Indian Journal of Paediatric Dermatology

CASE REPORT
Year
: 2014  |  Volume : 15  |  Issue : 3  |  Page : 114--116

Idiopathic eruptive macular pigmentation


Asiya Begum, BV Ramachandra, Ajay Kumar 
 Department of DVL, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Correspondence Address:
Asiya Begum
22/2, II Main, Pillanna Garden 1st Stage, Bengaluru - 560 084, Karnataka
India

Abstract

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.



How to cite this article:
Begum A, Ramachandra B V, Kumar A. Idiopathic eruptive macular pigmentation.Indian J Paediatr Dermatol 2014;15:114-116


How to cite this URL:
Begum A, Ramachandra B V, Kumar A. Idiopathic eruptive macular pigmentation. Indian J Paediatr Dermatol [serial online] 2014 [cited 2020 Sep 19 ];15:114-116
Available from: http://www.ijpd.in/text.asp?2014/15/3/114/143661


Full Text

 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}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}{Figure 7}{Figure 8}

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

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