Idiopathic guttate hypomelanosis: Presentation and Management.

Idiopathic guttate hypomelanosis (IGH) is a benign, typically asymptomatic, acquired leukoderma characteristically affecting mature individuals. Although the etiopathogenesis is unclear, chronic sun exposure and senile degeneration are important triggers. Researchers have been engaged in a continuous effort to unveil the gray areas encompassing different aspects of IGH pathogenesis. IGH is a clinical diagnosis; however, histopathology and dermoscopy may aid in quetionable cases. Patients often seek cosmetic treatment. There has been no standard therapy for this condition. Newer treatment modalities range from topical agents to procedure-based therapies and have enhanced the therapeutic armamentarium. Here we discuss the pathogenesis, presentation, and management of IGH.

Acquired disorders with hypopigmentation: A clinical approach to diagnosis and treatment.

Acquired hypopigmented skin changes are commonly encountered by dermatologists. Although hypopigmentation is often asymptomatic and benign, occasional serious and disabling conditions present with cutaneous hypopigmentation. A thorough history and physical examination, centered on disease distribution and morphologic findings, can aid in delineating the causes of acquired hypopigmented disorders. The second article in this 2-part continuing medical education series focuses on conditions with a hypopigmented phenotype. Early diagnosis and appropriate management of these disorders can improve a patient's quality of life, halt disease progression, and prevent irreversible disability.

A novel NCSTN gene mutation in a Chinese family with acne inversa.

Acne inversa (AI) is a chronic inflammatory disease of hair follicles. The pathogenesis of AI remains unclear. Haploinsufficiency of genes encoding γ-secretase components is the genetic basis for a subset of familial AI. Idiopathic guttate hypomelanosis (IGH) is a leukoderma characterized by multiple porcelain-white macules. Familial AI associated with IGH has not been reported previously. Herein, we present the pathogenic variation in a Chinese Han family with AI and IGH. Peripheral blood samples were collected from 16 members of the entire family. Eighteen exons and flanking introns of the NCSTN gene were amplified by polymerase chain reaction. Two hundred unrelated healthy Chinese subjects were used as controls. Sequencing results were analysed using CodonCode Aligner Software. Seven of the 16 family members in three generations were AI patients. Six AI patients also had IGH, while the other only had AI. One had IGH without AI. All AI patients carried the mutation, c.218delC, located in exon 4 of NCSTN. The deletion mutation led to a reading frame shift and the appearance of a premature termination codon (p.P73Lfs*15), resulting in the production of truncated protein. Family members without AI did not carry this mutation, indicating that it cosegregated with the phenotype. The mutation was not detected among the controls. This mutation has not been reported in the EXaC, HGMD, and dbSNP databases. In addition, we performed whole-exome sequencing on the proband and finally screened six candidate genes, ADAMTS2, BUB1B, CRB2, FBLN1, SEC24B, and WNK1, which we further validated in effected family members, and none of them were cosegregated. In conclusion, we identified a novel deletion mutation in exon 4 of NCSTN, which may underlie the molecular pathogenesis in this AI family. However, we found no relationship between this mutation and IGH.

Comparative study of ablative fractional photothermolysis versus topical retinoid cream in the treatment of idiopathic guttate hypomelanosis.

Background: Despite various treatments attempts, no prospective studies have compared the different therapeutic modalities for treatment of idiopathic guttate hypomelanosis (IGH). This study aimed to compare the efficacy of CO2 fractional laser (CO2FL), Er:YAG fractional laser (EYFL), and 0.025% topical tretinoin for treating IGH. Materials and Methods: A total of 122 lesions were analyzed from 18 patients (mean age, 70.00 ± 20.00 years). Treatment outcome evaluations included the visual analog scale (VAS) to assess lesion severity, investigator's global assessment (IGA) to assess the extent of improvement, and patient's satisfaction score (PSS). Results: Among the 122 lesions, 42, 40, and 40 were assigned to the CO2FL, EYFL, and topical tretinoin treatment groups, respectively. The VAS score was significantly improved after the second treatment. CO2FL and EYFL therapy had superior treatment efficacy compared to topical tretinoin (p < 0.01 in VAS, IGA, and PSS). There was no significant difference between the two types of fractional lasers. Conclusions: Ablative fractional laser (AFL) was an effective treatment with a relatively short treatment time without serious adverse events. We recommended AFL to treat IGH as an active treatment strategy.

