ABSTRACT
Antecedentes: Los pacientes oncológicos pediátricos pueden desarrollar lesiones dermatológicas secundario a terapia antineoplásica, afectando piel, cabello y uñas. Objetivo: Describir las manifestaciones dermatológicas en los pacientes oncológicos pediátricos hospitalizados. Métodos: Estudio descriptivo transversal llevado a cabo en sala de Hemato- Oncología Pediátrica, Hospital Escuela, período enero-junio 2022. Fueron incluidos 86 pacientes que presentaron manifestaciones dermatológicas durante el período del estudio. Para el análisis se generaron resultados como frecuencias, porcentajes y estadística descriptiva. La proporción de pacientes con manifestaciones dermatológicas se determinó como número total de pacientes con diagnósticos dermatológicos en el período del estudio/número total de pacientes hospitalizados en la sala de Hemato-Oncología Pediátrica multiplicado por 100. Resultados: La edad media fue 9.4 años (DS +/-5.0; rango: 5 meses-18 años). Predominó sexo masculino 62.8% (54/86), procedencia de Francisco Morazán 41.9% (36/86) y ambiente rural 53.5% (46/86). El diagnóstico oncológico fue leucemia linfoblástica aguda 60.5% (52/86), la clasificación fue malignidades hematológicas 67.4% (58/86). El principal fármaco oncológico administrado fue Vincristina 76.7% (76/86). El principal agente antineoplásico administrado fue alcaloides vegetales 75.6% (65/86). El tipo de lesión eritema 38.5% (34/86); la localización de la lesión fue en piel 47.6% (41/86). El diagnóstico dermatológico fue alopecia y mucositis 36.0% (31/86) respectivamente. La proporción de lesiones dermatológicas fue de 65.1% (86/132) y fueron clasificadas como secundarias al tratamiento oncológico 82.6% (71/86). Discusión: La proporción de lesiones dermatológicas fue 65%, siendo las principales manifestaciones alopecia y mucositis, posiblemente secundario a la administración de terapia antineoplásica específicamente alcaloides vegetales, similar a lo reportado en la literatura...(AU)
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Skin Diseases/diagnosis , Mucinosis, Follicular , AlopeciaABSTRACT
Abstract Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.
Subject(s)
Humans , Child , Skin Neoplasms , Carcinoma, Basal Cell , Mucinosis, Follicular , Hamartoma/complications , InflammationABSTRACT
Introduction@#Folliculotropic mycosis fungoides is a rare and aggressive subtype of mycosis fungoides characterized by small to medium-sized malignant T-cells that typically infiltrate the hair follicle. It represents 4% of primary cutaneous lymphomas and less than 10% of patients with MF.@*Case Summary@#We report 2 cases of 80-year old female, who presented with a 3-year history of an erythematous plaque on the left infraorbital area and forehead, and the other with an 8-month history of multiple erythematous indurated plaques on the left cheek, left eyebrow and malar area. Clinical presentation, histopathology and immunohistochemistry findings revealed a diagnosis of folliculotropic mycosis fungoides.@*Conclusion@#Folliculotropic mycosis fungoides has distinct clinical and microscopic features. Evaluation of clinical, pathologic, and immunophenotypic findings are important to identify this rare form of cutaneous T-cell lymphoma.
Subject(s)
Mycosis Fungoides , Lymphoma, T-Cell, Cutaneous , Mucinosis, FollicularABSTRACT
Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.
.Subject(s)
Child , Female , Humans , Facial Dermatoses/pathology , Leprosy, Tuberculoid/pathology , Mucinosis, Follicular/pathology , Diagnosis, Differential , Endemic DiseasesABSTRACT
Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.
Subject(s)
Humans , Male , Adolescent , Skin Neoplasms/pathology , Mycosis Fungoides/pathology , Mucinosis, Follicular/pathology , Mucins/analysisABSTRACT
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, and it rarely exhibits predilection for hair follicle and eccrine gland infiltration. Here, we present 2 similar cases that display folliculotropism with varying amounts of follicular mucinosis, with and without syringotropism. The features observed in both cases were cystic, comedo-like, acneiform lesions; generalized involvement with loss of body hair; pruritus; and hidradenitis suppurativa-like lesions. Hypohidrosis as well as nail and palmoplantar involvement with lichen planopilaris-like clinical features were unique characteristics of the first case. Despite the well-known aggressive behavior of follicular mycosis fungoides, the presented cases had a subtle, slowly progressive, but persistent, clinical course. Folliculotropic and syringotropic mycosis fungoides are variants of cutaneous T-cell lymphoma. Clinical presentations might be challenging, and multiple, deep biopsy specimens containing adnexal structures are required for this critical diagnosis. Aggressive treatment may not be necessary in cases having an indolent course, especially in those with syringotropism.
Subject(s)
Biopsy , Diagnosis , Eccrine Glands , Hair , Hair Follicle , Hidradenitis , Hypohidrosis , Lichens , Lymphoma , Lymphoma, T-Cell, Cutaneous , Mucinosis, Follicular , Mycosis Fungoides , PruritusABSTRACT
La mucinosis folicular es una enfermedad inflamatoria crónica de etiología desconocida, caracterizada histológicamente por el depósito de mucina dentro de los folículos pilosos y las glándulas sebáceas. Se presenta el caso de una paciente de 14 años con diagnóstico de mucinosis folicular primaria con 10 meses de evolución de placas eritematosas infiltradas en mejilla y frente.
