Idiopathic aseptic facial granuloma: A retrospective study of 43 cases.

Background: Idiopathic aseptic facial granuloma (IAFG) is an underrecognized pediatric skin disease, currently considered within the spectrum of rosacea. It usually manifests as a solitary, reddish, asymptomatic nodule on the cheek that resolves spontaneously.

Methods: Retrospective and descriptive observational study of 43 pediatric patients with a clinical diagnosis of IAFG, followed between 2004 and 2022, at two general hospitals in Argentina.

[Recommendations for psoriasis treatment in Pediatrics].

Introduction: in one third of patients with psoriasis, symptoms start during childhood and adolescence, with a strong emotional and psychosocial impact.

Objective: to develop a guideline for the systemic treatment of psoriasis in pediatric patients by means of recommendations based on the best available evidence.

Materials And Methods: Sources: articles indexed in PubMed, Epistemonikos, Google Scholar, Cochrane Library and Scielo, published between January 2010 and May 2022, in English, Spanish and Portuguese.

Early propranolol treatment of infantile hemangiomas improves outcome.

Background: Infantile hemangiomas (IH) are the most common soft tissue tumors of childhood. Although most of these tumors are not worrisome, some IH may be life or function-threatening, can lead to permanent disfigurement, or have associated structural congenital anomalies, requiring early recognition and referral to specialists for treatment consideration. Since 2008, oral propranolol has been widely considered to be the first-line treatment for IH.

Aplasia Cutis Congenita Associated with Fetus Papyraceus.

Aplasia cutis congenita (ACC), or congenital absence of skin, is a heterogeneous condition that may be due to varied mechanisms. ACC has rarely been reported in association with fetus papyraceus, representing a peculiar phenotype of ACC. We present six new cases of neonates born with symmetric ACC associated with intrauterine fetal demise of co-twins during the late first or early second trimester.

Inflammatory myofibroblastic tumor of the tongue: report of an unusual case in a teenage patient.

Inflammatory myofibroblastic tumor is a rare and distinctive lesion composed of myofibroblastic cells accompanied by an inflammatory infiltration of plasma cells, lymphocytes, and eosinophils that mainly affects viscera and soft tissues of children and young adults. It clinically manifests as a mass with rapid development that may occur at almost any site of the body, but predominantly in the lungs or the upper respiratory tract. It rarely arises in the oral cavity with approximately 15 cases reported in that location.

Nevus trichilemmocysticus: report of a new case of a recently recognized entity.

A new type of organoid epidermal nevus, characterized by the presence of multiple trichilemmal cysts disposed in a linear arrangement, was recently recognized and named nevus trichilemmocysticus. We report a 10-year-old girl with a congenital linear dermatosis affecting the head, neck, trunk, limbs, palms, and soles. Initially, the lesions that followed the lines of Blaschko consisted of yellowish plaques covered by verrucous papules and filiform hyperkeratoses.

Neonatal kerion Celsi: report of three cases.

Tinea capitis is a fungal infection caused by dermatophytes, frequent in children but uncommon in the neonatal period. Kerion Celsi is the inflammatory manifestation of tinea capitis secondary to host immunologic responses and its occurrence in newborns is extremely infrequent. We describe three neonates with the diagnosis of kerion Celsi.

Infantile myofibromatosis: report of nine patients.

Infantile myofibromatosis is a rare fibrous tumor of infancy that can be solitary or multiple. Although most of the cases are limited to the skin, in some instances systemic involvement can be present. Solitary tumors limited to the skin usually present a good prognosis with spontaneous regression.

[Fabry disease].

Fabry disease is an uncommon, X-linked lysosomal storage disorder, caused by partial or complete deficiency of the enzyme a-galactosidase A. The defect leads to accumulation of uncleaved globotriaosylceramide on the vascular endothelium and visceral tissues, being the skin, heart, kidneys and central nervous system the most affected organs. We performed review of the literature related to the disease and emphasized that early recognition of angiokeratomas and hypohidrosis are key diagnostic signs of this serious disease.

[Subungual exostosis in a 14 years old boy].

