Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome.

The typical rash of Still disease is an asymptomatic, salmon-coloured, macular, or maculopapular eruption that appears along with the fever spikes and fades when the body temperature drops. Although not included in the diagnostic criteria, there are other frequent, persistent, pruritic and polymorphic skin manifestations of Still disease that have distinctive clinical features and specific histological findings. Among these atypical persistent pruritic eruptions (PPEs), periorbital erythema and linear flagellate papules and plaques can resemble the cutaneous manifestations of dermatomyositis (DM).

Lipedematous scalp. A rare entity in the pediatric ageLipedema de cuero cabelludo: una entidad infrecuente en pediatría.

Lipedematous scalp is an uncommon entity of unknown etiology, rarely described in the pediatric age. It is characterized by boggy thickening of the scalp predominantly located at the vertex and occiput, which acquires a cotton-like consistency. This condition is palpable rather than visible.

[Solitary morphea profunda: An unusual form of localized scleroderma in childhood. Case report].

Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement.

Aplasia cutis congénita con defecto óseo subyacente: evolución favorable tras manejo conservador.

La aplasia cutis congénita es una rara alteración caracterizada por la ausencia de áreas localizadas de piel en el momento del nacimiento. Suele manifestarse como una lesión solitaria localizada principalmente en el cuero cabelludo, que puede estar asociada con otras malformaciones congénitas. Las complicaciones pueden ser fatales, por lo que es necesario un tratamiento individualizado que vendrá determinado por el tamaño, localización y grado de afectación de estructuras subyacentes.

Usefulness of ultrasonography in the diagnosis of subcutaneous granuloma annulare.

Subcutaneous granuloma annulare is a benign inflammatory disorder that primarily affects healthy children and manifests as a rapidly growing, painless, nonmobile mass, most commonly located in the leg. The current case highlights the sonographic appearance of subcutaneous granuloma annulare. Ultrasound color Doppler is a helpful noninvasive imaging test that may allow physicians to confirm the diagnosis of subcutaneous granuloma annulare and avoid unnecessary medical procedures.

Anetodermic pilomatricoma: clinical, histopathologic, and sonographic findings.

Pilomatricoma is a benign cutaneous tumor originatingfrom hair matrix cells. Anetodermic changes inthe skin overlying pilomatricomas are sometimesreported, although their precise mechanisms remainunknown. We present an unusual case of anetodermicpilomatricoma on the upper extremity of a 17-yearoldboy and report its clinical, histopathologic, andsonographic findings.

Papular epidermal nevus with "skyline" basal cell layer (PENS) with extracutaneous findings.

Papular epidermal nevus with "skyline" basal cell layer (PENS), a variant of epidermal nevus, was recently described in otherwise normal children. We describe herein a patient with multiple, typical PENS lesions associated with peculiar facies, bilateral Achilles tendon shortening, and mild psychomotor delay. The association of PENS with extracutaneous manifestations suggests the possibility of a new type of epidermal nevus syndrome, for which we propose the term PENS syndrome.

[Becker's nevus associated with epidermal nevus: another example of twin spotting?].

Linear epidermal nevi are believed to be caused by an autosomal dominant lethal mutation that can only be expressed by mosaicism. Becker's nevus can be explained by paradominant inheritance which is only manifested clinically by an acquired loss of heterozygosity. We present the case of a 16-year-old female with an epidermal nevus located on the left side of the neck, and also a Becker's nevus located on the ipsilateral shoulder.

[Kindler syndrome: presentation of a case].

Kindler syndrome is a very rare disease caused by mutations resulting in defects in the extracellular matrix-actin link. It usually presents with acral blistering from birth in trauma-prone areas, pronounced photosensitivity that improves with age and the development of poikiloderma and cutaneous atrophy. Mucosal involvement and degeneration have been described with relative frequency.

[Prurigo pigmentosa].

Prurigo pigmentosa is an infrequent inflammatory dermatosis of unknown etiology, characterized by recurrent episodes of pruritic erythematous papules which develop into intense reticulated pigmentation. It has been referenced most frequently in young women in Japan. Only thirty cases have been described outside of Japan.