is characteristic feature which was reported in a series of nine cases from India. [9] This finding was present in the present case as well. IEMP is self-resolving and has been reported to disappear spontaneously in months to years. The largest series of ten cases have been described by Jang et al.[4] An unusual case of a 24-year-old woman lasting 21 years was characterized by several periods of spontaneous resolution followed by recurrences. [3]
This case is reported for its rarity with an objective to increase its awareness among dermatologists, pathologists and pediatricians. A misdiagnosis can lead to unnecessary management of a clinical entity which otherwise regresses spontaneously. Awareness of this entity leads to precise diagnosis and better advice.
References | |  |
1. | Degos R, Civatte J, Belaïch S. Idiopathic eruptive macular pigmentation (author′s transl). Ann Dermatol Venereol 1978;105:177-82.  |
2. | Grover S, Basu A. Idiopathic eruptive macular pigmentation: Report on two cases. Indian J Dermatol 2010;55:277-8.  [PUBMED] |
3. | Mehta S, Aasi S, Cole R, Chu P, Weinberg JM. Idiopathic eruptive macular pigmentation: A case of 21 years′ duration. J Am Acad Dermatol 2003;49:S280-2.  |
4. | Jang KA, Choi JH, Sung KS, Moon KC, Koh JK. Idiopathic eruptive macular pigmentation: Report of 10 cases. J Am Acad Dermatol 2001;44:351-3.  |
5. | Mantovani L, Minghetti S, Ricci M, Zauli S, Marzola A, Corazza M. Idiopathic eruptive macular pigmentation in a 50-year-old man. Eur J Dermatol 2010;20:633-4.  [PUBMED] |
6. | Milobratovic D, Djordjevic S, Vukicevic J, Bogdanovic Z. Idiopathic eruptive macular pigmentation associated with pregnancy and Hashimoto thyroiditis. J Am Acad Dermatol 2005;52:919-21.  [PUBMED] |
7. | Sanz de Galdeano C, Léauté-Labrèze C, Bioulac-Sage P, Nikolic M, Taïeb A. Idiopathic eruptive macular pigmentation: Report of five patients. Pediatr Dermatol 1996;13:274-7.  |
8. | Verma S, Thakur BK. Idiopathic eruptive macular pigmentation with papillomatosis. Indian Dermatol Online J 2011;2:101-3.  [PUBMED] |
9. | Joshi R. Idiopathic eruptive macular pigmentation with papillomatosis: Report of nine cases. Indian J Dermatol Venereol Leprol 2007;73:402-5.  [PUBMED] |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
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