Subject(s)
Mucinosis, Follicular , Mycosis Fungoides , Skin NeoplasmsABSTRACT
Follicular mucinosis (FM) is an epithelial reaction pattern that is characterized by the accumulation of mucinous material in the epithelial hair follicle sheath and the sebaceous glands. Although various pharmacological agents have been employed in an attempt to treat FM, effective therapeutic options have remained elusive. We experienced a recalcitrant form of primary FM that we successfully treated with indomethacin and low-dose intralesional interferon alpha (IFN alpha), respectively. To the best of our knowledge, the primary type of FM that responded to indomethacin and low-dose IFN alpha, respectively, in a single case has not been reported in the English medical literature.
Subject(s)
Hair Follicle , Indomethacin , Interferon-alpha , Interferons , Mucinosis, Follicular , Mucins , Sebaceous GlandsSubject(s)
Humans , Male , Adolescent , Mucinosis, Follicular/diagnosis , Mucinosis, Follicular/pathologyABSTRACT
Follicular mucinosis, also known as alopecia mucinosa, is a rare disease characterized by mucinous follicular degeneration and may be accompanied by cutaneous lymphoma, particularly mycosis fungoides. Two categories of disease have been identified in patients with the benign primary form. The first occurs in younger patients with localized lesions and spontaneous regression within 2 years of the onset. The second occurs in elderly patients and shows a chronic relapsing, but benign course. In the second type, the patients are older and have widespread and infiltrated lesions that can progress to mycosis fungoides. We report a case of follicular mucinosis in a 5-year-old girl who had widely distributed lesions. We think that long-term follow-up is mandatory for the occurrence of lymphoma-associated disease.
Subject(s)
Aged , Humans , Follow-Up Studies , Lymphoma , Mucinosis, Follicular , Mucins , Mycosis Fungoides , Child, Preschool , Rare DiseasesABSTRACT
Follicular mucinosis occurring along with angiolymphoid hyperplasia with eosinophils (ALHE) has been described in a 54-year-old female. The patient presented with pruritic erythematous papules on the left frontoparietal scalp. Histopathological examination showed prominent blood vessels in the dermis lined by plump histiocytoid endothelial cells that were surrounded by a dense lymphoid infiltrate with numerous eosinophils; these findings are typical of angiolymphoid hyperplasia with eosinophils. Features of follicular mucinosis were observed in the same section with several hyperplastic follicular infundibula containing pools of mucin in the infundibular epithelium. The concurrent occurrence of these two distinct histopathological patterns in the same biopsy specimen has been described in only three cases to date.
Subject(s)
Angiolymphoid Hyperplasia with Eosinophilia/complications , Female , Humans , Middle Aged , Mucinosis, Follicular/complications , Mucins , Skin/pathologyABSTRACT
Eosinophilic pustular folliculitis (Ofuji's disease) is characterized by inflammatory infiltration of the pilosebaceous units by numerous eosinophils. Clinically, recurrent crops of papules and plaques studded with sterile follicular pustules can be seen. Follicular mucinosis is a reaction pattern which occurs in the follicular epithelium and is characterized by a mucinous degeneration of the outer sheath of follicles and sebaceous glands. The condition can occur in various diseases. Herein, we report a case of a 46-year old woman who presented with a recurrent episode of papules and follicular pustules, revealing inflammatory infiltration in and around the pilosebaceous units and prominent eosinophils, in addition to follicular mucinosis.
Subject(s)
Female , Humans , Middle Aged , Eosinophils , Epithelium , Folliculitis , Mucinosis, Follicular , Mucins , Sebaceous GlandsABSTRACT
La mucinosis folicular es una enfermedad cutánea caracterizada por acúmulo de mucina en los folículos pilosos. Fue descrita por primera vez por Pinkus, en 1957, bajo el nombre de 'alopecia mucinosa'. Se clasifica en: localizada o generalizada, o primaria (idiopática) y secundaria, cuando se relaciona con otras enfermedades de la piel, sobre todo con linfomas de células T. Presentamos una paciente de sexo femenino, de 16 años de edad, con diagnóstico de mucinosis folicular primaria, que respondió muy satisfactoriamente a la terapia con corticoides tópicos, únicamente.
Follicular mucinosis is a cutaneous disease characterized by accumulation of mucine in hair follicles. It was initially described by Pinkus in 1957, as alopecia mucinosa. It could be classified in localized or generalized, or primary (idiophatic) or secondary, when it has relation with other skin diseases, specially with T cells lymphomas. We report the case of a 16 year-old woman with diagnosis of primary follicular mucinosis and who responded satisfactorily to topical corticoid alone.
Subject(s)
Humans , Female , Adolescent , Mucinoses , Mucinosis, FollicularABSTRACT
We report a case of alopecia mucinosa in a 9-year-old girl. She presented with several papules on an erythematous patch on the right side of the ala nasi, a hairless nodular lesion on the same side of the nasal canal. Histopathologic findings obtained from the nasal canal revealed typical features of alopecia mucinosa, such as reticular degeneration, cystic space formation and mucin deposition in the pilosebaceous follicle. The case displayed no evidence or clinical features of any malignant changes, and we suspect the case to be an acute form of alopecia mucinosa.