RESUMO
Pseudoxanthoma elasticum (PXE) is an inherited disorder characterized by degradation and fragmentation of elastic fibers and calcium depos- its in the dermis. It clinically manifests as yellow papules or plaques in a cobblestone distribution or "plucked-chicken skin" appearance on the lateral neck and/or flexural areas. In addition, it can also affect the eyes, cardiovascular, and gastrointestinal systems. It is considered as the prototype of ectopic heritable mineralization disorders, usually diagnosed in the second decade of life. The majority of patients are sporadic but recessive, but pseudodominant autosomal forms have been described as well. Mutations affecting the ATP-binding cassette subfamily C member 6 (ABCC6) gene or gamma-glutamyl carboxylase (GGCX) gene lead to PXE. Accumulating evidence in the literature has found that numerous disorders may demonstrate cutaneous PXE-like clinical and/or histologic features without any other systemic evidence of PXE or any genetic documentation of inherited mutations. In this review, we aimed to highlight all the disorders that were reported to exhibit PXE-like clinical and/or microscopic changes and to discuss possible underlying mechanisms leading to such an overlap.
Assuntos
Pseudoxantoma Elástico , Humanos , Pseudoxantoma Elástico/genética , Pseudoxantoma Elástico/diagnóstico , Pseudoxantoma Elástico/patologia , Mutação , Proteínas Associadas à Resistência a Múltiplos MedicamentosAssuntos
Articulações dos Dedos , Humanos , Feminino , Articulações dos Dedos/patologia , AdolescenteAssuntos
Articulações dos Dedos , Humanos , Feminino , Articulações dos Dedos/patologia , AdolescenteRESUMO
Cutaneous fungal infections affect millions around the world. However, severe, multi-resistant fungal infections are increasingly being reported over the past years. As a result of the high rate of resistance which urged for drug repurposing, statins were studied and found to have multiple pleiotropic effects, especially when combined with other already-existing drugs. An example of this is the synergism found between several typical antifungals and statins, such as antifungals Imidazole and Triazole with a wide range of statins shown in this review. The main mechanisms in which they exert an antifungal effect are ergosterol inhibition, protein prenylation, mitochondrial disruption, and morphogenesis/mating inhibition. This article discusses multiple in vitro studies that have proven the antifungal effect of systemic statins against many fungal species, whether used alone or in combination with other typical antifungals. However, as a result of the high rate of drug-drug interactions and the well-known side effects of systemic statins, topical statins have become of increasing interest. Furthermore, patients with dyslipidemia treated with systemic statins who have a new topical fungal infection could benefit from the antifungal effect of their statin. However, it is still not indicated to initiate systemic statins in patients with topical mycotic infections if they do not have another indication for statin use, which raises the interest in using topical statins for fungal infections. This article also tackles the different formulations that have been studied to enhance topical statins' efficacy, as well as the effect of different topical statins on distinct dermatologic fungal diseases.
Assuntos
Antifúngicos , Dermatomicoses , Inibidores de Hidroximetilglutaril-CoA Redutases , Humanos , Antifúngicos/farmacologia , Antifúngicos/administração & dosagem , Inibidores de Hidroximetilglutaril-CoA Redutases/administração & dosagem , Inibidores de Hidroximetilglutaril-CoA Redutases/farmacologia , Dermatomicoses/tratamento farmacológico , Administração Cutânea , Reposicionamento de Medicamentos , Interações MedicamentosasRESUMO
In dermatology, the role of dietary modifications as a means to manage or prevent skin disorders has recently gained special attention among patients and physicians. This is especially true for the currently popular ketogenic diet (KD), which comprises low carbohydrate, high fat, and adequate amount of protein. Recent evidence from basic science research, small clinical trials, population studies, and reports has presented promising potential role of KD as a supplementary or adjuvant treatment in different cutaneous disorders mainly due to its anti-inflammatory properties. This review is directed at raising awareness among dermatologists on the potential uses of KD in managing skin disorders, such as acne, psoriasis, and hidradenitis suppurativa, among others. In addition, cutaneous adverse reactions, such as prurigo pigmentosa and nutritional deficiencies, which have been associated with KD, are also discussed in this review.
