Faculty Bibliography

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

Summary Background Large congenital melanocytic naevi (LCMN), which develop in utero and are present in approximately one in 20 000 newborns, are associated with markedly increased risks of cutaneous melanoma, leptomeningeal melanoma and neurocutaneous melanocytosis (NCM). Objectives This study examined clinical characteristics associated with melanoma and NCM among patients with LCMN, and estimated the risk of developing melanoma and NCM in these patients. Methods Two hundred and five LCMN patients enrolled in the New York University registry were studied. One hundred and seventy of these patients were followed prospectively. The remaining 35 patients had either melanoma at the time of entry into the registry (n = 6), or had insufficient follow-up information (n = 29). The outcome measures were the occurrence of melanoma and NCM. The associations between these outcomes and the clinical covariates (anatomical location of the LCMN, size of the LCMN, number of satellite lesions, family history of melanoma, patient sex and treatment) were assessed. Results Four of 170 (2.3%) prospectively followed patients developed melanomas, representing a standardized morbidity ratio of 324. Among the entire cohort (n = 205), there were associations between increasing numbers of satellite naevi and the occurrence of melanoma (P = 0.04), and the presence of NCM (P = 0.06). Compared with patients who did not develop these diseases, median LCMN diameters were larger among patients who developed melanoma (49 vs. 39 cm) and NCM (55 vs. 46 cm). Conclusions In LCMN patients, increasing numbers of satellite lesions and larger LCMN diameters are associated with melanoma and NCM