Fenómeno infrecuente la co-localización de alopecia frontal fibrosante y vitiligo en un varón: informe de un caso / Infrequent phenomenon the co-localization of fibrosing frontal alopecia and vitiligo in a male: case repor

La alopecia frontal fibrosante es una alopecia cicatricial que se caracteriza por la recesión de la línea de implantación frontotemporal que afecta principalmente a mujeres caucásicas en edad posmenopáusica y rara vez a hombres. Actualmente los mecanismos específicos de desarrollo continúan en estudio; sin embargo hay varias hipótesis sobre la asociación de la alopecia frontal fibrosante con otros trastornos autoinmunitarios. Se comunica el caso de un paciente masculino de 58 años con alopecia frontal fibrosante en áreas comprometidas por vitiligo. (AU)

Frontal fibrosing alopecia is a cicatricial alopecic characterized by progressive regression of the frontotemporal hairline. It usually affects postmenopausal caucasian women, and rarely men. Currently the specific mechanisms of development remain unknown, however there are several hypotheses about the association of frontal fibrosing alopecia with other autoimmune disorders. The case of a 58-year-old male patient with frontal fibrosing alopecia in areas affected by vitiligo. (AU)

Association between three autoimmune diseases: vitiligo, primary biliary cirrhosis, and Sjögren's syndrome

Abstract Although the association of multiple autoimmune diseases has already been widely described, no reports of the association between vitiligo, primary biliary cirrhosis and Sjogren's syndrome were retrieved in the SciELO and PubMed databases. The authors describe the case of a female patient who was diagnosed with primary biliary cirrhosis and Sjogren's syndrome at age 54. At age 58, she developed vitiligo restricted to the face, associated with significant impairment of self-esteem and quality of life. Antinuclear antibody was negative at the onset of the condition, but became positive after phototherapy initiation. In general, the occurrence of multiple autoimmune diseases in the same patient is known as a mosaic of autoimmunity. However, specific mechanisms appear to interconnect primary biliary cirrhosis and Sjogren's syndrome, such as PDC-E2-mediated generalized epithelitis.

Leucine-rich glioma inactivated 3: a novel keratinocyte-derived melanogenic cytokine in vitiligo patients

Abstract: Background: In-vitro studies showed that Leucine-rich glioma inactivated 3 (LGI3) is a keratinocyte-derived cytokine that stimulates melanin synthesis and is increased after ultra violet B (UVB) irradiation. So, we postulated that LGI3 may be involved in vitiligo aetiopathogenesis and may participate in narrow band ultra violet B (NB-UVB) induced pigmentation in vitiligo. Objectives: To assess this hypothesis, lesional LGI3 immunohistochemical expression of vitiligo patients before and after NB-UVB phototherapy was studied, and its correlation with repigmentation was evaluated. Methods: Forty vitiligo patients and 20 age, sex, and skin phenotype-matched controls were enrolled. Patients were treated with NB-UVB thrice weekly for 12 weeks. VASI score was evaluated before and after NB-UVB sessions. For vitiligo patients, baseline LGI3 immunohistochemical staining was estimated, and compared to that of controls and to its post-treatment data in those patients. Results: Baseline LGI3 immunohistochemical studied parameters (expression, intensity, percentage and H score) were significantly lower in vitiligo cases than controls (p=0.003, 0.013, 0.001 and 0.001 respectively). After 12 weeks of NB-UVB phototherapy, these LGI3 immunohistochemical parameters were up-regulated and became comparable to that of controls (p >0.05 for all). There was a significant positive correlation between the improvement of both VASI score and LGI3 H score mean values (r=-0.349 , p=0.027). Study limitations: Small number of investigated subjects. Conclusions: Decreased LGI3 protein may play an active role in vitiligo pathogenesis and its up-regulation after NB-UVB phototherapy, may actively participate in NB-UVB photo-induced melanogenesis.

