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M Larralde

Publications and source records attributed to M Larralde.

13 recordsLinked to original sources
PubMed1999

Congenital self-healing histiocytosis (Hashimoto-Pritzker).

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. METHODS: Twelve patients with CSHLCH were seen from 1989 to 1998. RESULTS: Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. CONCLUSIONS: Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.

Antigens, CD1↗
PubMedDate not supplied

Lymphedema as a postulated cause of cutis verticis gyrata in Turner syndrome.

Unusual skin lesions were present at birth in four infants with Turner syndrome. The skin changes in these patients appear to have resulted either from in utero entrapment or pinching of edematous skin or from redundant skin remaining after in utero resolution of lymphedema. Distention by lymphedema is thought to cause several of the phenotypic characteristics seen in patients with Turner syndrome, including nuchal webbing and nail changes. In three of these patients the clinical appearance of the skin changes was similar to cutis verticis gyrata, marked by fixed thickened plaques in folds.

Female↗
PubMedDate not supplied

Eosinophilic pustular folliculitis in infancy: report of two new cases.

Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features. We describe two patients who had recurrent episodes of pruritic follicular papular and pustular lesions on the face, extremities, and trunk. The eruptions lasted for 1 month with intermittent remissions. Laboratory tests disclosed no infectious or parasitic etiology in patient 2. In patient 1 we isolated methicillin-resistant Staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). Histologically both of our patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Their lesions responded well to topical corticosteroids.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Pseudo-Kaposi sarcoma with arteriovenous malformation.

Pseudo-Kaposi sarcoma with vascular malformation (Stewart-Bluefarb syndrome) is an uncommon and important entity characterized by congenital arteriovenous malformation and skin lesions that may resemble Kaposi sarcoma. This is usually seen in the lower limb of young people. We report a case of this syndrome in a 17-year-old boy who had skin lesions on the right leg and foot.

Adolescent↗
PubMedDate not supplied

Juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

Biopsy↗
PubMedDate not supplied

Disseminated cutaneous eruption after BCG vaccination.

We present nine infants (3 to 10 months of age) with numerous small, papular, papular-lichenoid, and papulo-pustular lesions predominantly on the upper and lower limbs associated with local (axillary) lymphadenopathy which appeared after BCG vaccination. Histopathology of the lesions showed small tuberculoid granulomas mainly in the papillary dermis. The presence of BCG bacillus was demonstrated in five out of seven samples from the lymph nodes after culture and in one skin biopsy specimen. All cases, whether treated or not, evolved to complete resolution of the skin lesions. We believe that this peculiar association results from hematogenous spread of the bacillus, which regresses after an adequate immune system reaction.

BCG Vaccine↗