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Background

Idiopathic Eruptive Macular Pigmentation (IEMP) is a rare disease and is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. The lesions gradually disappeared during a period of several months to years. No treatment is necessary because spontaneous resolution of the lesions can be expected within several months to a few years.

OUTLINE

Epidemiology  
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Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
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CLINICAL VARIANTS CHARACTERIZATION
Idiopathic eruptive macular pigmentation.

Trcko K, Marko PB, Miljkovic J.

Dept Dermatology, General Hospital Maribor, Ljubljanska cesta 5, 2000 Maribor, Slovenija.
Acta Dermatovenerol Alp Panonica Adriat. 2005 Mar;14(1):30-4. Abstract quote  

We present the case of a 10-year-old girl with a six months history of disseminated asymptomatic, brown pigmented macules on the trunk and proximal parts of the extremities.

The clinical picture, histological findings, and the course of disease were similar to those of idiopathic eruptive macular pigmentation. The cutaneous lesions gradually disappeared over the next two years without any treatment, and no relapse occurred.

The knowledge of this disease is important in order to avoid unnecessary treatment as spontaneous resolution of the lesions may be expected within months or a few years. The spontaneous regression without any treatment is an additional diagnostic criterion.
Idiopathic eruptive macular pigmentation: report of 10 cases.

Jang KA, Choi JH, Sung KS, Moon KC, Koh JK.

Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
J Am Acad Dermatol. 2001 Feb;44(2 Suppl):351-3. Abstract quote  

Idiopathic eruptive macular pigmentation (IEMP) is a rare disease. IEMP is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. This study describes 10 cases of idiopathic eruptive macular pigmentation seen during a 9-year period at the Asan Medical Center, Seoul, Korea.

We present these characteristic consecutive cases to provide more insight into the clinical picture and course of IEMP. Skin lesions of 8 patients were multiple brown macules involving the trunk, face, neck, and extremities. In 2 patients, multiple dark brown macules and patches were noted. The age of onset varied from 1 to 20 years.

Tentative diagnoses were usually ashy dermatosis (erythema dyschromicum perstans), fixed drug eruption, or mastocytosis. The history of any erythema and drug medication was absent. Darier's sign was absent. Skin biopsy specimens showed increased pigmentation of the basal layer in an otherwise normal epidermis. Pigmentary incontinence, melanophages, and mild perivascular lymphohistiocytic infiltrate in the papillary dermis were also revealed. Mast cells could not be found. The lesions gradually disappeared during a period of several months to years.

The alleged rarity of IEMP may be partially caused by medical unfamiliarity with this entity, despite its clinical and histopathologic characteristic picture. Treatment of IEMP is unnecessary because spontaneous resolution of the lesions can be expected within several months to a few years.
Idiopathic eruptive macular pigmentation: report of five patients.

Sanz de Galdeano C, Leaute-Labreze C, Bioulac-Sage P, Nikolic M, Taieb A.

Unite de Dermatologie Pediatrique, Hospitalier Pellegrin Enfants, Centre Hospitalier Universitaire de Bordeaux, France.
Pediatr Dermatol. 1996 Jul-Aug;13(4):274-7. Abstract quote  

We report the occurrence of idiopathic eruptive macular pigmentation in four children and one adolescent. This condition appears to be a distinct clinicopathologic and histologic entity.

It is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal limbs. All patients or their families denied the patients' having taken any drug before the eruption. In all of the patients the first sign was a pigmented spot without preceding erythematous, papular, or hypopigmented lesions.

Histologic study showed enhanced epidermal basal layer pigmentation with pigmentary incontinence, a mild perivascular lymphohistiocytic infiltrate, and many melanophages in the papillary dermis. Electron microscopy showed an increased number of melanosomes in basal and suprabasal keratinocytes as well as clustered melanosomes in dermal melanophages.

Treatment of this asymptomatic condition is unnecessary because spontaneous resolution of the lesions is to be expected within several months to a few years.

HISTOLOGICAL TYPES CHARACTERIZATION
General

Increased pigmentation of the basal layer in an otherwise normal epidermis, pigmentary incontinence, and many melanophages in the upper dermis

Mild perivascular, lymphohistiocytic infiltrate in the upper dermis
Mast cells were sparse.

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
Electron microscopy (EM)

An increased number of melanosomes in basal and suprabasal keratinocytes, and clustered melanosomes in dermal melanophages

Shape and number of melanocytes were normal

 

DIFFERENTIAL DIAGNOSIS CHARACTERIZATION
ERYTHEMA DYSCHROMICUM PERSTANS  
Erythema dyschromicum perstans in children: a report of 14 cases.

Torrelo A, Zaballos P, Colmenero I, Mediero IG, de Prada I, Zambrano A.

Department of Pediatric Dermatology, Hospital del Nino Jesus, Menendez Pelayo 65, 28009 Madrid, Spain.
J Eur Acad Dermatol Venereol. 2005 Jul;19(4):422-6. Abstract quote  

BACKGROUND: Erythema dyschromicum perstans (EDP) or ashy dermatosis is a peculiar, slowly progressive, macular hyperpigmentation, which leaves a permanent discoloration. It is an acquired dermatosis that occurs most frequently in Central and South America. EDP usually appears in adults, but some isolated cases and small series have been reported in prepubertal children.

METHODS: A retrospective review of cases of EDP in 10-year-old children or younger, attended in a pediatric hospital between 1990 and 2003.

RESULTS: We present 14 cases of EDC in children 10 years and younger. With an additional 25 cases reported so far in the English language literature, a total of 39 children have been described. Unlike adult patients, who are most commonly of Hispanic origin, children with EDP are usually Caucasian. Other important facts in children with EDP are the absence of consistent trigger factors and an eventual improvement or resolution of the lesions in 50% of prepubertal patients.

CONCLUSION: We suggest that EDP is a distinctive clinical entity, different from lichen planus and lichen planus pigmentosus, which may be identical to the so-called idiopathic eruptive macular pigmentation.

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
PROGNOSIS Spontaneously resolves within months to years
Idiopathic eruptive macular pigmentation: a case of 21 years' duration.

Mehta S, Aasi S, Cole R, Chu P, Weinberg JM.

Department of Dermatology, St. Luke's-Roosevelt Hospital Center and Beth Israel Medical Center, New York, NY 10025, USA.
J Am Acad Dermatol. 2003 Nov;49(5 Suppl):S280-2. Abstract quote  

Idiopathic eruptive macular pigmentation is a rare condition characterized by asymptomatic pigmented macules involving the neck, trunk, and proximal portions of the extremities.

Age at onset usually varies from 1 to 20 years. The lesions usually appear abruptly and remit spontaneously over months to years.

An unusual case of a 24-year-old woman with idiopathic eruptive macular pigmentation lasting 21 years was characterized by several periods of spontaneous resolution followed by recurrences.
TREATMENT Usually none

Pediatr Dermatol 1996;13:274-7.
J Am Acad Dermatol 2001;44:351-3
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated August 10, 2005

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