Mucosal vitiligo in angles of the mouth: clinical and fluorescence aspects

SUMMARY Vitiligo is the most common depigmenting, chronic acquired disease of the skin and mucosa. However, vitiligo of an unclassified type and mucosal subtype affecting only one area of the mucosa is considered quite uncommon. The diagnosis of vitiligo, regardless of its type, is clinical. Nonetheless, a device that allows the visualization of the tissue fluorescence may be useful for confirming the diagnosis. We present the use of wide-field optical fluorescence device for complementary examination and diagnosis of unusual cases of mucosal vitiligo located only in angles of the mouth.

RESUMO O vitiligo é a doença crônica adquirida despigmentante mais comum da pele e/ou da mucosa. Entretanto, o vitiligo do tipo não classificado e subtipo de mucosa afetando apenas uma área da mucosa é considerado bastante incomum. O diagnóstico de vitiligo, independentemente do seu tipo, é clínico. No entanto, o uso de um dispositivo que permite a visualização da fluorescência tecidual pode ser útil para a confirmação do diagnóstico de vitiligo. Apresentamos o uso do dispositivo de exame complementar de fluorescência óptica de campo amplo para o diagnóstico de um caso incomum de vitiligo de mucosa localizado apenas em ângulos da boca.

Macrophage migration inhibitory factor as an incriminating agent in vitiligo

Abstract: Background: Vitiligo is an autoimmune skin disorder in which the loss of melanocytes is mainly attributed to defective autoimmune mechanisms and, lately, there has been more emphasis on autoinflammatory mediators. Among these is the macrophage migration inhibitory factor, which is involved in many autoimmune skin diseases. However, little is known about the contribution of this factor to vitiligo vulgaris. Objective: To determine the hypothesized role of migration inhibitory factor in vitiligo via estimation of serum migration inhibitory factor levels and migration inhibitory factor mRNA concentrations in patients with vitiligo compared with healthy controls. We also aimed to assess whether there is a relationship between the values of serum migration inhibitory factor and/or migration inhibitory factor mRNA with disease duration, clinical type and severity in vitiligo patients. Methods: Evaluation of migration inhibitory factor serum level and migration inhibitory factor mRNA expression by ELISA and real-time PCR, respectively, were performed for 50 patients with different degrees of vitiligo severity and compared to 15 age- and gender-matched healthy volunteers as controls. Results: There was a highly significant increase in serum migration inhibitory factor and migration inhibitory factor mRNA levels in vitiligo cases when compared to controls (p<0.001). There was a significant positive correlation between both serum migration inhibitory factor and migration inhibitory factor mRNA concentrations in vitiligo patients, and each of them with duration and severity of vitiligo. In addition, patients with generalized vitiligo have significantly elevated serum migration inhibitory factor and mRNA levels than control subjects. Study limitations: Small number of investigated subjects. Conclusions: Migration inhibitory factor may have an active role in the development of vitiligo, and it may also be a useful index of disease severity. Consequently, migration inhibitory factor may be a new treatment target for vitiligo patients.

Diabetes mellitus and the skin

Abstract: Several dermatoses are routinely associated with diabetes mellitus, especially in patients with chronic disease. This relationship can be easily proven in some skin disorders, but it is not so clear in others. Dermatoses such necrobiosis lipoidica, granuloma annulare, acanthosis nigricans and others are discussed in this text, with an emphasis on proven link with the diabetes or not, disease identification and treatment strategy used to control those dermatoses and diabetes.

