Faculty Bibliography

A 61-year-old-man presented with a sudden onset of multiple, hyperpigmented papules with a central punctum on the face, chest, upper back, and arms. Histopathologic examination showed infundibular cysts. These findings are consistent with a diagnosis of multiple eruptive milia, which is a rare disorder that is characterized by the sudden development of crops of milia over weeks to months. They are more extensive in number and distribution than they are in primary milia. Milia may present spontaneously without a known cause, as part of an inherited familial condition, or as part of a genodermatosis. The etiologies are uncertain, and treatment options are varied

In many species, the germ cells, precursors of sperm and egg, migrate during embryogenesis. The signals that regulate this migration are thus essential for fertility. In flies, lipid signals have been shown to affect germ cell guidance. In particular, the synthesis of geranylgeranyl pyrophosphate through the 3-hydroxy-3-methyl-glutaryl coenzyme A reductase (Hmgcr) pathway is critical for attracting germ cells to their target tissue. In a genetic analysis of signaling pathways known to affect cell migration of other migratory cells, we failed to find a role for the Hedgehog (Hh) pathway in germ cell migration. However, previous reports had implicated Hh as a germ cell attractant in flies and suggested that Hh signaling is enhanced through the action of the Hmgcr pathway. We therefore repeated several critical experiments and carried out further experiments to test specifically whether Hh is a germ cell attractant in flies. In contrast to previously reported findings and consistent with findings in zebrafish our data do not support the notion that Hh has a direct role in the guidance of migrating germ cells in flies

A 76-year-old man with a 52-year history of urticaria pigmentosa was found to have an elevated serum tryptase level as well as osteoporosis. Histopathologic alterations of one his skin lesions showed an infiltrate of mast cells. Urticaria pigmentosa patients are at risk for osteoporosis because of elevated heparin and stem-cell factor levels. These patients should be screened with serum tryptase levels and bone density studies to detect osteoporosis, regardless of their age

An 8-year-old boy presented with a lifelong history of a vascular mass overlying his right mandible with central coarse telangiectasias and peripheral pallor. Histopathologic examination showed a proliferation of blood vessels in the dermis. Ultrasound examination identified a mix of high- and low-flow vessels within the lesion. These findings were consistent with a noninvoluting congenital hemangioma, a rare vascular tumor that is fully formed at birth that subsequently grows proportionately with the patient and does not regress

A 64-year-old woman presented with 2 years of pruritic and ulcerated nodules and tumors on the trunk and arms. Histopathologic examination showed a diffuse infiltrate that consisted of predominantly small lymphocytes and scattered large atypical multinucleated cells positive for CD30. These findings were consistent with a diagnosis of anaplastic large-cell T-cell lymphoma, which is a CD30+ cutaneous lymphoma. This case highlights the importance of considering both histopathologic and clinical criteria in diagnosing a patient with a CD30+ cutaneous lymphoma

Advanced melanoma has a poor prognosis, and standard adjuvant treatment offers little survival advantage. Current efforts are aimed at combining chemotherapy and novel immunomodulators, which these include melanoma vaccines, cytokines and anti-CTLA4 antibodies. Hundreds of combination therapies are currently in trials. All advanced melanoma patients should be considered for enrollment in a trial for their own benefit as well as for the advancement of melanoma treatment. Thus far, no single investigative approach stands out as highly effective, however, they all hold promise with rare patients showing durable responses. Most treatment protocols are evaluating combinations of adjuvant therapies, hoping to achieve a synergistic effect. Ongoing research into the biology of melanoma continues to suggest new drug targets that will block tumor progression or enhance host response. Until the day that an effective treatment for advanced melanoma is found, prevention and early detection will remain paramount in combating this deadly malignancy

A 29-year-old woman presented with 8 months of multiple vesicles, erosions, and milia on the dorsa of her hands and feet. Histopathologic examination demonstrated a subepidermal blister, with a paucity of eosinophils and a lack of blood vessel wall thickening or caterpillar bodies. A direct immunofluorescence test showed a linear deposit of IgG at the dermo-epidermal junction. These findings were consistent with a diagnosis of epidermolysis bullosa acquisita. This case is a classic example of this rare blistering disease, in which patients produce autoantibodies to collagen VII, which is the major component of the anchoring fibrils

A 45-year-old man presented with a 3-year history of asymptomatic, annular, erythematous plaques on the face and neck. Histopathologic examination showed sarcoidal granulomas with an associated mixed-cell infiltrate, loss of elastic fibers, and no increase in mucin. These findings were consistent with a diagnosis of actinic granuloma, which is an uncommon, idiopathic disease that is characterized by granuloma annulare-like plaques on sun-exposed areas

We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and reticulated papillomatosis, and the only report describing family members having both confluent and reticulated papillomatosis and tinea versicolor. This report lends further evidence to the hypothesis that confluent and reticulated papillomatosis may be etiologically linked to tinea versicolor, and also suggests a genetic predisposition for it