Linear Hypopigmentation Research Articles

Linear Hypopigmentation on the Right Arm.

A 73-year-old woman presented to the dermatology clinic with hypopigmentation along the right arm. Her medical history was notable for prior treatment with intralesional triamcinolone injections for De Quervain tenosynovitis.

Peri-lymphatic Hypopigmentation Following Intralesional Triamcinolone: An Unusual Case Presentation

Corticosteroids are one of the most common drugs to be used and abused in the field of medicine and surgery. It can be used as topical, oral, intravenous, intra-articular and intralesional forms. Intralesional route of administration have the advantage of achieving high concentration of drug locally with minimal systemic absorption and side effects. However adverse effects like local atrophy, ulceration, infections etc. can occur. We report a case of 20-year-old male, who presented with linear hypopigmentation following intralesional steroids which is a rarer side effect and has been highlighted in this case report.

A Rare Genetic Diseases; Incontinentia Pigmenti: A Case Report

A 3 years-old girl, born of a 25-year-old mother, and of 30-year-old father, with a 1st degree consanguineous marriage. She was born full term after an uneventful pregnancy. History of similar disease was not present in her family. The parents consulted pour management of skin lesion. with no associated functional signs including no mental retardation, no epilepsy. they report that her skin had been fiery red at the birth time and vesicles had developed shortly afterwards. Then the lesions had cleared gradually and left linear hypo-pigmentation. On clinical examination, showed a facial asymmetry, especially mandibular. Hypopigmented atrophic streaks were seen in her face abdomen, back and limbs, with a baschko-linear path. And she had a syndactyly from the toes of the feet. And. The disease was diagnosed as Incontinentia pigmenti (IP). The ophthalmic exam was normal. Her genetic counseling was otherwise normal. A dental radio-panoramic, and MRI of the cerebral, and lumbosacral spine have been requested.

Cutaneous Linear Hypopigmentation as a Result of Periarticular Corticosteroid Injection

A 63-year-old African American woman presented to a dermatology clinic for evaluation of a 2-month history of asymptomatic progressive hypopigmentation of her right hand and forearm. She had a family history of unspecified hypopigmentation in her father, and she wanted to be evaluated for possible vitiligo.

Cutaneous Linear Hypopigmentation as a Result of Periarticular Corticosteroid Injection

A 63-year-old African American woman presented to a dermatology clinic for evaluation of a 2-month history of asymptomatic progressive hypopigmentation of her right hand and forearm. She had a family history of unspecified hypopigmentation in her father, and she wanted to be evaluated for possible vitiligo.

Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome.

Hypopigmentation along Blaschko’s lines is a hallmark of a poorly defined group of mosaic syndromes whose genetic causes are unknown. Here we show that postzygotic inactivating mutations of RHOA cause a neuroectodermal syndrome combining linear hypopigmentation, alopecia, apparently asymptomatic leukoencephalopathy, and facial, ocular, dental, and acral anomalies. Our findings pave the way towards elucidating the etiology of pigmentary mosaicism and highlight the role of RHOA in human development and disease.

Intralesional Corticosteroid-Induced Perilesional and Perilymphatic Linear Hypopigmentation Successfully Treated With a 308-nm Excimer Light

Intralesional Corticosteroid-Induced Perilesional and Perilymphatic Linear Hypopigmentation Successfully Treated With a 308-nm Excimer Light

Absence of an osteopetrosis phenotype in IKBKG (NEMO) mutation-positive women: A case-control study.

