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.

Langerhans cell hyperplasia of the skin mimicking Langerhans cell histiocytosis: a report of two cases in children not associated with scabies.

Langerhans cells histiocytosis (LCH) affecting the skin most commonly has clinical and histopathologic diagnostic features. We are reporting two examples of Langerhans cell (LC) hyperplasia recognized in the skin biopsies of two children initially interpreted as LCH. The first was an 8-year-old boy finally interpreted as having an atypical type of contact dermatitis, while the second, an 8-year-old girl, was assumed to have Pytiriasis lichenoides et varioliformis acuta. None showed evidences of scabies. Both presented spongiotic dermatitis with numerous CD1a+ cells. As more cases of LC hyperplasia are recognized, new details emerge helping in the differential diagnosis. Strict clinical-pathologic correlation is suggested in order to avoid misdiagnosis.

Urticaria vasculitis en una niña de cinco años / Urticarial vasculitis in a girl of five years

La urticaria-vasculitis es una forma de vasculitis que se presenta en forma de habones eritematosos clínicamente semejantes a la urticaria hemorrágica de tipo alérgica, pero que histológicamente muestra cambios de vasculitis leucocitoclástica. Puede acompañarse de normocomplementemia o hipocomplementemia. En ambas situaciones puede estar asociada a síntomas sistémicos (como por ejemplo, angioedema, artralgias, dolor abdominal, fiebre, enfermedad pulmonar o renal, epiescleritis y uveitis), aunque en los casos en que se acompaña de hipocomplementemia ha sido vinculada con mayor frecuencia a enfermedades del tejido conectivo. El estudio histopatológico permite diferenciar la urticaria hemorrágica alérgica de la urticaria-vasculitis. Presentamos una niña de 5 años con urticaria-vasculitis y normocomplementemia, con lesiones cutáneas típicas asociadas a artralgias y dolor abdominal.

Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histollogicaly shows leukocytoclastic vasculitis. Urticarial vasculitis may be divided into normocomplementemic and hypocomplementemic variants. Both subsets can be associated with systemic symptoms (for example, angioedema, arthralgias, abdominal or chest pain, fever, pulmonary or renal disease, epiescleritis and uveitis), more often the second variant which has been linked to connective-tissue disease. Histopathological evaluation allows to differentiate between hemorrhagic allergic urticaria and urticarial vasculitis. We report the case of a 5-year-old girl presenting with normocomplementemic uticarial vasculitis, with typical cutaneous lesions associated to arthralgias and abdominal pain.

Linear IgA disease of childhood developing IgA nephropathy.

We report the case of a 10-year-old girl presenting with linear IgA disease of childhood who in the follow-up at age 16 developed hematuria and proteinuria resulting from IgA nephropathy. The combination of linear IgA disease and Berger nephropathy appears to be extremely uncommon but clinical alertness to this association might discover additional cases.

Noninclusion-body infantile digital fibromatosis: a lesion heralding terminal osseous dysplasia and pigmentary defects syndrome.

This report describes the histologic and immunohistochemical features of a peculiar type of digital fibroma that shares some clinical and microscopic features with the more common inclusion-body type infantile digital fibromatosis. However, this type does not exhibit inclusion bodies and its cells are reactive for vimentin but not for actin. Significantly, it presents in combination with a constellation of other clinical findings, i.e., mainly positional and bone abnormalities of the fingers and toes, and skin pigmentary defects. Thus, noninclusion-body digital fibromatosis may represent the first clue for the diagnosis of the so-called terminal osseous dysplasia and pigmentary defects syndrome.

Hamartoma de músculo liso / Smooth muscle hamartoma

El hamartoma de músculo liso, es una lesión cutánea habitualmente congénita, que fue inicialmente descrita bajo otras denominaciones (tumor de músculo liso de la piel, nevo de músculo liso, hamartoma organoide, etc.) En este trabajo presentamos nuestra experiencia con 3 casos, dos de ellos congénitos

Hamartoma de músculo liso / Smooth muscle hamartoma

El hamartoma de músculo liso, es una lesión cutánea habitualmente congénita, que fue inicialmente descrita bajo otras denominaciones (tumor de músculo liso de la piel, nevo de músculo liso, hamartoma organoide, etc.) En este trabajo presentamos nuestra experiencia con 3 casos, dos de ellos congénitos