Subungual exostosis is a benign bony outgrowth surrounded by a fibrocartilaginous capsule that mainly affects hallux terminal phalanx with subungual or periungular localization, and that clinically determines variable degree of nail plate or periungular soft tissues alterations, respectively, It is uncommon in childhood and more frequent in females. Clinical suspicion is confirmed by the radiographic finding of a distinct trabecular bone growth arising from the dorsal aspect of the phalanx of the affected finger, on the lateral incidence. Curettage or surgical excision is the treatment of choice.

Eccrine angiomatous hamartoma: report of five congenital cases.

Eccrine angiomatous hamartoma is a rare entity characterized histologically by the combination of proliferative eccrine and vascular elements. It generally arises before puberty, as solitary or multiple lesions, with a heterogeneous clinical appearance, affecting predominantly the distal extremities, with or without associated pain or hyperhidrosis. It may require surgical treatment due to cosmetic concern, progressive enlargement or the presence of pain or excessive hyperhidrosis.

Dyskeratosis congenita--report of a case with emphasis on gingival aspects.

A case of dyskeratosis congenita (DC) of an 11-year-old male is reported. He presented with the characteristic clinical triad of reticular pigmentation of the skin, dystrophic nails and oral lesions, and up to the present he had not developed hematological compromise. Oral lesions consisted of extensive tongue erosions and keratosis, and exuberant gingivitis associated.

Gingival manifestations of orofacial granulomatosis.

Background: Orofacial granulomatosis is a clinical entity presenting with swelling of the facial and/or oral tissues in association with histologic evidence of noncaseating granulomatous inflammation. Labial swelling is the most common finding. Compromise of the gingival and periodontal tissues may occur but has rarely been described in the literature.

Lupus erythematosus: clinical and histopathological study of oral manifestations and immunohistochemical profile of the inflammatory infiltrate.

Background: Lupus erythematosus (LE) is a multifactorial autoimmune disease, which may affect the oral mucosa in either its cutaneous and systemic forms, with varied prevalence.

Methods: Forty-six patients with confirmed diagnosis of LE, presenting oral lesions were included in the study. Oral mucosal lesions were analyzed clinically, their histopathological features were investigated and inflammatory infiltrate constitution was assessed using immunohistochemistry against the following clusters of differentiation: CD3, CD4, CD8, CD20, CD68 and CD1a.

Scurvy in a 10-month-old boy.

We report a 10-month-old boy with inflammatory and necrotic gingival lesions, fever, irritability, and pseudoparalysis of the legs. Laboratory examinations revealed moderate anemia and skeletal X-rays showed osteopenia, scorbutic rosary at the costochondral junctions, and "corner sign" on the proximal metaphyses of the femora. The boy had been fed only with diluted cow's milk.

Polarized light microscopy of hair shafts aids in the differential diagnosis of Chédiak-Higashi and Griscelli-Prunieras syndromes.

Purpose: To study and compare the appearance of hairs from patients with Chédiak-Higashi and Griscelli-Prunieras syndromes under light and polarized light microscopy.

Method: Hairs from 2 Chédiak-Higashi and 2 Griscelli-Prunieras patients were obtained and examined under normal and polarized light microscopy.

Results: Under light microscopy, hairs from Chédiak-Higashi patients presented evenly distributed, regular melanin granules, larger than those seen in normal hairs.

Childhood oral mucous membrane pemphigoid presenting as desquamative gingivitis in a 4-year-old girl.

Mucous membrane pemphigoid is a chronic inflammatory, bullous subepithelial auto-immune disease, with predominant involvement of the mucosal surfaces. Oral mucous membrane pemphigoid occurring in childhood is extremely rare. We describe a new case of this entity occurring in a 4-year-old girl who presented with desquamative gingivitis.

Fabry disease: a study of 6 hemizygous men and 5 heterozygous women with emphasis on dermatologic manifestations.

Objective: To determine the significance of the dermatologic and systemic abnormalities found in 11 patients with Fabry disease (FD) which is an X-linked lysosomal storage disorder caused by the partial or complete deficiency of the alpha-galactosidase A enzyme. This defect leads to the accumulation of uncleaved glycosphingolipids throughout vascular endothelium and visceral tissues.

Design: Case series.