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Biomedical subjects

R Grimalt

Publications and source records attributed to R Grimalt.

At least 37 records · Page 2Linked to original sources
PubMed1995

The various clinical patterns of xanthoma disseminatum. Considerations on seven cases and review of the literature.

BACKGROUND: Xanthoma disseminatum (XD) is a rare mucocutaneous xanthoma nowadays classified as a benign non-Langerhans cell histiocytosis. OBJECTIVE: We studied 7 cases of XD with a long follow-up and reviewed the literature in order to compare and discuss previous reports. METHODS: We studied clinical manifestations, course, histopathologic, ultrastructural and immunohistochemical findings of 7 cases of XD followed for 1-20 years. RESULTS: We report our findings and compare our cases with those described in the literature. CONCLUSIONS: XD and Langerhans cell histiocytosis were distinguished by clinical differences, as well as ultrastructural and immunohistochemical methods. Three clinical patterns of XD appeared to emerge in our group of patients and in the cases reviewed from the literature.

Adult↗
PubMed1995

Self-healing juvenile cutaneous mucinosis.

BACKGROUND: Mucinoses represent a puzzling and heterogeneous group of rare diseases, and self-healing juvenile cutaneous mucinosis is an extremely rare disease among them. OBSERVATIONS: A scleroedematous condition of the face, associated with papular lesions and arthropathies, had occurred in a 5-year-old boy 10 days after onset of fever, arthralgia, muscle tenderness, and weakness. RESULTS: Histologic examination revealed an edematous dermis, occupied by mucin. Skin lesions and the joint swellings disappeared spontaneously after 2 months. At the follow-up 5 years later, the patient remains in excellent health. CONCLUSION: Although exceptional, this entity has a well-defined clinical picture, marked by manifestations that are initially worrisome but which, surprisingly, prove to be temporary and benign.

Child, Preschool↗
PubMed1994

An unusual case of non-Langerhans cell histiocytosis.

A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.

Child, Preschool↗
PubMed1994

Pityriasis rotunda: report of a familial occurrence and review of the literature.

Pityriasis rotunda is an uncommon dermatosis characterized by multiple, widely distributed, strikingly circular hypopigmented or hyperpigmented patches that are slightly scaly. It has been described in Oriental and black patients, usually in association with certain infective or malignant systemic diseases. Pityriasis rotunda is rare in white patients and does not act as a marker of malignancy. Our ultrastructural and histologic findings demonstrated that pityriasis rotunda is more closely related to congenital ichthyoses than ichthyosis vulgaris, contrary to previous reports. On the basis of our studies and a review of the literature, it seems that two types of pityriasis rotunda exist with significant prognostic differences.

Adult↗
PubMed1994

Asymmetric periflexural exanthem of childhood: report of two new cases.

A newly described exanthem of infancy consists of an eczematous or scarlatiniform eruption that starts initially from one axillary fold and spreads unilaterally and centrifugally on the trunk and the proximal part of the upper limb. Minor lesions are infrequently present on the contralateral side. The rash may be slightly pruritic and sometimes associated with a moderate regional lymphadenopathy. The appearance in small epidemics and the seasonal prevalence imply a viral agent as the probable origin. We report two new cases of this entity, which appears to be not so rare.

Arm↗
PubMed1994

A case of congenital reticular ichthyosiform erythroderma--ichthyosis 'en confettis'.

We describe the third case of congenital reticular ichthyosiform erythroderma (CRIE), a rare inherited keratinization disorder, the second with the peculiar reticulate skin pattern. The same case had been previously described and defined, for the clinical appearance, as ichthyosis 'en confettis'. An 18-year-old girl was born with the clinical features of an erythrodermic lamellar ichthyosis. Patches of normal skin enclosed by erythematous-ichthyotic skin in a reticular arrangement appeared on the trunk at the age of 10 years, and they enlarged slowly during 6 years. The treatment with etretinate, started 2 years ago, further increased this process. Another peculiar clinical feature is a remarkable hypertrichosis. At the ultrastructural level, perinuclear deposits of filamentous material in vacuolized keratinocytes of the upper epidermis, pathognomonic for CRIE, were demonstrated. This suggests that CRIE and ichthyosis 'en confettis' are the same disorder. In addition the peculiar clinical presentation of this rare genodermatosis develops only during late childhood and puberty. The identification of three sporadic cases only leaves the problem of inheritance still unsolved.

Adolescent↗
PubMed1993

Paederus dermatitis: an easy diagnosable but misdiagnosed eruption.