NF-κB essential modulator (NEMO), encoded by IKBKG, is necessary for activation of the ubiquitous transcription factor nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB). Animal studies suggest NEMO is required for NF-κB mediated bone homeostasis, but this has not been thoroughly studied in humans. IKBKG loss-of-function mutation causes incontinentia pigmenti (IP), a rare X-linked disease featuring linear hypopigmentation, alopecia, hypodontia, and immunodeficiency. Single case reports describe osteopetrosis (OPT) in boys carrying hypomorphic IKBKG mutations. We studied the bone phenotype in women with IP with evaluation of radiographs of the spine and non-dominant arm and leg; lumbar spine and femoral neck aBMD using DXA; μ-CT and histomorphometry of trans-iliac crest biopsy specimens; bone turnover markers; and cellular phenotype in bone marrow skeletal (stromal) stem cells (BM-MSCs) in a cross-sectional, age-, sex-, and BMI-matched case-control study. X-chromosome inactivation was measured in blood leucocytes and BM-MSCs using a PCR method with methylation of HpaII sites. NF-κB activity was quantitated in BM-MSCs using a luciferase NF-κB reporter assay. Seven Caucasian women with IP (age: 24-67 years and BMI: 20.0-35.2 kg/m2) and IKBKG mutation (del exon 4-10 (n = 4); c.460C>T (n = 3)) were compared to matched controls. The IKBKG mutation carriers had extremely skewed X-inactivation (>90:10%) in blood, but not in BM-MSCs. NF-κB activity was lower in BM-MSCs from IKBKG mutation carriers (n = 5) compared to controls (3094 ± 679 vs. 5422 ± 1038/μg protein, p < 0.01). However, no differences were identified on skeletal radiographics, aBMD, μ-architecture of the iliac crest, or bone turnover markers. The IKBKG mutation carriers had a 1.7-fold greater extent of eroded surfaces relative to osteoid surfaces (p < 0.01), and a 2.0-fold greater proportion of arrested reversal surface relative to active reversal surface (p < 0.01). Unlike mutation-positive males, the IKBKG mutation-positive women did not manifest OPT.

Intralesional Corticosteroid-Induced Perilesional and Perilymphatic Linear Hypopigmentation Successfully Treated With a 308-nm Excimer Light

Department of Dermatology, China Medical University Hospital, China Medical University, Taichung, Taiwan Department of Dermatology, China Medical University Hospital, Taichung, Taiwan School of Medicine, China Medical University, Taichung, Taiwan The authors have indicated no significant interest with commercial supporters.

Linear hypopigmentation following vein path

Linear hypopigmentation following vein path

Branch-shaped Cutaneous Hypopigmentation and Atrophy after Intralesional Triamcinolone Injection

Cutaneous changes after local corticosteroid administration may include dermal atrophy, hyperpigmentation, alopecia, and hypopigmentation. Linear hypopigmentation and atrophy after intralesional injection of triamcinolone acetonide has been reported in the literature as a very rare side effect. A 30-year-old woman visited our dermatology department for a linear hypopigmented patch with atrophy from her left foot to the lower margin of the knee. The lesion developed after injection of an intralesional corticosteroid. The patient was diagnosed with linear hypopigmentation and atrophy secondary to the triamcinolone injection.

Varied presentations of incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis with cutaneous, neurological, ophthalmological and dental manifestations. 80% of cases have a deletion mutation in the nuclear factor-κB (NF-κB) essential modulator (NEMO) gene located on chromosome Xq28. This gene is required for activation of the transcription factor NF-κB which is central to many immune, inflammatory and apoptotic pathways. The authors present two cases of IP to demonstrate the varied presenting features of this condition. The first case is a 9-month-old girl who presented mildly unwell, with erythematous lines on her abdomen in a “Chinese-writing” distribution. Further examination showed hyperkeratotic lesions on her calves, and a hyperkeratotic papule and blister which had been present on her hand since birth. Her mother had a history of two spontaneous abortions. She had linear hypopigmentation on the legs and only 16 teeth. A diagnosis was made of a viral exanthem arising on a background of IP. She is now 5 years old and has linear hyperpigmentation on her legs, teeth abnormalities and patchy alopecia. Her mother has a confirmed mutation in the NEMO gene. The second case is a 3-month-old baby girl who had developed seizures on the second day of life. An MRI showed two unusual areas on the lateral ventricle which had been interpreted as small areas of ischaemia. She developed linear erythematous papules, blistering and crusting on her groin and trunk on day 4 and a clinical diagnosis of IP was made. Genetic analysis confirmed a mutation in the NEMO gene. Her mother has no mutation in the NEMO gene. There are four cutaneous stages classically seen in IP from birth: vesicular, verrucous, hyperpigmented and, in adulthood, an atrophic hypopigmented stage. In both of our cases, the presenting features were not the classical cutaneous features of IP: case 1 presented with a viral exanthem within the lines of Blaschko; case 2 presented with neurological involvement. The authors present these two cases of IP as examples of the varied presentations of this condition to increase awareness of possible presenting features of this unusual genodermatosis.

