Idiopathic eruptive macular pigmentation in a pediatric patient and a review of the literature.

Idiopathic eruptive macular pigmentation (IEMP) is a rare, benign, self-resolving melanosis consisting of hyperpigmented macules typically on the face, trunk, and extremities that can occur in children and adolescents and often presents a diagnostic conundrum. We report a case involving an 8-year-old female whose previous clinical presentation was concerning for an atypical presentation of cutaneous mastocytosis or neurofibromatosis. The clinical and histopathologic evaluation was consistent with the diagnosis of IEMP, and no active intervention was pursued. Our accompanying literature review serves to better characterize this condition, highlight key diagnostic features, and emphasize the tendency for spontaneous resolution to avoid unnecessary treatments with limited clinical efficacy.

Disorders of hyperpigmentation. Part I. Pathogenesis and clinical features of common pigmentary disorders.

Disorders of hyperpigmentation are common and, depending on the extent and location of involvement, can affect the quality of life and pose a significant psychologic burden for patients. Given the similarities in presentation of the various causes of hyperpigmentation, it is often difficult to elucidate the etiology of these conditions, which is important to guide management. Furthermore, certain disorders, such as lichen planus pigmentosus and ashy dermatosis, have similar clinical and/or histologic presentations, and their classification as distinct entities has been debated upon, leading to additional confusion. In this review, the authors selected commonly encountered disorders of hyperpigmentation of the skin, subdivided into epidermal, dermal, or mixed epidermal-dermal disorders based on the location of pigment deposition, along with disorders of hyperpigmentation of the mucosa and nails. Melanocytic nevi, genetic disorders, and systemic causes of hyperpigmentation were largely excluded and considered to be outside the scope of this review. We discussed the pathogenesis of hyperpigmentation as well as the clinical and histologic features of these conditions, along with challenges encountered in their diagnosis and classification. The second article in this 2-part continuing medical education series focuses on the medical and procedural treatments of hyperpigmentation.

Dietary lutein plus zeaxanthin and choline intake is interactively associated with cognitive flexibility in middle-adulthood in adults with overweight and obesity.

BACKGROUND: The xanthophyll carotenoids lutein+zeaxanthin and the dietary component choline have been linked to benefits in cognition. However, knowledge on the interactive influence of these dietary components on cognitive function is sparse. DESIGN: 80 middle-aged adults with overweight and obesity (Body Mass Index: (BMI) ≥25.0 kg/m²), completed 7-day diet records, venous blood draws, heterochromatic flicker photometry, assessment of intelligence quotient (IQ), and a cognitive flexibility task while undergoing electroencephalographic recording for event-related potential (ERP) extraction. Multiplicative interaction terms and hierarchical linear regressions, controlling for age, BMI, sex, annual household income, and IQ were utilized to assess independent and interactive contributions of dietary and biomarker data on Switch task outcomes. RESULTS: Higher intake of lutein+zeaxanthin and choline was associated interactively, but not independently, with faster reaction time (RT), after controlling for pertinent covariates. Dietary intake of lutein+zeaxanthin and choline was associated with serum lutein concentrations, but not with plasma choline metabolites nor macular pigmentation. Plasma phosphatidylcholine (PC) concentrations were associated with higher accuracy in Switch trials, while no other biomarkers were associated with cognitive outcomes. Dietary intake and biomarker data were not related to the N2 nor P3 ERP component. CONCLUSIONS: Among a sample of adults with overweight and obesity, greater intake of choline and lutein+zeaxanthin was associated with faster performance on a cognitive flexibility task. Future work examining methods of increasing consumption of both of these dietary components as a possible means of improving or maintaining cognitive flexibility among adults with overweight and obesity is therefore warranted.

An open-label non-randomized prospective pilot study of the efficacy of Q-switched Nd-YAG laser in management of facial lichen planus pigmentosus.

Lichen planus pigmentosus (LPP) is a cosmetically disfiguring condition. Many treatment options are available which are effective in halting the progression of the disease. The treatment of persistent pigmentation is still a challenge. We describe the use of Q-switched Nd-YAG laser in 13 patients with stable LPP using a standard protocol. Most of our patients showed complete to near-complete resolution of pigment without much complications.

