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E Ermacora

Publications and source records attributed to E Ermacora.

32 records · Page 2Linked to original sources
PubMed1987

[Cutis marmorata telangiectatica congenita. 4 new cases and review of the literature].

Cutis marmorata telangiectatica congenita (CMTC) is characterized by a persistent vascular mottling of the skin, usually on the limbs. Four cases (2 males and 2 females) followed up for varying lengths of time are reported. In the first patient, whose vascular lesions were mild, careful re-examination after 10 years revealed some scars. In the second patient the lesions were located on the right side of the body, notably on the leg; 6 years later atrophic scars and a brownish plaque were visible on that leg. In the third patient the lesions occupied almost the entire body, although one side was more affected than the other and hypotrophic; the child's face was asymmetrical because of hypoplasia of the jaw and curvature of the nose; after 1 year the patient's general condition was satisfactory and the skin lesions were less evident but still present. In the fourth patient hypoplasia of a lumbar vertebra was discovered; the skin lesions were similar to those observed in the third patient. Examination of the deep vessels, performed whenever possible, did not show any abnormality, but the possibility of future vascular defects, such as varicosities, must be considered. While some authors emphasize the functional character of the disease, in our opinion the high frequency of multiple associated congenital abnormalities makes CMTC not only a vascular disorder but also a syndrome including other neuroectodermal and mesodermal defects. Therefore, any patient presenting with the cutaneous changes typical of CMTC should be examined with this in mind.

Blood Vessels↗
PubMed1986

Long-term follow-up of dermatitis herpetiformis in children.

Dermatitis herpetiformis was diagnosed in seventy-six children by its clinical features and by detection of granular IgA deposits in the papillary dermis of perilesional skin. Enteric alterations demonstrated by measurement of D-xylose absorption and by small intestinal biopsies were detected in over 90% of all patients. A complete follow-up was obtained for all children who were followed for 3 to 10 years. Treatment with a gluten-free diet alone led to a reversal of the intestinal abnormality in 100% of our children and to the disappearance of cutaneous lesions in 82% of reported cases. This suggests that a complete remission of symptoms can be obtained with a gluten-free diet alone. Dapsone alone is effective therapy for the rash but does not affect the intestinal alterations. Furthermore, the lack of side effects to dietetic therapy for a long period of time makes the diet the treatment of choice in this disease.

Child↗
PubMed1986

Benign cephalic histiocytosis.

Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head. Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution. All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.

Female↗
PubMed1975

Erythema gyratum atrophicans transiens neonatale.

An unknown dermatosis was observed in an infant girl. During the newborn period, she had a generalized, patchy, erythematous eruption of about 60 round erythematous patches on the trunk, thighs, head, labial mucosa, and palate. After a few weeks, the lesions became whitish, atrophic, and depressed, and they were surrounded by an erythematous and slightly infiltrated border, which was occasionally lobulated. Histologically, the lesions demonstrated a thin epidermis overlying an edematous dermis. Around the border of the lesions the collagen bundles were infiltrated with mononuclear cells. Granular deposits of IgG, C'3, and C'4 were found at the dermoepidermal junction and around the superficial capillaries. The lesions healed completely and spontaneously within a year.

Diagnosis, Differential↗
PubMedDate not supplied

Multiple familial smooth muscle hamartomas.

Smooth muscle hamartoma is a cutaneous abnormality characterized by a disorganized proliferation of normal muscle fibers of arrector pili. Usually a single congenital hypertrichotic plaque involves the trunk and the extremities. Multiple lesions have rarely been reported in the literature. We describe three members of the same family with multiple skin-colored patches on the back and legs, histologically confirmed as smooth muscle hamartomas. To our knowledge this is the first report of multiple smooth muscle hamartomas in different members of the same family and quite interestingly involving the same skin site.

Adult↗
PubMedDate not supplied

[Prenatal diagnosis of a case of Hallopeau-Siemens recessive dystrophic epidermolysis bullosa].

Fetal dystrophic recessive epidermolysis bullosa of the Hallopeau-Siemens type (EBdr-HS) was prenatally diagnosed in a couple at risk whose previous child was severely affected of the same disorder. Prenatal diagnosis was made at 18 weeks of gestation by ultrastructural examination of fetal skin samples obtained under continuous ultrasonic guidance. Four of the five specimens obtained showed dermal epidermal separation under the lamina densa with athophic and rare anchoring fibrils and superficial collagenolysis. The parents decided to terminate the pregnancy and ultrastructural skin examination of the aborted fetus, performed in multiple sites including the scalp, trunk, abdomen and limbs, confirmed the diagnosis showing no regional variation in the expression of the ultrastructural marker.

Biopsy, Needle↗