Nail abnormalities in patients with vitiligo

Abstract: Background: Vitiligo is an acquired pigmentary skin disorder affecting 0.1-4% of the general population. The nails may be affected in patients with an autoimmune disease such as psoriasis, and in those with alopecia areata. It has been suggested that nail abnormalities should be apparent in vitiligo patients. Objective: We sought to document the frequency and clinical presentation of nail abnormalities in vitiligo patients compared to healthy volunteers. We also examined the correlations between nail abnormalities and various clinical parameters. Methods: This study included 100 vitiligo patients and 100 healthy subjects. Full medical histories were collected from the subjects, who underwent thorough general and nail examinations. All nail changes were noted. In the event of clinical suspicion of a fungal infection, additional mycological investigations were performed. Results: Nail abnormalities were more prevalent in the patients (78%) than in the controls (55%) (p=0.001). Longitudinal ridging was the most common finding (42%), followed by (in descending order): leukonychia, an absent lunula, onycholysis, nail bed pallor, onychomycosis, splinter hemorrhage and nail plate thinning. The frequency of longitudinal ridging was significantly higher in patients than in controls (p<0.001). Conclusions: Nail abnormalities were more prevalent in vitiligo patients than in controls. Systematic examination of the nails in such patients is useful because nail abnormalities are frequent. However, the causes of such abnormalities require further study. Longitudinal ridging and leukonychia were the most common abnormalities observed in this study.

Manejo despigmentante de vitiligo generalizado con monobenzil eter de hidroquinona / Depigmenting management of generalized vitiligo with hydroquinone monobenzyl ether

El vitiligo es una patología crónica, recidivante y difícil de tratar. El objetivo primario del tratamiento es inducir la repigmentación, sin embargo, en casos extensos refractarios a tratamiento, se puede realizar despigmentación para corregir la discromía. Dentro de los tratamientos despigmentantes en vitiligo, a la fecha el único aprobado por la FDA es el Monobenzil éter de hidroquinona (monobenzona). Se expone el caso de una paciente con vitiligo extenso y refractario a tratamiento que fue manejado con monobenzona. El resultado fue exitoso durante tiempo prolongado, con recaída parcial al suspender el medicamento. La recaída remitió con el reinicio de la monobenzona. Sin nueva recaída actualmente con tratamiento de mantención 3v/semana. La monobenzona induce acromía secundaria a necrosis de los melanocitos. Se requiere su uso 1 a 2 veces al día por 6-12 meses para lograr la despigmentación. En pacientes adecuadamente seleccionados, es una alternativa válida para el manejo del vitiligo. Se presenta un caso exitoso de despigmentación con monobenzona. Actualmente, la paciente está muy satisfecha con los resultados.

Vitiligo is a chronic, recurrent pathology, difficult to treat. The primary goal of treatment is to induce repigmentation, however, in extensive cases refractory to treatment depigmentation of surrounding skin may be performed to correct the cosmetic misbalance. To date the only depigmenting treatment for vitiligo approved by the FDA is the hydroquinone monobenzyl ether (monobenzone). We report the case of a patient with extensive vitiligo refractory to treatment managed with monobenzone. The result was successful for a long time, with partial relapse when the drug was discontinued. The relapse ended with the restart of the monobenzone. No new relapse seen with maintenance treatment 3 times a week. Monobenzone induces acromy due to melanocyte necrosis. To achieve depigmentation, it is used 1 to 2 times a day for 6 to 12 months. In adequately selected patients, it is a valid alternative for the management of vitiligo. A successful case of monobenzone depigmentation in dyschromia due to extensive vitiligo. Patient currently very satisfied with the results.

Vitiligo - Part 2 - classification, histopathology and treatment

In an unprecedented effort in the field of vitiligo, a global consensus resulted on a suggested new classification protocol for the disease. The main histopathological finding in vitiligo is the total absence of functioning melanocytes in the lesions, while the inflammatory cells most commonly found on the edges of the lesions are CD4+ and CD8+ T lymphocytes. Physical and pharmacological treatment strategies aim to control the autoimmune damage and stimulate melanocyte migration from the unaffected edges of lesions and the outer hair follicle root sheath to the affected skin; moreover, surgical treatments can be combined with topical and physical treatments.