A retrospective clinico-pathological study comparing lichen planus pigmentosus with ashy dermatosis.

BACKGROUND/OBJECTIVES: Controversy persists as to whether lichen planus pigmentosus and ashy dermatosis are separate clinical entities. This study was conducted to examine the clinicopathological features and treatment outcome of the two conditions. METHODS: A retrospective medical chart review of all patients who were diagnosed with lichen planus pigmentosus or ashy dermatosis was conducted. The information collected included the participants' age at onset, site of onset, duration of disease, presence of precipitating factors, distribution of disease, pigmentation and presence of symptoms. In patients from whom a biopsy was taken the histopathological reports were included. RESULTS: Altogether 26 patients with ashy dermatosis and 29 with lichen planus pigmentosus were included in the study. Compared with ashy dermatosis, lichen planus pigmentosus had a more localised distribution with a preponderance for facial involvement, compared with the truncal preponderance in ashy dermatosis. Ashy dermatosis tended to have a more stable clinical course than lichen planus pigmentosus, which was more likely to wax and wane. The utility of histopathology in differentiating between the two conditions is low. CONCLUSION: Ashy dermatosis and lichen planus pigmentosus, as defined in this study, appear to be two separate clinical entities with distinguishable clinical features and natural histories.

Macular pigmentation of uncertain aetiology revisited: two case reports and a proposed algorithm for clinical classification.

Ashy dermatosis, erythema dyschromicum perstans, lichen planus pigmentosus and idiopathic eruptive macular pigmentation are various types of acquired macular hyperpigmentation disorders of the skin described in literature. However, a global consensus on the definitions of these entities is lacking. We report two cases of acquired macular (hyper)pigmentation of uncertain aetiology diagnosed as ashy dermatosis and attempt to clarify the various confusing nosologies based on existing literature. We infer that acquired small and large macular pigmentation of uncertain aetiology should be considered separate from that associated with lichen planus. We also propose a diagnostic algorithm for patients with acquired macular hyperpigmentation.

Idiopathic eruptive macular pigmentation in a child with citrin deficiency.

Idiopathic eruptive macular pigmentation (IEMP) is a rare dermatological disorder with generally unclear etiology and pathogenesis. A 5½-year-old girl was referred to hospital with a 10 month history of brown skin rashes. In early infancy, citrin deficiency had been diagnosed with the SLC25A13 genotype c.851_854del4/c.998G > A, but all clinical and laboratory abnormalities recovered following the introduction of a lactose-free and medium-chain triglyceride-enriched formula. Physical examination at referral indicated symmetric, multiple and non-scaly brown macules on the neck, trunk, buttocks and proximal parts of the extremities. Histopathology indicated epidermal basal layer hyperpigmentation with an irregular distribution, along with a large number of melanophages in the upper dermis. The diagnosis of IEMP was thus made. Within 2 years of follow up, the rashes disappeared spontaneously and gradually. To our knowledge, this is the first description of IEMP in a patient with silent citrin deficiency.

Idiopathic Eruptive Macular Pigmentation with Papillomatosis: A Common but Underdiagnosed Entity.

Idiopathic Eruptive Macular Pigmentation (IEMP) is an uncommon and possibly underdiagnosed dermatosis. It manifests as asymptomatic pigmented macules over the face, trunk and proximal extremities among children and adolescents. Degos et al. first documented this condition in 1978, unveiling its distinct characteristics. The macules, initially dark brown-black, naturally diminish over several months to years, with no lasting pigmentation or scarring. In this report, we highlight the case of a 14-year-old girl displaying all the distinctive traits of IEMP.

Histological Evaluation of Acquired Dermal Macular Hyperpigmentation.

