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Background: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations., Objective: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies., Methods: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas., Results: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis., Conclusion: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.

Epidermolysis bullosa acquisita (EBA) is an immunologically distinctive disease with characteristic clinical features. These include extreme skin fragility, trauma-induced blisters and erosions, and healing with scars and milia. These clinical features are currently the major means of routinely distinguishing EBA from bullous pemphigoid (BP) and cicatricial pemphigoid (CP). In this study we describe five patients with EBA who presented with or developed clinical as well as histologic and immunohistologic features indistinguishable from those characteristic of BP or CP. At different times during the course of their disease, it closely resembled classic BP or CP and cases of BP or CP with atypical features or evolving features of EBA. To determine how often EBA might present and be misdiagnosed as BP, we reviewed our experience with bullous diseases and screened eighty-five "BP" sera for EBA antibodies. The results of this study show EBA can mimic the clinical, histologic, and immunohistologic features of BP and CP, suggest that a BP-like clinical presentation occurs in as many as 50% of EBA patients, and suggest that 10% of patients referred to medical centers and diagnosed as BP have EBA.

There is an expanding list of syndromes that combine ichthyosis with neuroectodermal and mesodermal defects. We present the case of a young woman with the following features: lamellar ichthyosis, dwarfism, mental retardation, nail and dental abnormalities, unusual facies, poor sexual maturation, punctate cataracts, and hair shaft abnormalities. There were no neurologic or auditory defects. Hair examination revealed pili torti, trichoschisis, bright and dark bands with polarizing microscopy, and a low hair sulfur content. The patient did not have aminoaciduria or immunoglobulin abnormalities. We believe that this case is a link between the ichthyosis-associated syndromes and the group of syndromes that have in common abnormal hair with clean transverse fractures (trichoschisis) and low sulfur content. A more complete examination of the hair in other patients with ichthyosis and associated abnormalities will probably reveal more patients with this new syndrome.

A superficial shave biopsy is described as a simple, essentially painless, more reliable method for the demonstration of the scabies mite, eggs, and feces in human skin.

Generalized hyperpigmentation developed over 2 years in a 65-year-old woman. A diagnosis of pernicious anemia was made, and treatment with vitamin B12 led to complete reversion of her hyperpigmentation to normal. Literature pertinent to hyperpigmentation and its association with megaloblastic anemias is reviewed and several possible mechanisms are mentioned.

Definitions are offered for quality of health care, reasonable costs or fees, an appropriate share of the dermatologic market, and reasonable income or profit for dermatologists. Physician control of these items was almost complete in the recent past, but control is now passing rapidly to government in the form of regulations and to large health care corporations in the form of unbridled competition in the marketplace. Physicians, including dermatologists, must mobilize and unite to retain reasonable controls, especially over quality care and personal income. The Academy (as an organization of all of us) can help in this process, but individual dermatologists at local, regional, and national levels must play major roles in retention of controls.

Most of the world's population is eventually infected with herpes simplex virus type 1, type 2, or both. Most infected persons harbor latent virus in nerve ganglia for the rest of their lives. True primary infections, nonprimary first-episode infections, and recurrent infections can be asymptomatic or clinical, but all of these are contagious. Past estimates of recurrent herpes affecting 20% to 40% of the population are probably low because of inadequate studies of asymptomatic shedding and genital recurrences. Herpes infections are spread primarily by close personal contact but some are contracted by fomites and medical-dental procedures. Latent virus harbored by almost everyone with frequent reactivation to produce symptomatic or asymptomatic, infectious recurrent disease poses a huge problem for control or eradication of herpes simplex infections. Public health measures will probably fail because they require too much alteration of the public's life-styles. So far, antiviral drugs do not eliminate latent virus from infected ganglia. That naturally infected persons can be reinfected, superinfected, or autoinoculated raises serious questions whether vaccines will eradicate the disease, although they might control some serious manifestations and limit some spreading. The problem of eradicating herpes is different from that of eradicating smallpox, where latent infections with infectious recurrences did not exist.

Rapid advances have occurred in the characterization of human papilloma virus (HPV) types applying the new advanced techniques of restriction endonuclease analysis and molecular hybridization to human wart virus. Human papilloma virus can no longer be viewed as a single, homogeneous virus producing all varieties of clinical warts. At least three antigenically heterogeneous HPV types have been associated with common and plantar warts. Two additional HPV types have been found in patients with epidermodysplasia verruciformis. Condylomata acuminata and laryngeal papillomas contain viruses which are also distinct from the preceding viruses and may represent additional HPV types. This antigenic heterogeneity of HPV has important implications concerning the immunology of human warts which have not been taken into account in most previously published studies. Both antibody and cell-mediated responses may be seen in patients with active warts, but many patients with warts have no demonstrable immune reactions. The role of immunity in wart regression remains poorly understood. Nevertheless, the increased frequency of warts in patients receiving immunosuppressive drugs and with immune deficiency states and the immunologic alterations which occur in patients with regressing or cured warts compared to patients with active warts, particularly the increased frequency of cell-mediated responses and antibodies specific for viral antigens, support a possible role for immunity in the resolution of warts. The evidence to date, however, does not prove that immune mechanisms are directly responsible for the elimination of warts.

Because of latency and infectious recurrences, eradication of herpes simplex and herpes zoster from the world by vaccines is likely to be much more difficult to accomplish than eradication of smallpox. For some time we may have to settle for control of these diseases rather than their eradication. The live, attenuated varicella vaccine protects immunocompromised and normal persons against clinical chickenpox but it does not completely prevent subsequent infection with natural chickenpox virus or latency. With herpes simplex vaccines we may have to be satisfied with amelioration or control of clinical infections and diminution of latency and recurrent disease. Varicella zoster immune globulin plays a useful role in attenuation of varicella in immunocompromised or special-risk persons exposed to varicella zoster virus. Passive immunization for herpes simplex (herpes simplex immune globulin or other antibody preparations) has been studied very little. Maybe passive immunization will have a place in future therapies, especially for serious herpes simplex virus infections in immunocompromised hosts.

A case of multiple clear cell acanthomas is described with a review of the literature. This benign glycogen-rich epidermal tumor is generally unable to accept melanin and lacks phosphorylase or cytochrome oxidase activity. However, the basement membrane zone appears antigenically normal, as documented by the presence of epidermolysis bullosa acquisita antigen, bullous pemphigoid antigen, heparan sulfate proteoglycan, type IV collagen, and laminin by immunofluorescence microscopy.