Congenital atrichia with papular lesions.

Congenital atrichia with papular lesions is a rare, autosomal recessive and irreversible form of total alopecia of the body hair characterized by hair loss soon after birth and the development of keratinfilled cysts or horny papules over extensive areas of the body. The condition is associated with a mutation of the human hairless gene on chromosome region 8p12. We report a 1-year-old boy presenting with the absence of scalp and body hair since birth. On examination, he had complete absence of hair on the scalp, eyebrows, and eyelashes. Multiple, discrete, pearly-to-skin-colored papules of 1-3mm in size were present over the scalp. The skin biopsy from a scalp papule revealed normal overlying epidermis with multiple keratin cysts and hypoplastic hair follicles in the upper dermis.

Rare Vesiculopustular Eruptions of the Neonatal Period.

Numerous disorders present with vesiculopustular eruptions in the neonatal period, ranging from benign to life-threatening. Accurate and prompt diagnosis is imperative to avoid unnecessary testing and treatment for benign eruptions, while allowing for adequate treatment of potentially fatal disorders. In this review, we highlight several rare blistering diseases of the newborn. A diagnostic approach is outlined to provide clinicians with a framework for approaching a neonate with vesicles, pustules, or ulcers.

Congenital atrichia with papular lesions: a rare cause of irreversible childhood alopecia.

Congenital atrichia with papular lesions (APL) is a disease characterized by the complete absence of hair from the whole body occurring within a few months of birth and the presence of papules distributed over the body. A 9-year-old boy presented with sparse hair over his body and with a history of losing his hair soon after birth. Multiple skin-colored papules were observed over the scalp. A biopsy from a papule showed keratinous cysts in the dermis and the absence of hair follicles. A diagnosis of congenital APL was made after vitamin D-dependent rickets was excluded. This case highlights congenital APL as a cause of total alopecia.

Neutrophilic dermatoses: Kids are not just little people.

Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by an overactive innate immune system with dysregulation of neutrophils without underlying infectious etiology. The major representative conditions discussed are Sweet syndrome; pyoderma gangrenosum; neutrophilic eccrine hidradenitis; palmoplantar eccrine hidradenitis; subcorneal pustular dermatoses; bowel-associated dermatosis arthritis syndrome; and synovitis, acne, pustulosis, hyperostosis, and osteitis. We will also discuss other neutrophilic conditions present almost exclusively in the pediatric population, including congenital erosive and vesicular dermatosis with reticulated supple scarring and the recently described group of autoinflammatory diseases. The clinical characteristics, diagnostic approach, and treatment management in the pediatric and adult population are discussed.

Epidermólisis bullosa: piel de mariposa. A propósito de un caso / Epidermolysis bullosa: butterfly skin. Apropos of a case

La epidermólisis bullosa comprende un grupo heterogéneo de enfermedades ampollosas de la piel y las mucosas, son de origen congénito y hereditario. Hacer el diagnóstico no es difícil si se tiene experiencia dermatológica, pero su clasificación es compleja y para ello se necesita considerar la clínica, la genética, la microscopia y la evaluación de laboratorio. El tratamiento de esta enfermedad es también dificultoso y son necesarias ciertas medidas, para proteger al paciente, evitar la aparición de lesiones y las complicaciones derivadas de ellas. Se describe el tratamiento de estas lesiones en un recién nacido, al que se administraron antibióticos profilácticos y se colocaron vendajes en las lesiones. Se describieron todos los cuidados y recomendaciones para evitar, especialmente los roces y las presiones en estas lesiones, así como las temperaturas altas. Para la confección del presente trabajo se consultaron 18 materiales entre revistas y libros de Pediatría. El caso reportado fue un recién nacido con epidermólisis bullosa atendido en el Hospital Universitario "Dr. Mario Muñoz Monroy" de Colón, Matanzas. Se demostró lo poco frecuente y raro de esta patología para los especialistas del tema (AU).

The epidermolysis bullosa includes a heterogeneous group of bullous skin and mucous diseases of congenital and hereditary origin. Diagnosing them is not difficult if the specialist has dermatologic experience, but their classification is complex and it is necessary to take into account the clinical, genetic and microscopic factors, and the laboratory assessment. The treatment of this disease is also difficult and it is necessary to take certain measures to protect the patient, avoid the onset of lesions and the complications derived from them. The treatment of these lesions in a newborn is described. Prophylactic antibiotics were administered and bandages were put on the lesions. All the cares and recommendations to avoid rubbings and pressures on these lesions, and also the high temperatures, are described. To develop the current term, 18 materials (journals and pediatric books) were consulted. The reported case was the case of a newborn with epidermolysis bullosa attended in the University Hospital "Dr. Mario Muñoz Monroy" of Colon, Matanzas. It was demonstrated the low frequency and rarity of this pathology for the specialists of the theme (AU).

Congenital erosive and vesicular dermatosis with reticulated supple scarring.