Assuntos
Acne Vulgar , Dieta Cetogênica , Hidradenite Supurativa , Psoríase , Humanos , Dieta Cetogênica/efeitos adversos , PeleAssuntos
Atrofia , Extremidade Inferior , Adulto , Feminino , Humanos , Extremidade Inferior/fisiopatologiaRESUMO
Annular lichenoid dermatitis of youth (ALDY) is a newly described controversial benign lichenoid inflammatory cutaneous disorder often characterized by annular patches with hypopigmented center and surrounding erythematous border. Primarily, it affects the trunk and groin of young patients. Since its first description in 2003, additional patients have been reported, leading to better characterization of the entity; nevertheless, the pathogenesis is still unclear, and several hypotheses have been provided about possible triggering or causative factors. It tends to follow a chronic course, with some lesions spontaneously remitting, while others may be persistent or recur post-treatment. No standard validated treatment has been indicated so far for this disorder. Commonly prescribed topical treatment includes corticosteroids and calcineurin inhibitors with variable response.
Assuntos
Erupções Liquenoides , Neurodermatite , Humanos , Adolescente , Erupções Liquenoides/diagnóstico , Erupções Liquenoides/etiologia , Erupções Liquenoides/terapia , Pele/patologia , Neurodermatite/diagnóstico , Diagnóstico Diferencial , Administração CutâneaRESUMO
Erosive pustular dermatosis (EPD) is a rare entity, but it is generally overlooked or missed, rather than rarely encountered. It presents with erosions and shallow ulcers, accompanied by delayed healing and associated with cutaneous atrophy, rather than pustules. It exhibits predominance for women, with a predilection for a chronically sun-damaged scalp and, less commonly, the extremities, particularly the legs, as well as the face and mucosal surfaces. The role of infection, actinic damage, trauma, hormones, autoimmune disease, cutaneous atrophy, and genetics in the pathogenesis of EPD has been described in literature. Increased awareness and a high index of suspicion permit prompt treatment with topical corticosteroids, with or without oral zinc, followed by maintenance therapy with topical calcineurin inhibitors. Prevention, prior recognition, and prompt treatment are required for addressing this complex condition. (SKINmed. 2023;21:12-19).
Assuntos
Dermatoses do Couro Cabeludo , Dermatopatias Vesiculobolhosas , Humanos , Feminino , Couro Cabeludo/patologia , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/tratamento farmacológico , Dermatopatias Vesiculobolhosas/etiologia , Glucocorticoides/uso terapêutico , Cicatrização , Atrofia/complicações , Dermatoses do Couro Cabeludo/diagnóstico , Dermatoses do Couro Cabeludo/tratamento farmacológicoRESUMO
The term 'inherited ichthyosis' refers to a heterogeneous group of mendelian disorders of cornification that involve the integument with varying degrees of scaling. The management of ichthyosis poses a challenge for most physicians. Treatment options proposed in the literature include moisturizers, topical keratolytics, topical and systemic vitamin D analogues, and topical and systemic retinoids; however, some of these modalities are less reliable than others. Despite the therapeutic impasse imposed by the options above, the emergence of pathogenesis-based treatments along with novel gene therapies appear promising and hold the potential to halt or even revert disorders that arise from single genetic mutations, although research is still quite lacking in this domain. Hence, this review aims to highlight the various treatment modalities available for the management of the cutaneous manifestations of non-syndromic inherited ichthyosis, with an added emphasis on pathogenesis-targeted therapies.