Incontinentia pigmenti in a male infant with limited cutaneous expression

<h3>Aims</h3> Incontinentia pigmenti (IP) is an uncommon X-linked dominant neurocutaneous syndrome, lethal in the majority of affected males in utero and variably expressed in females. The authors report a clinical case of IP in a male infant but without the usual genetic mutations. <h3>Methods</h3> Case report and review of the literature. <h3>Results</h3> A newborn male infant was noted to have a blistering rash on his left lower leg. On examination, there were linearly arranged pustules, vesicles and hyperpigmented macules affecting the dorsal aspect of his left leg and his left axilla. These became verrucous in part at 2 months of age and subsequently resolved leaving linear patches of hyperpigmentation. Aged 9 months, he is otherwise well and thriving on the 75th centile with/without hair, nail or ocular abnormalities and normal development. There is no family history of ocular or neurological disease or rashes in the neonatal period, but his mother does have a few subtle hypopigmented streaks on her right lower leg. On the basis of a history of typical sequential cutaneous lesions and the presence of linear hypopigmentation in his minimally affected mother, a diagnosis of IP was made. His karyotype was 46XY and DNA analysis on blood did not reveal the NEMO Δ 4–10 rearrangement. <h3>Conclusions</h3> IP is caused by mutations in the NEMO gene on chromosome Xq28. Clinical presentation varies considerably even among family members ranging from subtle cutaneous signs as in our case through to severe, incapacitating neurological and ophthalmological manifestations. Many male patients have disease limited to cutaneous involvement of one or two limbs.1 Some male cases have Klinefelter9s syndrome (XXY) or as we postulate for our case may arise as a result of somatic mosaicism or less deleterious mutations in the NEMO gene.2

Combined use of intense pulsed light and Q‐switched ruby laser for complex dyspigmentation among asian patients

Dyspigmentation is a common cosmetic concern among Asians. Many individuals exhibit mixed pigmentary entities including melasma, flat/small seborrheic keratoses, ephelides, solar lentigines and acquired bilateral nevus-of-Ota-like macules (ABNOM). We term this phenomenon complex dyspigmentation (CD) and suggest that a combination strategy may be more efficacious than any singular modality. To determine the effectiveness of combined intense-pulsed light (IPL) for global photorejuvenation along with Q-switched ruby laser (QSRL) for targeted pigment dissolution. Twenty-five Korean women with CD (defined as >2 types of facial pigmentary disorders) were initially treated with IPL followed by repeat treatments every 3-4 weeks as needed. The QSRL treatments, set at low fluence, were added either during the same session or within 1 week of the IPL treatment. Improvement was assessed by the patient, the treating physician and a blinded evaluation of pre- and post-treatment photographs. Using a 4-point scale (1 = poor, 2 = fair, 3 = good, 4 = excellent), 19/25 patients (76%) reporting good-to-excellent response (score "3" or "4"). Two independent physician assessment revealed that 15/25 patients (60%) showed 76-100% improvement while 19/25 patients (76%) showed at least 50% improvement. Side-effects were minimal: 3 patients had transient post-inflammatory hyperpigmentation (12%) and 1 patient (4%) had linear hypopigmentation. Combination treatment with IPL and QSRL is an effective and safe treatment for CD among Asian patients.

