Beard infantile hemangioma and subglottic involvement: are median pattern and telangiectatic aspect the clue?

Background: Identification of patient at risk of subglottic infantile hemangioma (IH) is challenging because subglottic IH can grow fast and cause airway obstruction with a fatal course.

Objective: To refine the cutaneous IH pattern at risk of subglottic IH.

Methods: Prospective and retrospective review of patients with cutaneous IH involving the beard area.

Kaposiform Haemangioendothelioma-spectrum Lesions with Kasabach-Merritt Phenomenon: Retrospective Analysis and Long-term Outcome.

Kasabach-Merritt phenomenon (KMP) is a rare life-threatening vascular condition of infancy. Prognosis factors and long-term follow-up data are lacking. We retrospectively analysed the records of 24 infants (10 females, 14 males) treated for KMP in the Department of Dermatology of Necker-Enfants Malades Hospital, Paris, France, from 1984 to 2012.

Life-threatening hemorrhaging in neonatal ulcerated congenital hemangioma: two case reports.

Importance: Congenital hemangiomas (CHs) are rare benign vascular tumors that differ from common infantile hemangiomas in that they grow in utero and are fully developed at birth. While ulceration is a common, predominantly benign complication in infantile hemangioma, little is known about the prognosis of ulcerated CH. However, it has been observed that ulcerated CH may be complicated by life-threatening bleeding episodes.

Infantile myofibromatosis: a series of 28 cases.

Background: Infantile myofibromatosis (IM) is a rare disorder of fibroblastic/myofibroblastic proliferation in children.

Objectives: We sought to document common and unusual characteristics of patients with IM.

Methods: This was a retrospective study of 28 children diagnosed with histopathologically confirmed IM between 1992 and 2012.

Sirolimus improves pain in NF1 patients with severe plexiform neurofibromas.

Plexiform neurofibromas (PNs) are common and potentially debilitating complications of neurofibromatosis 1 (NF1). These benign nerve-sheath tumors are associated with significant pain and morbidity because they compress vital structures. The mammalian target of rapamycin (mTOR) pathway is a major mediator involved in tumor growth in NF1.

Lymphomatoid papulosis in children: a series of 25 cases.

Background: Lymphomatoid papulosis (LyP) is an uncommon cutaneous T-cell lymphoproliferative disorder (CTLPD) rarely encountered in children.

Objectives: To specify characteristics of paediatric LyP and to describe both diagnostic difficulties and the course of the disease with the experience of 10 years' follow-up.

Methods: This was a retrospective, single-centre study of 25 children diagnosed with LyP according to the 2008 World Health Organization guidelines, and a clinical and pathological correlation by two experts.

Propranolol for treatment of ulcerated infantile hemangiomas.

Background: Ulcerated infantile hemangiomas (IH) are a therapeutic challenge. Propranolol, a nonselective beta-blocker, was recently introduced as a novel treatment for IH.

Objective: To evaluate our experience of propranolol in the management of ulcerated IH.

Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-κB essential modulator gene mutations.

Background: Incontinentia pigmenti (IP) is a multisystem disorder, in which cutaneous symptoms can be accompanied by dental, ocular, and central nervous system defects. In adults, the clinical diagnosis of IP is based principally on the late onset of stage 4 lesions and their association with dental, nail, ocular, or central nervous system anomalies. Nevertheless, these lesions are often unrecognized.

Neonatal rhabdomyosarcoma misdiagnosed as a congenital hemangioma.

Highly vascularized malignant soft-tissue tumors can clinically and radiologically mimic deep hemangiomas. We present a case of congenital rhabdomyosarcoma of the neck, which was initially identified as congenital hemangioma.

Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature.

Background: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood. Histologic tufts of capillaries infiltrating the whole dermis in a "cannonball" distribution pattern associated with dilated lymphatic vessels are characteristic of the disease and confirm the diagnosis. Few case series of TA have been published, and the morphologic structure and evolution of TA seem to vary.

Subcutaneous fat necrosis of the newborn: a systematic evaluation of risk factors, clinical manifestations, complications and outcome of 16 children.

Background: Subcutaneous fat necrosis (SFN) of the newborn is a rare acute transient hypodermatitis that develops within the first weeks of life in term infants. It often follows a difficult delivery. Prognosis is generally good except for the development of hypercalcaemia in severe cases.

Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid.

Background: It is not clear whether bullous pemphigoid (BP) of infancy is linked to maternal transmission of pathogenic autoantibodies. Objectives To search for anti-BP180 antibodies in the sera of infants with BP and their mothers, using sensitive and specific methods.

Methods: Four infants (<6 months) with BP and their mothers were tested for anti-BP180 antibodies by indirect immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA).

Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy.

Background: Plexiform fibrohistiocytic tumor is a soft-tissue tumor of intermediate malignancy occurring in children and young adults but is only rarely found in infants. The tumor usually involves the upper limbs and is slow growing and painless. Recurrence rate is high.

[Hereditary cholestasis, an unusual etiology of pruritus in the infant].

Introduction: Pruritus in the infant is predominantly related to common dermatosis. General causes remain exceptional. We report two cases of pruritus in infants revealing anicteric cholestasis.

Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease.

Background And Aims: Most human and animal cholestatic disorders are associated with changes in hepatocyte cytoskeleton and tight junctions (TJs). These changes are usually secondary and nonspecific phenomena, both in intra- and extrahepatic cholestasis. Recently, missense mutations in TJ protein 2 (ZO-2) have been identified in patients with familial hypercholanemia.

Skin markers of occult spinal dysraphism in children: a review of 54 cases.

Objectives: To verify the diagnostic value of lumbosacral midline cutaneous lesions in asymptomatic children to detect occult spinal dysraphism (OSD) and to propose a practical approach for clinical investigations with respect to the type of cutaneous lesions observed.

Design: Retrospective study of 54 children referred to the Department of Pediatric Dermatology between 1990 and 1999 for congenital midline lumbosacral cutaneous lesions.

Setting: The private or institutional practices of participating dermatologists and pediatricians.

Clinical study of 40 cases of incontinentia pigmenti.

Objective: To analyze the distribution of manifestations in a pediatric cohort and define guidelines for follow-up of incontinentia pigmenti (IP).

Design: Retrospective study of 47 children referred to the Department of Pediatric Dermatology with a diagnosis of IP between 1986 and 1999.

Setting: The private or institutional practice of participating dermatologists and pediatricians.

[Chronic urticaria in children].

Chronic urticaria (more than 6 weeks) in childhood is rare; either the history and the clinical examination are very important to make distinction between "isolated chronic urticaria" and "urticaria associated with systemic diseases". The etiologic factors responsible for "isolated chronic urticaria" are parasitologic, infectious diseases, drugs, physical factors. If those are not present, the question of food responsibility will be discuss.