Syndrome in Question

Multiple autoimmune syndrome is a rare condition, described by Humbert and Dupond in 1988. It is defined by the association of at least 3 autoimmune diseases in the same patient. Vitiligo is the most common skin condition in this syndrome. This article presents the case of a 31-year-old male with vitiligo, alopecia areata, Crohn's disease, psoriasis vulgaris and oral lichen planus. The rarity of this case is highlighted by the coexistence of four autoimmune skin diseases in association with Crohn's disease, never described in the literature.

Compensation for Occupational Skin Diseases

The Korean list of occupational skin diseases was amended in July 2013. The past list was constructed according to the causative agent and the target organ, and the items of that list had not been reviewed for a long period. The revised list was reconstructed to include diseases classified by the International Classification of Diseases (10th version). Therefore, the items of compensable occupational skin diseases in the amended list in Korea comprise contact dermatitis; chemical burns; Stevens-Johnson syndrome; tar-related skin diseases; infectious skin diseases; skin injury-induced cellulitis; and skin conditions resulting from physical factors such as heat, cold, sun exposure, and ionized radiation. This list will be more practical and convenient for physicians and workers because it follows a disease-based approach. The revised list is in accordance with the International Labor Organization list and is refined according to Korean worker's compensation and the actual occurrence of occupational skin diseases. However, this revised list does not perfectly reflect the actual status of skin diseases because of the few cases of occupational skin diseases, incomplete statistics of skin diseases, and insufficient scientific evidence. Thus, the list of occupational diseases should be modified periodically on the basis of recent evidence and statistics.

Clinicopathological correlation of acquired hypopigmentary disorders.

Acquired hypopigmentary disorders comprise a significant group of disorders that affect Indians and Asians. The pigment disturbance in darker skin individuals can be very distressing to the patient and the family. These disorders cover a wide array of pathologies including infections, autoimmune processes, lymphoproliferative disorders, and sclerosing diseases. Histological diagnosis is particularly important because treatments for these diseases are varied and specific. This review will focus on histopathological diagnosis based on clinicopathological correlation for commonly encountered disorders such as leprosy, vitiligo, lichen sclerosus, pityriasis alba (PA), and pityriasis versicolor (PV). Atypical or uncommon clinical presentation of classic diseases such as hypopigmented mycosis fungoides (HMF) and hypopigmented sarcoidosis are also included.

Relationship of macrophage migration inhibitory factor levels in PBMCs, lesional skin and serum with disease severity and activity in vitiligo vulgaris

Melanocyte loss in vitiligo vulgaris is believed to be an autoimmune process. Macrophage migration inhibitory factor (MIF) is involved in many autoimmune skin diseases. We determined the possible role of MIF in the pathogenesis of vitiligo vulgaris, and describe the relationship between MIF expressions and disease severity and activity. Serum MIF concentrations and mRNA levels in PBMCs were measured in 44 vitiligo vulgaris patients and 32 normal controls, using ELISA and real-time RT-PCR. Skin biopsies from 15 patients and 6 controls were analyzed by real-time RT-PCR. Values are reported as median (25th-75th percentile). Serum MIF concentrations were significantly increased in patients [35.81 (10.98-43.66) ng/mL] compared to controls [7.69 (6.01-9.03) ng/mL]. MIF mRNA levels were significantly higher in PBMCs from patients [7.17 (3.59-8.87)] than controls [1.67 (1.23-2.42)]. There was also a significant difference in MIF mRNA levels in PBMCs between progressive and stable patients [7.86 (5.85-9.13) vs 4.33 (2.23-8.39)] and in serum MIF concentrations [40.47 (27.71-46.79) vs 26.80 (10.55-36.07) ng/mL]. In addition, the vitiligo area severity index scores of patients correlated positively with changes of both serum MIF concentrations (r = 0.488) and MIF mRNA levels in PBMCs (r = 0.426). MIF mRNA levels were significantly higher in lesional than in normal skin [2.43 (2.13-7.59) vs 1.18 (0.94-1.83)] and in patients in the progressive stage than in the stable stage [7.52 (2.43-8.84) vs 2.13 (1.98-2.64)]. These correlations suggest that MIF participates in the pathogenesis of vitiligo vulgaris and may be useful as an index of disease severity and activity.