Diffuse hyperpigmentation with guttate hypopigmentation: a new pigmentary disorder.

Diffuse hyperpigmentation with guttate hypopigmentation (DHGH) is a new acquired pigmentary disorder. Only a few cases have previously been reported in the Chinese population, in Chinese. To summarise the clinical, dermoscopic, and histopathological findings of DHGH in the English literature, to improve the recognition and management of this condition. This was a retrospective study to summarise the clinical, dermoscopic, and pathological findings of nine cases of DHGH. All nine patients with DHGH were female. The age at onset varied from 6 to 24 years (median 17 years). Patients were generally in good health without systemic disease. The lesions were often generalised to the trunk and extremities without any discomfort. Typical lesions were characterised by multiple uniform hypopigmented spots, 2-5 mm in diameter, irregularly distributed over diffuse hyperpigmentation. Dermoscopy revealed multiple blurred patchy areas of brownish pigmentation, sparse linear and dotted vessels, and perifollicular pigmentation on a white to bright white background, surrounded by brown hyperpigmentation. Histopathological findings included mild abnormal pigment of the epidermis, focal vacuolar degeneration of the basal cells, mild pigment incontinence and perivascular lymphocytic infiltration in the dermis. DHGH is a new entity with distinctive clinical manifestations that differ from those of other known pigmentary disorders. So far, DHGH has only been reported in the Chinese population. It may not be uncommon and has not received much attention due to the few reports. The aetiology and pathogenesis of DHGH are still unknown and require further investigation.

Do oxidative stress and melanin accumulation contribute to the pathogenesis of idiopathic guttate hypomelanosis: A prospective case-control study.

BACKGROUND: Oxidative stress parameters have been reported to be significantly higher than controls in diverse disorders of pigmentation but no such data seem to be extant for IGH. OBJECTIVES: To verify whether heightened oxidative stress levels are seen in patients with IGH (compared with matched controls) and quantify the same in blood using appropriate biochemical markers. Further, an attempt to determine if there might be any correlation between the amount of oxidative stress and the severity of the disease. METHODOLOGY: This prospective case-control study was conducted in Northern India. Thirty Indian patients (Fitzpatrick skin phototype (SPT) IV-V) were included. The diagnosis of IGH lesions was confirmed based on characteristic clinical, and dermoscopic characteristics and confirmed on histopathological examination (H & E) of one of the lesions. RESULTS: The oxidative stress enzyme level was significantly higher among the cases compared to controls which were statistically significant (p < 0.05). A positive correlation was also observed between the levels of these markers and number of lesions, but this finding was statistically not significant [p > 0.05]. Assessment of melanin level by Dopa staining method in the IGH lesions revealed irregularly distributed melanin with areas of high and low melanin content and greater accumulation in the basement layer. CONCLUSION: The results of this probably the first study evaluating oxidative stress in IGH show that the oxidative stress is increased in patients with IGH. Level of oxidative stress seems to be correlated with both age of the patient (more in the elderly) and number of lesions.

Dermatoses in the Elderly: Clinico-Demographic Profile of Patients Attending a Tertiary Care Centre.

BACKGROUND: Elderly population is vulnerable to develop a multitude of dermatological diseases, owing to comorbidities and polypharmacies. OBJECTIVE: To know the prevalence of dermatological conditions in elderly patients attending outpatient department, determine the pattern and relative frequency of skin diseases, and find the relation with associated comorbidities. MATERIALS AND METHODS: We performed a cross-sectional study on 250 patients, aged ≥60 years. Clinical diagnosis was done, followed by appropriate investigations when required. Descriptive data was analyzed on the parameters of range, mean ± S.D., frequencies, etc., Continuous variables were analyzed using unpaired t-test/Mann-Whitney U test and categorical data by Fisher's exact test/Chi-square test. Statistical software Medcalc version 10.2.0.0 for Windows vista was used. P value =0.05 was considered statistically significant. RESULTS: 250 patients were evaluated, 164 males (65.5%) and 86 females (34.4%). Mean age was 67.87 ± 7.29 years. Commonest disease category was infection (30%), followed by dermatitis (29.6%), papulo-squamous (18.4%), and immunobullous (6.4%). Difference in acute and chronic disease was significant (P = 0.0001). 30% had infections; fungal (50.66%), bacterial (32%), and viral (17.33%). 74 patients had dermatitis (29.6% of study population). Commonest systemic disease was hypertension (23.2%), followed by diabetes mellitus (19.6%). Association of diabetes mellitus was significant (P = 0.0014), more in infective dermatoses (P = 0.0007). All had signs of aging; idiopathic guttate hypomelanosis (51.2%), xerosis (45.2%), seborrheic keratosis (42.6%), cherry angioma (33.2%), senile acne (6.6%). Photoaging was noted as wrinkling (98.8%), freckles (35.6%), purpura (10.8%), telangiectasia (5.6%). People involved in outdoor activity had higher Glogau scale (3.01 ± 0.69) compared to those indoors (2.44 ± 0.74), statistically significant difference (P = 0.001). CONCLUSION: Our study is the first of its kind, in Eastern India, where we evaluated and explored the disease pattern and extent of geriatric dermatoses among patients attending dermatology OPD of a tertiary care hospital.