Paederus dermatitis is a peculiar irritant contact dermatitis characterised by erythemato-bullous lesions of sudden onset on exposed areas of the body. The disease is provoked by an insect belonging to genus Paederus. This beetle does not bite or sting, but accidental brushing against or crushing the beetle over the skin provokes the release of its coelomic fluid which contains paederin, a potent vesicant agent. Due to the pathogenic mechanism, the morphology and location of the dermatitis change from case to case. The lesion usually resembles the accidental dropping of a caustic or hot liquid. The uncommon association of acute dermatitis with minimal or no complaints, which would be noteworthy in the case of chemical or thermal burns, facilitates diagnosis which is corroborated by the season and by the case history.

Acute Disease↗
PubMed1993

Unusual aspects of juvenile xanthogranuloma.

We describe three unusual features of juvenile xanthogranuloma that were observed in three different children. We also describe the mixed and clustered forms of juvenile xanthogranuloma and a giant juvenile xanthogranuloma of the nose.

Female↗
PubMed1993

Spiralled variant of syringomatous carcinoma.

Carcinomas with eccrine differentiation include a complex group of tumors with many different histopathological subtypes. Most of these tumors share some histopathological features, namely, horn cysts and dermal tubular structures. In this work we describe an eccrine carcinoma with a peculiar spiralled pattern suggesting acrosyringeal differentiation.

Carcinoma↗
PubMed1993

Encephalocraniocutaneous lipomatosis: case report and review of the literature.

Encephalocraniocutaneous lipomatosis is a congenital disorder characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. Distinguishing histopathologic features are dysgenesis and neoplasia of the adipose tissue. A Caucasian boy had soft tumors and elastic papules on his head since birth, associated with atrophic areas, and a bilobed lesion on the upper right eyelid. On the bulbar conjunctiva of the right eye, an oval 6-mm lesion was present. Ultrasonogram, computerized tomographic scan, and magnetic resonance imaging revealed a dilation of the right lateral ventriculus, a mass on the pontocerebellar angle, agenesia of the corpus callosum, an arachnoidal cyst on the right hemisphere, microcalcifications, and pachygyria. The histology of a soft cutaneous tumor was consistent with a fibrolipoma, and dispersed extracellular lipid globules in the upper dermis were found on electron microscopy. The diagnosis suggested by these findings was encephalocraniocutaneous lipomatosis. Even in view of the rarity of the syndrome (11 cases described in the literature), this patient seems unusual because of the bilateral distribution of the cutaneous lesions and because of the agenesia of the corpus callosum. The peculiar ultrastructural findings require further confirmation.

Brain Neoplasms↗
PubMed1992

Eruptive vellus hair cysts: case report and review of the literature.

A 6-year-old Caucasian girl had dozens of asymptomatic, flesh-colored, 2- to 5-mm eruptive vellus hair cysts. These papules on the buttocks, thighs, and groin increased in number for three months. Histologic examination revealed poorly defined, keratin-filled cysts in the upper middermis, containing numerous transversely or obliquely cut portions of vellus hair. The histopathologic differential diagnosis with other epithelial cysts containing hair shafts is debated, and new clinical differential diagnoses are proposed. Review of the literature suggests that eruptive vellus hair cyst is not a rare disorder, but its frequency is probably underestimated due to paucity of symptoms. Nevertheless, the clinical relevance of some of the differential diagnoses should convince clinicians to obtain histologic confirmation.

Buttocks↗
PubMed1991

Lichen aureus in childhood.

Lichen aureus is a rare asymptomatic dermatosis of unknown origin that is now classified in the group of pigmented purpuric dermatoses. The eruption consists of asymptomatic, roundish or irregular, lichenoid erythematous-purpuric papules with a tendency to coalesce in patches, most prevalent on the limbs. No meaningful laboratory abnormalities have been found. Histologically, the epidermis is normal, with a lymphohistiocytic, bandlike infiltrate with extravasated blood red cells and hemosiderin deposits observed in the dermis. During the last 20 years we have followed eight new patients, which represent 0.05% of our hospitalized patients. In five of eight patients the disease resolved in two to four years. No therapy has been carried out on these children, since lichen aureus has a tendency for slow, spontaneous improvement and resolution.

Adolescent↗
PubMed1991

Unilateral eruptive psoriasis and lichen striatus.

A 2-year-old boy experienced lichen striatus localized to the left half of the body. Some weeks later he developed a peculiar form of eruptive psoriasis limited to the right half of the body. To our knowledge, unilateral eruptive psoriasis has never been described in the literature. The coincidence in this young patient of lichen striatus and this unusual form of psoriasis arising in so short a period of time leads us to hypothesize a common trigger factor.

Child, Preschool↗