Accidental PUVA burns, vitiligo and atopic diathesis resulting in prurigo nodularis: a logical but undocumented rarity / Prurigo nodular induzido por queimaduras acidentais por PUVA, vitiligo e diátese atópica: uma raridade lógica, mas não documentada

Vitiligo is a dreaded disease in India due to its social and cultural consequences. PUVA and PUVAsol are the main treatment modalities for vitiligo vulgaris. To the best of our knowledge, this is the first case of accidental PUVA burns eventuating in prurigo nodularis lesions to be reported in a female patient who was undergoing home PUVA therapy. The itch is so prominent and disabling that the focus of the patient has shifted from treating her vitiligo to ameliorating the pruritus.

O vitiligo é uma doença temida na Índia por suas consequências sociais e culturais. As principais modalidades de tratamento do vitiligo vulgar são as terapias PUVA e PUVAsol. Pelo que sabemos, este é o primeiro relato de caso de prurigo nodular induzido por queimaduras acidentais por PUVA em paciente do sexo feminino em tratamento domiciliar com PUVA. O prurido é tão intenso e incapacitante que o foco da paciente passou do vitiligo à busca constante pela melhora do prurido.

Association of giant congenital melanocytic nevus, halo nevus and vitiligo in a 75-year-old patient / Associação de nevo melanocítico congênito gigante, nevo halo e vitiligo. Relato de caso em paciente de 75 anos

A giant congenital melanocytic nevus represents a rare condition. The halo phenomenon may be seen in congenital or acquired melanocytic nevi. In the literature, association of halo nevus and giant congenital melanocytic nevus is rare and the association of both with vitiligo even more rare. A 75-yearold woman at first consultation complained of a hyperchromic bluish-brown hairy macula on the lower back, buttocks and thighs present since birth and an achromic halo of onset three years ago. The histological features were consistent with congenital melanocytic nevus and halo nevus, respectively. After two years the patient developed achromic areas in normal skin, histologically consistent with vitiligo. The authors emphasize the rarity of this triple combination, the patient's age and the absence of malignant degeneration to date.

Nevo melanocitico congênito gigante constitui uma condição rara. O fenômeno halo pode ser observado em nevos melanocíticos congênitos ou adquiridos. Na literatura a associação nevo halo e nevo melanocítico congênito gigante é rara e a associação de ambos com vitiligo ainda mais rara. Mulher de 75 anos que à primeira consulta apresentava mácula hipercrômica castanho-azulada pilosa na região lombar, nádegas e coxas desde o nascimento e halo acrômico de aparecimento há 3 anos. Os histológicos foram compatíveis com nevo melanocítico congênito e nevo halo respectivamente. Após dois anos evoluiu com áreas de acromia à distância, com histológico de vitiligo. Os autores ressaltam a raridade desta tripla associação; a idade da paciente e a ausência de degeneração maligna até o presente momento.

Función del peróxido de hidrógeno en el vitíligo / Function of hydrogen peroxide in cases of vitiligo

El vitíligo es un desorden de la pigmentación que tiene una incidencia entre el 0,5 y 3 por ciento en la población mundial. Se caracteriza por una despigmentación de la piel provocada por la presencia de melanocitos afuncionales en la epidermis. Cuando ocurren eventos desencadenantes del estrés oxidativo, las concentraciones de peróxido de hidrógeno se incrementan ostensiblemente en melanocitos y queratinocitos. Los queratinocitos actúan como fuente de especies reactivas de oxígeno y transfieren a los melanocitos grandes cantidades de peróxido de hidrógeno. Evidencias experimentales han demostrado que como consecuencias de las altas concentraciones de peróxido de hidrógeno en la epidermis de pacientes con vitíligo, se afectan las concentraciones de butirilcolinesterasa y acetilcolineterasa, la actividad enzimática en el ciclo de las 6HB4, los péptidos derivados de proopiomelanocortinas y las proteínas del estrés entre otros muchos eventos. Todos estos eventos, tras una situación precipitante sobre un individuo genéticamente predispuesto, podrían promover cambios en la pigmentación de la piel que lo conducen, en última instancia, al vitíligo