Congenital erosive and vesicular dermatosis with reticulated supple scarring is a rare cutaneous disorder of unknown aetiology first described by Cohen and colleagues in 1985. It classically presents in the neonate as erosions and vesicles that heal within the first months of life, with supple scarring of a reticulated pattern. Along with a review of the literature, this article presents two atypical cases of congenital erosive and vesicular dermatosis with reticulated supple scarring. Patient one presented with neither erosions nor vesicles at birth, yet continued to experience sporadic blistering at the age of 15 months and patient two is the second documented case to develop congenital erosive and vesicular dermatosis after birth.

Blistering diseases in neonates.

PURPOSE OF REVIEW: Neonatal blistering diseases are rare yet potentially fatal. Therefore, it is crucial for clinicians to know its broad range of differential diagnoses. This review discusses the recent literature on the causes and the most appropriate clinical approach to neonatal blistering diseases. RECENT FINDINGS: Neonatal infections are the commonest causes for neonatal blistering. On the other hand, autoimmune blistering diseases are extremely rare with the literature limited to case reports and one systematic review only. Inherited genodermatoses are also rare, with recent developments in epidermolysis bullosa classification. SUMMARY: In conclusion, as neonatal infections are the commonest cause for blistering, any neonate with blistering should have their blister fluid investigated for infection, while an antimicrobial should be initiated early. Autoimmune blistering diseases should be considered in neonates with a maternal history of autoimmune blistering disease. Although pemphigus and bullous pemphigoid have overall good prognoses, linear IgA bullous dermatoses has a poor prognosis and requires aggressive treatment. Inherited genodermatoses should be suspected when there is a family history of genodermatoses or consanguinity. In this case, the clinician should not hesitate to seek dermatology advice, perform a skin biopsy and consider genetic testing.

Bilateral canalicular and nasolacrimal duct obstruction in congenital erosive and vesicular dermatosis: a case report and review of the literature.

We report the ocular findings in a 2.5-year-old girl with a history of congenital erosive and vesicular dermatosis at birth. We highlight the complexity of the associated nasolacrimal duct obstruction with canalicular scarring and review the ocular manifestations of this rare disease.

Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features.

BACKGROUND: Congenital erosive and vesicular dermatosis (CEVD) healing with reticulated supple scarring, a condition usually observed in premature neonates, presents at birth with vesicles and erosions. Lesions typically heal within a few months, leaving behind scarring with a distinctive supple and reticulated texture. OBJECTIVES: We sought to merge existing literature with new cases to further define CEVD. METHODS: We analyzed 19 previous reports of CEVD and added 9 additional patients; we identified unifying characteristics of this cohort. RESULTS: In 28 total cases, notable features included: preterm birth (79%), nail abnormalities (46%), hyperthermia/hypohidrosis (46%), a history of maternal chorioamnionitis (43%), alopecia (43%), neurodevelopmental and ophthalmologic abnormalities (36% each), tongue atrophy (29%), or a combination of these. Patients with CEVD may be prone to postnatal herpetic superinfections. Previously unreported findings included: erosive lichen planus, digital tip gangrene, and hydronephrosis. LIMITATIONS: The small patient sampling makes it difficult to define diagnostic criteria. As certain findings are associated with prematurity, it is unclear to what extent these features result from CEVD, premature birth, or another intrauterine pathology. CONCLUSIONS: Although rare, CEVD should be considered in the differential diagnosis of neonatal vesicles/erosions in the context of a negative infectious workup. This review strengthens the spectrum of CEVD features, thus facilitating its recognition by clinicians.

Dermatosis congénita vesicular y erosiva con cicatrices reticuladas. Presentación de un caso y revisión de la literatura / Congenital erosive and vesicular dermatosis healing with reticulated supple scarring. A case report and review of the literature

La dermatosis congénita vesicular y erosiva con cicatrices reticuladas es un raro desorden cutáneo de etiología desconocida con lesiones evidentes al nacimiento. Presentamos una niña con esta condición, la cual representa el primer caso diagnosticado en la Argentina, junto a una revisión dela literatura.

Congenital erosive and vesicular dermatosis healing with reticulated, supple scarring is a rare cutaneouscondition of unknown etiology. It presents with patchy or generalized erosion and vesiclesrecognizable at birth, that heal with reticulated scarring. We report a female child with thiscondition, the first recognized in Argentina, together with a review of the literature.

Congenital erosive and vesicular dermatosis with reticulated scarring in a newborn: an innovative treatment using a silicone dressing.

Congenital erosive and vesicular dermatosis healing with reticulated supple scarring is a rare entity presenting in the newborn with crusted erosions and vesicles that heal relatively rapidly, forming unique reticulated scars. We report the case of a premature baby 31 weeks old. Diagnosis was confirmed by skin biopsies, and the clinical improvement was excellent, with complete healing observed within 7 weeks. This report highlights clinical and histopathologic features, and a new successful treatment approach using a silicone dressing.