Congenital circumscribed acral hypokeratosis.

Circumscribed acral hypokeratosis (CAH) is a rare dermatosis that typically appears as well-dermarcated erythema with a scaly border on the palms or soles of middle-age patients. A pediatric case of CAH has been recently reported in a 10-year-old boy affecting the medial foot since birth. In this report we describe the second congenital case of CAH on the sole.

A congenital case of circumscribed acral hypokeratosis.

Circumscribed acral hypokeratosis is a disorder characterized by areas of erythematous depressed skin with distinct histopathological findings typically found on the palmar and plantar surfaces. Most patients are middle-aged women who report a multiyear history. We present an 10-year-old African American boy who had an asymptomatic, irregularly shaped erythematous lesion on his left medial foot that had been present since birth. A biopsy showed an abrupt, well-demarcated decrease in the thickness of the stratum corneum layer, with no parakeratosis, that was consistent with a diagnosis of circumscribed hypokeratosis. This represents the first pediatric and congenital case of circumscribed hypokeratosis reported. We review the literature and discuss the ramifications of a congenital case on understanding the etiology of circumscribed hypokeratosis.

Verruciform xanthoma: a special epidermal nevus.

Congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) syndrome is a rare X-linked hereditary disorder. Presentation varies from ichthyosiform nevus to complete limb amelia. We present a 17-year-old adolescent girl who presented with a 16-cm exophytic mass of the right foot that had been growing for 7 years as well as knee contracture. Deformed nails with onychorrhexis were noted bilaterally. History of multiple nonlinear erythematous skin lesions covered by dry waxy scales involving multiple body folds with sharp midline demarcation was obtained. The patient's family history was negative for consanguinity and similar conditions. Radiography showed right leg hypoplasia and osteopenia. These findings fulfill the diagnosis of CHILD syndrome. Microscopically, psoriasiform epidermal hyperplasia with marked orthohyper-keratosis and neutrophilic exocytosis were noted. The papillary dermis was packed with foamy macrophages consistent with xanthomatous changes, namely verruciform xanthoma. Verruciform xanthoma, although rarely found in other conditions, is a characteristic finding.

Nevo «spilus» congénito plantar / Foot congenital nevus spilus

El nevo spilus, también denominado nevo lentiginoso moteado, se define como una mácula café con leche, con presencia de máculas y pápulas hiperpigmentadas distribuidas de forma irregular en la superficie. Suele comenzar durante la primera infancia, y con menor probabilidad es congénito. Estas lesiones pueden malignizarse, por lo que se debe realizar un seguimiento. Presentamos el caso clínico de una niña de 4 años de edadc on un nevo spilus congénito en una localización inusual en el talón izquierdo (AU)

Nevus spilus, also known as speckled lentiginous nevus, is characterized by a café-au-lait macule, with the presence of hyper pigmented macules and papules irregularly distributed on the surface. It usually starts during the first childhood, and with less probability it is a congenital lesion. These lesions can turn malignant, so they have to be followed-up. We present a case report of 4-year-old child with a congenital nevus spilus on an unusual localization on the left heel (AU)

Precalcaneal congenital fibrolipomatous hamartoma.

Precalcaneal congenital fibrolipomatous hamartoma (PCFH) is a condition that is seldom reported in the paediatric literature and rarely in the dermatological literature. It has other disparate and confusing names and as histology is rarely obtained, the condition may be under-reported. We describe this disorder in the male twin of a pair of nonidentical twins.

Autosomal dominant precalcaneal congenital fibrolipomatous hamartoma.

Precalcaneal congenital fibrolipomatous hamartoma is a rare minor malformation previously reported only as sporadic. We report this finding in three family members from two generations, suggesting autosomal dominant inheritance.

Dermoscopy of an acral congenital melanocytic nevus.

Congenital melanocytic nevi carry a risk for malignant transformation into melanoma, therefore early detection of suspicious features is crucial to reduce mortality rates. Dermoscopy improves the early detection of melanoma while reducing the number of unnecessary excisions of benign pigmented skin lesions. Dermoscopically, congenital melanocytic nevi are often characterized by the presence of a cobblestone pattern, but to date, little is known about the dermoscopic features of acral congenital melanocytic nevi. We report an acral congenital melanocytic nevus typified by the presence of three different dermoscopic patterns that are commonly seen in acquired melanocytic nevi of palms and soles.

Precalcaneal congenital fibrolipomatous hamartomas: report of occurrence in half brothers.

Precalcaneal congenital fibrolipomatous hamartomas are uncommon, congenital, nontender papules located on the medial plantar aspects of the heel. We report the occurrence of this rare disorder in two half brothers, suggesting that it may occur in a familial pattern.

Precalcaneal congenital fibrolipomatous hamartoma: a report of four cases.

Four infants had soft, skin-colored nodules in the midline plantar region of the heels since birth. The lesions were asymptomatic, bilateral, and symmetric, and measured approximately 1 cm in diameter. Their size increased in proportion to the growth of the child. In three infants the lesions persisted at 1 year of age, while in the fourth they remained at age 12 years. The nodules were not associated with any other disease or abnormality. A review of the literature revealed only one Argentinean series comprising four cases, and two communications at international pediatric congresses by French and American authors who reported five and three cases, respectively. We describe four additional cases of precalcaneal congenital fibrolipomatous hamartoma and comment on their histopathologic and ultrastructural characteristics.