CONTEXT: An umbrella term, acquired dermal macular hyperpigmentation (ADMH), has been proposed to denote conditions including ashy dermatosis, erythema dyschromicum perstans, lichen planus pigmentosus, and idiopathic macular eruptive pigmentation. AIMS: To classify the patients manifesting ADMH on the basis of histology. SETTINGS AND DESIGN: In this retrospective, cross-sectional study, histology specimens of patients of ADMH, who underwent skin biopsy in our institution from 1.1 2015 to 31.12.2017, were included after obtaining ethical clearance. MATERIALS AND METHODS: The histology specimens of patients of ADMH were reviewed by the pathologist and classified. Clinical features of individual patient were collected from previous records and the data analyzed. STATISTICAL ANALYSIS USED: Pearson's Chi-square test was used to determine significance of association between age of onset and duration of pigmentation with histology type. RESULTS: Three patterns of histology were identified in the study group (17 males and 13 females). Type 1: Basal cell degeneration and moderate to dense inflammation (12 patients, 40%), type 2: Significant pigment incontinence and sparse inflammation without basal cell degeneration, (12 patients, 40%), and type 3: sparse inflammation without basal cell degeneration or significant pigment incontinence (six patients, 20%). Statistically significant association was noted between age of onset of pigmentation and histology type (P value, 0.02). LIMITATIONS: Main limitation was the small sample size. CONCLUSIONS: Prospective studies evaluating the clinical progression and dermoscopy features and analyzing serial biopsies of ADMH patients may confirm whether the histology patterns observed represent different stages of same disease process or are different entities.

Idiopathic Eruptive Macular Pigmentation - Uncommon Presentation of an Uncommon Condition.

Idiopathic eruptive macular pigmentation (IEMP) is an asymptomatic condition with dark brown, nonconfluent lesions chiefly occurring in children and adolescents. The usual sites involved are face, trunk, extremities, and the lesions resolve over few months to years. We report an unusual presentation of this uncommon condition. A 22-year-old male presented with multiple dark-colored asymptomatic lesions on the scalp and forehead for the past 3 years. There was no history of preceding symptoms, drug, or applications, past or family history of such lesions. Examination revealed multiple hyperpigmented macules and barely elevated nonscaly plaques with a velvety feel akin to acanthosis nigricans (AN). Dermoscopy was similar to AN. Blood sugar, thyroid, and lipid profiles were normal. Histology showed "pigmented papillomatosis" concluding the final diagnosis of IEMP. Confluence of lesions is a rarely described phenomenon, with the scalp being a hitherto unreported site. The velvety feel of lesions, the dermoscopic, and histopathological findings further substantiate the hypothesis of this entity being an eruptive variant of AN.

Idiopathic eruptive macular pigmentation in a Chinese child.

We present a case of a healthy 8-year-old boy who presented with disseminated asymptomatic brown macules on the face, neck, trunk, and proximal extremities for 3 months. Dermatologic examination revealed multiple, smooth, nonscaly, brown macules involving the face, neck, trunk, and proximal limbs. The Darier's sign was negative. Histopathologic study showed normal epidermis and basal membrane, and increasingly scattered melanophages in the papillary dermis. The final diagnosis was idiopathic eruptive macular pigmentation.

Idiopathic eruptive macular pigmentation with papillomatosis.

We present a case of an otherwise healthy 10-year-old girl who presented with asymptomatic brown macules over the trunk and proximal extremities, of three months' duration. The cutaneous examination revealed multiple, dark brown, discrete, round to oval macules and a few mildly elevated lesions over the trunk and proximal limbs. The individual lesion was 1-3 cm in diameter and a few showed velvety appearance over the surface. Darier's sign was negative. The histopathological study from the velvety lesion showed acanthosis, papillomatosis and increased melanin in the basal layer. The upper dermis showed sparse perivascular infiltrate of lymphocytes without any dermal melanophages. It fulfilled the criteria for idiopathic eruptive macular pigmentation with additional histological finding of papillomatosis.

Dermatosis cenicienta: revisión y diagnóstico diferencial con otros trastornos pigmentarios idiopáticos / Ashy dermatosis: revision and differential diagnosis with other idiopathic pigmentary disorders

La dermatosis cenicienta es un trastorno pigmentario infrecuente que se presenta en individuos de piel morena, especialmente en centroamericanos. Las lesiones consisten en manchas hiperpigmentadas de coloración gris-azulada, principalmente localizadas en la cara, tronco y brazos. Su principal diagnóstico diferencial debe ser planteado con tres dermatosis pigmentarias de origen idiopático: eritema discrómico perstans, pigmentación macular eruptiva idiopática y liquen plano pigmentoso. Hay consenso en considerar al eritema discrómico perstans como la misma enfermedad, pero con un borde eritematoso transitorio. La pigmentación macular eruptiva idiopática es clínicamente similar, pero sin una dermatitis de interfase al estudio histopatológico, y sólo muestra una pigmentación de la capa basal, incontinencia pigmentaria y melanófagos en la dermis. Finalmente, el liquen plano pigmentoso debe ser considerado como una variedad pigmentada de liquen plano.