Idiopathic Guttate Hypomelanosis Treated with 308-nm Excimer Light and Topical Bimatoprost.

Idiopathic guttate hypomelanosis (IGH) is an acquired pigmentary disorder that is characterized by the presence of multiple hypopigmented macules on the shins and forearms. Albeit asymptomatic, it can cause considerable cosmetic anxiety. The pathogenesis is not fully understood and to date, there have been no successful treatments. We report a case of a 48-year-old female who presented with an 8-year history of multiple hypopigmented macules on both legs, typical of IGH. She previously failed to respond to topical pimecrolimus. She received targeted phototherapy with an excimer lamp (308 nm, 250-480 mJ), and a small patch was treated once daily with topical bimatoprost, in addition to the excimer lamp. After five sessions, better improvement was noted on the combination treatment patch; she received combination treatments for further six sessions. Good repigmentation has been achieved on the smaller macules. The larger depigmented macules continue to improve with further treatments. A combination of excimer light with topical bimatoprost appears to be a promising potential treatment option for IGH, a condition where management options are otherwise limited.

Peripilar "Guttate" Hypopigmentation of the Scalp and Idiopathic Guttate Hypomelanosis in Frontal Fibrosing Alopecia.

Frontal fibrosing alopecia (FFA) is characterized by progressive band-like scarring alopecia involving the frontotemporal hairline and limbs and irreversible alopecia of the eyebrows. Two opposite types of pigmentary alterations have been reported in FFA: hyper- and hypopigmentation. Here, we report a 57-year-old Hispanic female with FFA who presented with peripilar hypopigmentation and idiopathic guttate hypomelanosis on the upper extremities. To our knowledge, this is the first report of peripilar white halos in a patient of a White Hispanic origin. Upon review of other previous publications on trichoscopy of FFA, this pattern has not been detected. The common pattern of focal absence of melanocytes and melanin in both is another confirmation that FFA is associated with pigmentation abnormalities.

Efficacy of tip cryotherapy in the treatment of idiopathic guttate hypomelanosis (IGH): a randomized, controlled, evaluator-blinded study.

BACKGROUND: Idiopathic guttate hypomelanosis (IGH) is a common hypopigmentation affecting a large amount of older population. However, there is no standard treatment. Cryotherapy has been reported as an alternative therapy for years; nevertheless, there is no randomized controlled study to determine its efficacy. OBJECTIVES: To evaluate the efficacy and side effects of tip cryotherapy in IGH treatment. MATERIAL AND METHODS: Total 101 lesions were included. Forty-three lesions were treated with cryotherapy and 58 lesions were assigned as control. A single session of tip cryotherapy was delivered and remained for 5 s. Colorimeter was used to measure lesional luminosity at baseline and then monthly until 4 months. Digital photographs were evaluated by two blinded dermatologists. In addition, patients' assessments and side effects were assessed. RESULTS: Mean luminosity scale gradually decreased from baseline. Also, the score of the treated lesions has been significantly lower than that of the control lesions since week 8 (p = .005). At the fourth month, dermatologists' assessment revealed that 82.3% of the treated lesions comparing to only 2% of the control showed more than 75% improvement (p < .001). Burning sensation was the most common side effect. CONCLUSION: Tip cryotherapy appears to be an effective therapy with minimal adverse effect for IGH.

Combination of non-ablative fractional photothermolysis and 0.1% tacrolimus ointment is efficacious for treating idiopathic guttate hypomelanosis.