Assuntos
Ictiose , Humanos , Ictiose/genética , Ictiose/terapia , Ceratolíticos/uso terapêutico , Mutação , Retinoides/uso terapêutico , Vitamina DRESUMO
ABSTRACT: Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) can often demonstrate clinical and histopathologic overlap. A recent study demonstrated significant plasmacytoid dendritic cell (pDC) recruitment in lesions of PLEVA, whereas another study reported minimal pDC recruitment in lesions of LyP. To confirm the possible diagnostic value of pDCs in differentiating PLEVA and LyP, we compared the presence and distribution of pDCs and myxovirus protein A (MxA) expression (an indirect assessment of pDC activity). In total, 19 cases of PLEVA (16 patients) and 14 cases of LyP (11 patients) were examined using immunohistochemical stains for anti-blood-derived dendritic cell antigen-2 and MxA. Individual semiquantitative scoring systems were used to assess the immunohistochemical results, and a Mann-Whitney test with a subsequent 2-tailed P test was performed for statistical analysis. No statistically significant difference in the number of pDCs in both groups was found. However, most PLEVA cases (84%) demonstrated intense and diffuse MxA expression, whereas LyP cases (71%) demonstrated weak patchy staining (P < 0.007). Our study suggests that although additional studies may be needed to determine whether pDCs are more relevant to the pathogenesis of PLEVA or LyP, pDC activity through MxA staining may play a role in differentiating PLEVA from LyP and may serve as a platform for additional studies.
Assuntos
Células Dendríticas/patologia , Papulose Linfomatoide/patologia , Pitiríase Liquenoide/patologia , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Estudos RetrospectivosAssuntos
Eritema Endurado , Vacina BCG/efeitos adversos , Humanos , Imunoterapia/efeitos adversos , PeleRESUMO
Eosinophilic ulcer of the oral mucosa is a benign, reactive, self-limited lesion. Its pathogenesis is unclear, and it clinically manifests as a punched-out ulcer with surrounding indurated border, raising suspicion for a possible malignancy. On histology, an acute and chronic dense inflammatory infiltrate can be appreciated, which can extend to the deep underlying tissue (including skeletal muscle). Eosinophils are especially conspicuous on hematoxylin and eosin staining and might play an important role in the development of the ulcer. Removal of possible triggers followed by monitoring and possible biopsy are among the initial recommendations. Most lesions heal spontaneously without the need for intervention. This review aims at assisting dermatologists to easily recognize this entity, avoiding unnecessary interventions and apprehension.
Assuntos
Eosinofilia , Úlceras Orais , Amarelo de Eosina-(YS) , Eosinofilia/diagnóstico , Eosinofilia/etiologia , Eosinofilia/terapia , Hematoxilina , Humanos , Mucosa Bucal/patologia , Úlceras Orais/etiologia , Úlceras Orais/terapia , Úlcera/complicações , Úlcera/patologiaRESUMO
Children with genodermatoses are at an increased risk of developing behavioural disorders which may impart lasting damage on the individual and their family members. As such, early recognition of childhood mental health disorders via meticulous history taking, thorough physical examination, and disorder-specific testing is of paramount importance for timely and effective intervention. If carried out properly, prompt psychiatric screening and intervention can effectively mitigate, prevent or even reverse, the psychiatric sequela in question. To that end, this review aims to inform the concerned physician of the manifestations and treatment strategies relevant to the psychological sequelae of genodermatoses.
Assuntos
Transtornos Mentais , Dermatopatias Genéticas , Criança , Humanos , Transtornos Mentais/diagnóstico , Transtornos Mentais/etiologia , Transtornos Mentais/psicologia , Dermatopatias Genéticas/complicaçõesRESUMO
The genital skin may be affected by a variety of dermatoses, be it inflammatory, infectious, malignant, idiopathic, or others. The red scrotum syndrome is characterized by persistent erythema of the scrotum associated with a burning sensation, hyperalgesia, and itching. Its cause is unknown, but proposed mechanisms include rebound vasodilation after prolonged topical corticosteroid use and localized erythromelalgia. The condition is chronic, and treatment is often difficult. Here we review the etiology, the physical and histopathologic findings, and the management of this condition. We also describe related conditions such as red scalp syndrome, red ear syndrome, and red vulva syndrome. Finally, we summarize the different cases reported in the literature and discuss the features that help in the differentiation of red scrotum syndrome from its mimickers.