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Biomedical subjects

A H Jacobs

Publications and source records attributed to A H Jacobs.

18 recordsLinked to original sources

Congenital smooth muscle hamartoma. A report of six cases and a review of the literature.

Congenital smooth muscle hamartoma (CSMH) represents a proliferation of randomly oriented dermal smooth-muscle bundles. Six patients with CSMH were observed, the largest series to date, and the literature was reviewed. Congenital smooth muscle hamartoma has presented as congenital patches or slightly indurated plaques with prominent overlying hair (88% of cases), or rarely as patches with perifollicular papules without prominent hair (12% of cases). Most lesions (61% of cases) have been somewhat hyperpigmented, but 39% of cases have been flesh colored. Congenital smooth muscle hamartoma has occurred on the torso and proximal extremities, except for one case on the eyebrow and eyelid (present study). A positive pseudo-Darier's sign (temporary induration or piloerection after rubbing) helped to differentiate CSMH from congenital hairy nevo-cellular nevus. Congenital smooth muscle hamartoma is a distinct entity that is at one end of a spectrum that includes Becker's nevus, and should be considered in the differential diagnosis of any congenital hairy lesion.

Biopsy

Jessner's lymphocytic infiltrate in two girls.

Two girls demonstrated waxing and waning predominantly facial eruptions. Clinically as well as histologically, the lesions are consistent with lymphocytic infiltrate to Jessner and Kanof and constitute the first reported childhood cases. Although primarily a disease of male adults, this entity should be included in the differential diagnosis of facial plaques in children.

Age Factors

Tinea in tiny tots.

Dermatophyte infections in infants and toddlers, with the exception of tinea capitis, are considered rare. We describe representative patients to illustrate the broad clinical spectrum of these infections in this age group. We review possible pathophysiologic mechanisms, diagnostic modalities, and therapeutic regimens. Our experience suggests that involvement of areas other than the scalp may be more common than current estimates suggest. Recognition of affected patients is important in view of the wide differential diagnosis and the efficacy of current treatment.

Antifungal Agents

Infantile psoriasis: a follow-up study.

The appearance of psoriasis in infancy has been questioned by many dermatologists and pediatricians, although childhood psoriasis is a well-recognized entity. To elicit further information on this matter, a follow-up group of nine children who had been diagnosed initially as having infantile psoriasis was reexamined 6 to 13 years later to determine the course of the disease over time. Seven of the nine children had recurrent psoriatic lesions and two remained completely clear after the resolution of the initial eruption. The majority did not have a severe disease course; on the contrary, progression of the illness was mild for most of them. Familial aggregation was noted in almost all of the children. In fact, over the intervening years the number of relatives with psoriasis significantly increased. Most patients were not aware of the role of such factors as infections, injury, and stress in precipitating psoriasis. Participation in a self-help workshop is recommended as a way for both parents and children to learn to cope with the clinical and psychologic aspects of the illness.

Age Factors

Immunohistologic patterns of congenital nevocellular nevi.

To establish sensitive histologic criteria for small congenital nevi (SCN), we examined 29 biopsy specimens of SCN from patients younger than age 1 year by serial sectioning and S100 immunoperoxidase staining. The depth of papillary and reticular dermal infiltration was variable; only the results of six biopsy specimens contained nevomelanocytes in the lower third of reticular dermis. However, all cases had focal nevomelanocytic involvement of adnexa at the midreticular dermis or below (26 of 29 cases in eccrine and 15 of 29 in pilosebaceous structures). Follow-up specimens in ten patients were obtained (mean interval, 10.25 years), and no difference in histologic pattern or cytology was observed. There were variable size increases in the surface area of SCN, ranging from no increase to a maximal ninefold increase.

Biopsy

Cutis marmorata telangiectatica congenita (congenital generalized phlebectasia).

Thirteen original examples of cutis marmorata telangiectatica congenita are presented and the English literature is reviewed. CMTC is a benign, distinct congenital cutaneous anomaly manifested by a reticulate vascular pattern at birth, which frequently improves with age, and may be associated with other abnormalities in at least 50% of the patients. Our data suggest that in contrast to previous reports, the sex distribution is equal, and the condition as a whole is more prevalent than previously recognized.

Female

Infantile psoriasis.

We report 14 infants younger than 2 years of age with psoriasis vulgaris in order to call attention to the existence of infantile psoriasis. Three of the patients were noted to have psoriasis in the neonatal period; the onset may have been prenatal in one. In 13 of the 14 patients the site of onset was determined by the isomorphic effect (Köbners phenomenon). Although nail pitting is usually said to be infrequent in childhood psoriasis, 11 (79%) of our patients had typical psoriatic nail changes. Psoriasis must be considered in the differential diagnosis of papulosquamous disorders in infants.

Diagnosis, Differential

Recurring digital fibrous tumors of childhood: a review.

Two new cases that conform to the clinical and histopathological features of recurring digital fibrous tumor of childhood, described by Reye in 1965, are reported. This tumor, which is considered a distinct entity among the juvenile fibromatoses, characteristically presents in infancy and early childhood, involves only the digits, recurs frequently following surgical excision without metastatic spread, and demonstrates the distinct histopathological finding of intracytoplasmic inclusion bodies within proliferated fibroblasts. This tumor is reviewed with reference to clinical features, histopathology, etiological considerations, and management.

Adolescent

Pigmented lesions in newborn infants.

1058 newborn infants were examined. Forty-one (3-9%) had clinically discernible pigmented lesions compatible with melanocytic naevi. Biopsy was performed on thirty-four of the forty-one and of these; eleven, representing 1-01% of the infants, proved to be melanocytic naevi. No giant (garment) naevi were seen in this series. Two of the eleven naevi pathologically examined showed histological changes similar to those that have been reported in some giant naevi, but the remaining nine were not only different from criteria usually assigned to giant naevi, but they also differed from the usual adult naevi, in that most were predominantly junctional. None of the melanocytic naevi in this series showed any suggestion of malignant change. In newborn infants it is often impossible clinically to distinguish naevi from other types of pigmented lesions, as only eleven out of the thirty-four pigmented lesions were melanocytic naevi. Seven of the eleven melanocytic naevi were under 1-5 cm in diameter. No pigmented lesions were found on the palms, soles or genitalia.

California

The incidence of birthmarks in the neonate.

The presence of various types of birthmarks was determined in 1,058 newborn infants under 72 hours of age. Of these, 79.5% were white, 6.2% were black, 11.2% were ladinos, and 2.6% were Asiatic. Mongol spots were present in 9.6% of the white babies, 95.5% of the black babies, 81% of the Asiatic babies, and 70.1% of ladino infants. Pigmented lesions were present in 42 (4%) of the infants. Biopsies obtained in 34 (3.2%) revealed that only one-third (11) of these were melanocytic nevi. Salmon patches were present in 40.3% of the infants, recognizable early strawberry marks in 2.6%, and port-wine strains in 0.3%. In addition to birthmarks, it was determined that 30.3% of the 508 babies examined at one of the two hospitals had toxic erythema of the newborn.

California

Birthmarks.

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Adolescent