Ashy dermatosis is an infrequent disorder of pigmentation, which predominantly occurs in darkly pigmented individuals, specially in central americans. The lesions consist of bluish-gray hyperpigmented patches mainly located on the face, trunk and arms. Its main differential diagnosis must be raised with three idiopathic pigmentary disorders: erythema dyschromicum perstans, idiopathic eruptive macular pigmentation and lichen planus pigmentosus. It has been widely accepted that erythema dischromicum perstans is the same disease, but with a transient erythematous border. Idiopathic eruptive macular pigmentation is clinically similar but without an interfase dermatitis at histopathologic studies, only showing increased pigmentation of the basal layer and pigmentary incontinence and many melanophages in the dermis. Finally, lichen planus pigmentosus must be considered as a pigmented variant of lichen planus.

Idiopathic eruptive macular pigmentation: report on two cases.

Idiopathic eruptive macular pigmentation (IEMP) is a rather under-reported condition of unknown etiology. Clinically consisting of benign hyperpigmented macules, the condition is characterized histopathologically by dermal melanization. It must be differentiated from lichen planus pigmentosus, erythema dyschromicum perstans, fixed drug eruption and mastocytosis.

A Case of Idiopathic Eruptive Macular Pigmentation Limited to Flexural Areas.

Idiopathic eruptive macular pigmentation is a rare condition characterized by asymptomatic pigmented macules involving the neck, trunk, and proximal portions of the extremities. On histopathologic examination, there was increased pigmentation of the basal layer in otherwise normal epidermis and scattered melanophages in the papillary dermis. We report a case of a 26-year-old woman with idiopathic eruptive macular pigmentation involving only the flexural areas of the body. This condition should be considered in the differential diagnosis of flexural hyperpigmented skin lesions.

Pigmentación macular eruptiva idiopática / Idiopathic eruptive macular pigmentation

La pigmentación macular eruptiva idiopática (PMEI) afecta principalmente a niños y adolescentes. Se caracteriza por manchas ovales diseminadas, de color café claro o gris pizarra, asintomáticas, de evolución crónica, involución espontánea y etiología desconocida. Se realizó un estudio retrospectivo y descriptivo, de corte transversal, mediante la revisión de las historias clínicas y archivos histológicos de pacientes con diagnóstico de PMEI correspondientes al período que va de noviembre de 2006 a julio de 2009.En el lapso estudiado se diagnosticó PMEI en siete pacientes. Rango de edad: 5-18 años (media:10,14 años), sexo femenino (100%). No se observaron antecedentes relacionados con la patología. El tiempo de evolución varió entre 1 y 36 meses (media: 11,28 meses) a partir del momento de la consulta. El seguimiento posterior se realizó en 4 casos, y 3 no regresaron a control. En todos los casos se realizaron estudios histopatológicos, en los cuales se observaron los criterios propios descriptos para esta entidad. Conclusiones. Las características clínicas y la edad de comienzo concuerdan con los reportes de la literatura. Todos los pacientes eran de sexo femenino, a diferencia de los casos previamente reportados, donde no hay diferencias entre ambos sexos o sólo un leve predominio del sexo masculino.

Idiopathic eruptive macular pigmentation (IEMP) affects mostly children and adolescents. It ischaracterized by scattered oval spots, light brown or slate gray, asymptomatic, a chronic evolution,spontaneous resolution and unknown etiology.We performed a retrospective cross-section analysis through review of clinical and histological recordsof patients with diagnosis of IEMP for the period November 2006 to July 2009.During the period of study IEMP was diagnosed on 7 patients. Age range: 5-18 years (media: 10.14years), female (100%). There was no previous history related to the disease.The duration of evolution ranged from 1 month to 36 months (media: 11.28 months) from thetime of consultation. Subsequent follow-up was performed on four patients, of which three didnot return to control. In all cases histopathological studies were performed, fulfilling the criteriadescribed for this entity.