Vitiligo is a pigmentation disorder with an incidence between the 0,5 and the 3 percent at worldwide. It is characterized by a skin depigmentation provoked by presence of dysfunctional melanocytes in epidermis. When oxidative stress triggering events are present, hydrogen peroxide concentrations clearly increase in the melanocytes and keratinocytes. The keratinocytes act as a source of oxygen and transfer to melanocytes high amounts of hydrogen peroxide. Experimental evidences have demonstrated that due to the high hydrogen peroxide concentrations in epidermis of vitiligo patients affecting the concentrations of butyryl-cholinesterase and the acetylcholinesterase, the enzymatic activity in 6HB4 cycle, propiomelanocortins and stress protein among many events. All these events after a hasty situation on a genetically predisposing subject could to promote changes in skin pigmentation that as a last resort leading to vitiligo

Leucodermia sifilítica: expressão rara do secundarismo diagnosticada por exame histopatológico / Leucoderma syphiliticum: a rare expression of the secondary stage diagnosed by histopathology

Biópsias são ocasionalmente necessárias para confirmação diagnóstica de sífilis secundária, normalmente obtida por correlação clínico-sorológica. Entretanto, o exame histopatológico pode oferecer pistas que conduzam a um diagnóstico em casos antes insuspeitos ou de apresentação clínica incomum. Apresentamos um paciente de 35 anos, há dois com lesões acrômicas vitiligoides, para o qual sífilis foi sugerida somente após o exame histopatológico. Alguns aspectos microscópicos observados são discutidos e comparados com dados disponíveis na literatura.

Biopsies are occasionally necessary to confirm the diagnosis of secondary-stage syphilis, currently achieved by clinico-serological correlation. However, histopathologic examination may offer clues that can lead to the diagnosis of the disease in previously unsuspected or unusual cases. We report the case of a 35-year-old male patient with vitiligo-like lesions for two years, whose diagnosis of syphilis was suggested only after histopathologic examination. Some microscopic aspects observed are discussed and compared to data from the literature.

Thyroid dysfunctions in Sudanese patients with vitiligo

Vitiligo is a chronic acquired skin condition that causes loss of pigment, resulting in irregular pale patches of skin. The precise cause of vitiligo is not fully understood. The autoimmune base of the disease is supported by the frequent observation that several autoimmune disorders particularly thyroid diseases, are associated with vitiligo. To determine the frequency of thyroid dysfunctions in Sudanese patients with vitiligo. Two groups, i.e. vitiligo patients and control, were collected with simple random collection. The control group included individuals free of vitiligo. 5 ml of venous blood was taken from every individual in both groups and the ELISA test was done for thyroid hormones, i.e. T3, T4 and TSH, using the DRG-USA kits. The number of patients with vitiligo in the study was 46, while the control group was 45. Nine [19.56%] patients were found to have abnormal levels of thyroid hormones. No abnormal levels in the control group. Mean T3 level in patients was 1.46 ng/l, while in control group it was 1.467 ng/l. Mean T4 level in patients was 102.761 nmol/l, while in control group it was 90.844 nmol/l. mean TSH level in patients was 0.841 micro IU/l, while in control group it was 1.50 micro IU/l. The t-test was done to determine the significance of difference between means of T3, T4, and TSH between the patients and control groups. The P-values were found to be significant. There is a strong pathogenetic relationship between vitiligo in Sudanese patients and thyroid dysfunctions