BACKGROUND: The efficacy of fractional photothermolysis and topical use of calcineurin inhibitors as treatments of idiopathic guttate hypomelanosis (IGH) have been reported. Data on combination treatments are lacking. OBJECTIVES: To evaluate the efficacy and safety of 1550-nm ytterbium/erbium fiber laser combined with 0.1% tacrolimus ointment as a treatment of IGH. METHODS: In each patient with IGH, two lesions were assigned as a treatment group, whilst two lesions on another side were chosen as control. Four treatments by fractional 1550-nm ytterbium/erbium fiber laser were delivered every four weeks combined with a twice daily topical application of 0.1% tacrolimus ointment. Lesional skin color was measured by colorimeter. Digital and dermoscopic digital photographs were taken and evaluated by three dermatologists. RESULTS: A total of 120 lesions were treated. Combination treatment normalized the relative lightness index of IGH which reached statistical significant compared with the control at week 12, after three sessions of laser treatment (p = 0.026). Physicians' assessment score revealed that 91.67% of the lesions on treatment side showed an improvement. Swelling and redness were the most common side effects which spontaneously resolved. CONCLUSION: Fractional 1550-nm ytterbium/erbium fiber laser combined with topical 0.1% tacrolimus ointment was effective for IGH.

Dermoscopic evaluation of idiopathic guttate hypomelanosis: A preliminary observation.

BACKGROUND: Depigmented skin lesions are of great concern in the society, especially in the Indian subcontinent. These comprise many infective and inflammatory conditions that cause apprehension and anxiety among patients due to the social stigma attached to these conditions. Idiopathic guttate hypomelanosis (IGH) appears similar to many depigmented lesions and differentiation of IGH from these conditions is difficult clinically as well as histopathologically. METHODS: Clinically suspected IGH were included in the study. All IGH lesions were confirmed by histopathology. Clinical data and dermoscopic patterns were analyzed and presented in proportions and percentages. RESULTS: Thirty patients were included in the study with 16 females and 14 males. Dermoscopy showed amoeboid, feathery, petaloid and nebuloid patterns in 12, 7, 6 and 1 patients respectively. Four patients had combination of these patterns. CONCLUSION: Dermoscopy of IGH revealed consistent patterns. Hence, we propose these patterns were specific to IGH and help clinician to differentiate many depigmented skin lesions from IGH in clinical practice. However, histopathology must be done for confirmation of diagnosis. Further studies on dermoscopy of IGH with histopathology correlation are proposed.

Skip areas of retained melanin: a clue to the histopathological diagnosis of idiopathic guttate hypomelanosis.

Biopsy findings in 55 cases of idiopathic guttate hypomelanosis (IGH) are reported. Most cases had a flat epidermis with loss of the rete pattern and a thickened orthokeratotic basket weave stratum corneum. The epidermis had markedly decreased to absent melanin in the basal layer and reduced numbers of melanocytes at the dermoepidermal junction. One-third of patients had a sparse perivascular lymphocytic infiltrate, whereas the rest had no significant dermal inflammation. These findings are in concordance with current literature. However, small foci of retained melanin in the basal layer (skip areas) alternating with larger areas of melanin loss were present in almost 80% of cases. This finding has not been reported earlier and appears to be quite specific to IGH and may be used as a clue to differentiate IGH from other similar conditions such as vitiligo and guttate morphea.

Therapeutic wounding - 88% phenol in idiopathic guttate hypomelanosis.

CONTEXT: Therapeutic wounding includes wounding the skin to induce pigmentation of the depigmented skin patches that was earlier used for repigmenting small patches of stable vitiligo. In this study, we have used the same principle to induce pigmentation in idiopathic guttate hypomelanosis (IGH) by spot peel with 88% phenol. AIMS: To study the efficacy of phenol in causing repigmentation in IGH and its adverse effect profile. SETTINGS AND DESIGN: Open prospective study. MATERIALS AND METHODS: Twenty patients with 139 IGH macules were subjected to spot peel. Eighty-eight percent phenol was applied with an ear bud once a month for two sittings. Patients were assessed both subjectively and objectively after every session and at the end of 3 months of initiation of therapy. RESULTS: Repigmentation was noted in 64% of IGH macules. More than 75% improvement was seen in 45% of the total IGH macules, while 41.5% showed 50-75% improvement at the end of three months. Persistent scabbing was the common adverse effect noted in 17.26% of lesions. CONCLUSION: Spot peel with 88% phenol is a safe, simple, cost-effective, outpatient procedure for IGH, which can be combined with other medical therapies.