Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry.

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.

Laryngeal and nasal involvement in pemphigus vulgaris.

BACKGROUND: The presence of lesions of pemphigus vulgaris in the larynx and nasal cavity has been reported in individual case reports. However, the frequency with which these sites are involved is not known. OBJECTIVE: Our purpose was to investigate the incidence of laryngeal and nasal disease involvement in patients with pemphigus vulgaris. METHODS: This was a retrospective analysis conducted in a referral private practice in an academic department of dermatology. A total of 53 sequential patients with pemphigus vulgaris diagnosed by clinical, histologic, and immunofluorescence criteria were selected on the basis of having been treated by one of us (J. C. B.) during most of their illness. Patients' charts were reviewed for documentation of laryngeal and nasal symptoms, ear/nose/throat evaluation, and response to treatment. RESULTS: Twenty-six (49%) of the patients complained of laryngeal or nasal symptoms at some time during the course of their disease. Twenty-one patients had laryngeal symptoms and 12 had nasal symptoms. More than 80% of patients with laryngeal or nasal symptoms had evidence of pemphigus involvement based on ear/nose/throat examination or rapid response to increased doses of corticosteroids, and 2 patients had candidiasis confirmed by fungal culture. CONCLUSION: These observations indicate that laryngeal and nasal symptoms are common in pemphigus vulgaris. In the majority of cases, this appears to be a result of involvement with the disease or with candidiasis.

Atypical herpes simplex can mimic a flare of disease activity in patients with pemphigus vulgaris.

We present a 69-year-old white woman with pemphigus vulgaris limited to the oral mucosa who presented with oral pain and difficulty swallowing of 2 days duration, followed by multiple irregular ulcers arising from normal mucosa with no grouping of individual lesions--herpes simplex should be considered in the differential diagnosis of lesions that appear suddenly in patients with PV, particularly if the lesions fail to respond to an increased dose of corticosteroids.