Faculty Bibliography

Two patients are reported who experienced multiple pilomatricomas beginning in childhood. Although one patient had no evidence of associated diseases, the second patient was diagnosed with myotonic dystrophy subsequent to the onset of the pilomatricomas. Previous reports of multiple pilomatricomas and the association with myotonic dystrophy are reviewed

BACKGROUND--Violence is a public health issue that disproportionately affects the poor. Homelessness, drug abuse, and physical violence are seen with increasing frequency in poor communities. This article reviews the cutaneous manifestations of violence and the dermatologic problems commonly seen in the homeless. Particular emphasis is placed on the experience of municipal hospitals serving the urban poor. OBSERVATIONS--Dermatologic diseases are common in the homeless, and foot-related problems such as cellulitis and pyodermas are frequent causes of hospitalization. Unusual patterns of scarring and bruises in different stages of healing are seen in victims of physical violence. Trauma and sexually transmitted diseases result from sexual abuse. Serious skin infection and self-mutilating scarring are seen in intravenous drug abusers. CONCLUSION--Dermatologists are able to diagnose and treat the many skin problems seen in the poor and to identify the physical manifestations of abuse during routine skin examination. Findings of violence should be documented and reported to the appropriate investigational agencies

BACKGROUND--Various primary dermatoses can affect the vulva of children and be misdiagnosed as child abuse, with devastating social consequences. OBSERVATION--A 3-year-old girl with vulvar erosions was removed from her parent's care after a pediatrician and gynecologist diagnosed child abuse. Histopathologic and immunofluorescence studies were consistent with vulvar pemphigoid. CONCLUSION--Localized pemphigoid should be added to the differential diagnosis of vulvar lesions. Dermatologists, pediatricians, and gynecologists should be familiar with nonvenereal diseases that can exclusively affect this area. The dermatologist's expertise is particularly valuable in differentiating between primary diseases and sexual abuse

BACKGROUND--A small percentage of patients with alopecia areata have connective diseases such as systemic lupus erythematosus, discoid lupus erythematosus, rheumatoid arthritis, and scleroderma. Lupus erythematosus is associated with a number of different types of alopecia, but the incidence of alopecia areata in lupus erythematosus has not been examined. OBSERVATIONS--Of our cohort of 39 patients with lupus erythematosus, alopecia areata developed in 10% (four patients), in contrast to 0.42% of general dermatologic patients. Biopsy specimens of alopecia areata lesions in each of our patients showed continuous granular deposition of IgG at the dermoepidermal junction, a finding usually found in only a minority of alopecia areata cases. Intralesional injections of corticosteroids were effective treatment. CONCLUSIONS--The incidence of alopecia areata in patients with lupus erythematosus is increased. Recognition of this form of alopecia allows for specific therapy with intralesional corticosteroids

Filaggrin is a histidine-rich, basic protein whose name was first proposed based on its ability to aggregate intermediate filaments in vitro. Based on this in vitro observation, it has generally been assumed that filaggrin functions in vivo as a matrix protein which causes keratin filaments to become densely packed in the terminally differentiated cornified cells. Inconsistent with this view however, is the well-known observation that keratin aggregation appears to proceed normally in the affected epidermis of ichthyosis vulgaris patients despite a greatly reduced quantity of filaggrin. To address this issue, we used immuno-electron microscopy to localize filaggrin and its cross-reactive precursor, profilaggrin, in human and mouse epidermis, as well as in ichthyosis vulgaris epidermis. We found that the localization of filaggrin in lower cornified cells correlates precisely with the formation of aggregated keratin filaments, and the disappearance of filaggrin in upper cornified cells correlates precisely with the loosening of keratin filaments. Furthermore, we showed that, even in ichthyosis vulgaris, small amounts of filaggrin/profilaggrin are present as electron-dense deposits associated with keratin filaments in the granular cells, and that the localization of this small amount of antigen again correlates with the aggregation state of keratin filaments. These data strongly suggest that filaggrin is indeed involved in filament aggregation in vivo

The cases of three HIV-positive men with generalized psoriasis and staphylococcal sepsis are reported. In each case the skin appeared to be the source of infection. While the patients received antibiotic therapy, the psoriatic plaques resolved despite minimal or no topical treatment.

Three patients with severe, treatment-resistant cutaneous sarcoidosis were treated with low-dose oral methotrexate on a weekly basis. Facial granulomas and ulcerations responded best. A response was apparent after several weeks of treatment, but 6 to 9 months were required to reach maximal effect