Sternal malformation/vascular dysplasia syndrome with linear hypopigmentation

We report a 7-year-old boy who presented with a facial haemangioma, a circumscribed depression over the sternum, coarctation of the aorta, ventricular septal defect and dysplastic cerebral arteries responsible for an episode of acute infarct. This combination of clinical features has been described as the sternal malformation/vascular dysplasia syndrome or PHACES syndrome. At the age of 5 years, lines of hypopigmentation were noted on the right arm, the right hand and the back, along the lines of Blaschko, with no history of any preceding inflammatory changes, and have persisted unchanged. These pigmentary changes have not previously been reported in association with this syndrome.

Linear Hypopigmentation After Triamcinolone Injection: A Rare Complication of a Common Procedure

The development of linear hypopigmentation after intralesional or intraarticular injection of triamcinolone acetonide has been reported in the literature as a very rare side effect. This case report describes a patient with linear hypopigmentation and discusses the possible pathophysiology. Clinicians involved in the care of hypertrophic scars and keloids need to be aware of this rare side effect so that they can guide their patients appropriately. They need to understand the pathogenesis of this complication better so that it may become avoidable.

Linear hypopigmentation and hyperpigmentation, including mosaicism.

Linear streaks of hypopigmentation or hyperpigmentation along Blaschko's lines are currently grouped under the names hypomelanosis of Ito (HI) and linear and whorled hypermelanosis (LWH). Recent studies have suggested that these linear pigmentary anomalies reflect underlying genetic mosaicism. Mosaic individuals are composed of two or more genetically distinct cell populations, a normal and an abnormal population. In HI and LWH, the types of genetic defects that are detectable in the abnormal population are highly variable, including tetraploidy, partial or complete trisomies, translocations, and point mutations. These results, together with recent studies indicating the incidence of extracutaneous anomalies is lower in HI but higher in LWH than previously estimated, have important clinical implications. The need for a revised nomenclature as well as possible modifications in current recommendations for patient management are discussed.

Hypomelanosis of Ito with Growth Hormone Deficiency

We report a case of Hypomelanosis of Ito, a neurocutaneous syndrome, with GH deficiency.Case: A 10-year-old Japanese girl was referred to our hospital with hypopigmentation and short stature. She was born at term (birth weight, 2, 460g) and delivery was normal. Her growth failure was severe and her height was 105.2cm (-5.3SD). Symmetrical linear hypopigmentation on the trunk and extremities was noted. Her mental development was retarded, but she had no convulsions. Her bone age was 7 years. Peak GH level after insulin and arginine stimulation was 5.5 and 6.6ng/ml, respectively. Human GH therapy was started at a dose of 0.5U/kg/week. Her height gain was 6.4cm/year for 6 months.This is the first report on GH secretion in hypomelanosis of Ito with short stature. GH therapy was effective in the short term in this patient.

HEMIFACIAL HYPOPLASIA, HYPOPIGMENTATION, AND DIGITAL ANOMALY SYNDROME

We report a unique syndrome of craniofacial anomalies, hypopigmentation and digital abnormalities in 2 unrelated children. Patient 1, a 5 y.o. male of normal intelligence, has right facial hypoplasia, coloboma, cupped ear, sensorineural heating loss, microdontia, hypodontia, and focal areas of scalp alopecia. There is linear hypopigmentat ion on the lower lip, chest, eight arm, hand and leg. The right 2nd metatarsal is short. Growth and development were normal. Patient 2 is a 7 y.o. female with facial features identical to patient 1 including right facial hypoplasia, eyelid coloboma, focal scalp alopecia and microdontia. Linear and swirled areas of hypopigmented skin are present on her left chest, shoulder and right right leg. The tight 2nd metatarsal is shott and there is postaxial polysyndactyly. This patient also has imperforate anus and a double collecting system. Intelligence is normal. Chromosome analysis of petiphetal blood and normally pigmented and depigmented skin from both patients was normal, ruling out mosaic ism. We propose that this new syndrome may involve a defect in neural crest cell prolifetation or migration causing both the facial hypoplasia and pigmentary abnormalities.

Linear hypopigmentation after intra-articular corticosteroid injection

Linear hypopigmentation after intra-articular corticosteroid injection