A case of hypopigmented mycosis fungoides

A case of hypopigmented mycosis fungoides

P2117 P2201 A case of hypopigmented mycosis fungoides Rajesh Yalamanchili, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Yelva Lyn...

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P2117

P2201

A case of hypopigmented mycosis fungoides Rajesh Yalamanchili, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Yelva Lynfield, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States; Sharon Glick, MD, SUNY Downstate Medical Center, Brooklyn, NY, United States

Molecular detection of melanoma-associated markers in blood: MUC-18 is associated with high grade malignancy and poor prognosis Maria Cristina Rapanotti, Department of Laboratory Medicine, University Hospital ‘‘Tor Vergata,’’ Rome, Italy; Ilaria Ricozzi, MD, Dermatology Department-University Hospital ‘‘Tor Vergata,’’ Rome, Italy; Sergio Bernardini, MD, Department of Laboratory Medicine, University Hospital ‘‘Tor Vergata,’’ Rome, Italy; Luca Bianchi, MD, Dermatology Department-University Hospital ‘‘Tor Vergata,’’ Rome, Italy

We present a case of an otherwise healthy, asymptomatic 10-year-old AfricanAmerican boy with several months of hypopigmented macules. Prior to presentation, the patient had been evaluated by his pediatrician who made a presumptive diagnosis of early vitiligo. The patient was referred to Dermatology for further management. On exam, he demonstrated scattered macules of varying degrees of hypopigmentation over his bilateral upper and lower extremities, trunk, and buttocks, some coalescing into patches. There was no associated depigmentation, erythema, scaling, or pruritus. He was born and raised in the United States, did not report any recent travel outside of the country, and had a negative family history for autoimmune diseases. A punch biopsy performed on a representative patch over the left superolateral buttock revealed mild psoriasiform epidermal hyperplasia, a sparse superficial lymphocytic perivascular infiltrate, and lymphocytic epidermotropism. Given these histologic findings and the patient’s clinical picture, the diagnosis of hypopigmented mycosis fungoides (HMF) was made. HMF respresents a distinct subtype of mycosis fungoides (MF) and is uniquely characterized by a younger age of onset, preferential occurrence in darker-skinned individuals, and a benign clinical course. With our patient, we recommended the use of narrowband UVB phototherapy, which is the currently recommended treatment of choice in children with limited patch-stage disease. Our case highlights the importance of recognizing that persistent hypopigmented patches with no inflammatory signs may not represent post-inflammatory pigmentary changes, but rather a more complex diagnosis that may necessitate a biopsy. Additionally, in light of the excellent prognosis seen in patients with HMF, it has been suggested that this entity be considered independent of the MF clinical spectrum. However, given the paucity in the literature of longterm follow-up of these patients, redefining HMF remains problematic as the ultimate outcomes of these patients remains to be seen. In order to gather this valuable data, we encourage dermatologists to enroll patients with HMF in the International Registry for Pediatric Cutaneous T-cell Lymphoma at the Hospital for Sick Children in Toronto.

Background: Detection of circulating melanoma cells (CMC) in peripheral blood (PB) was first reported by Hoon D. S. et al (1995), who described the use of a multimarker RT-PCR-assay as a predictive procedure of early disease recurrence and progression. We analyzed the usefulness of the simultaneous expression of selected melanoma-associated mRNAs (MAMs) in PB of 100 patients affected by melanoma. The aim of our study was to better evaluate the clinical effectiveness of cancer treatment and to detect ‘‘subclinical’’ disease in clinically disease-free patients. Methods: We enrolled 49 males and 51 females. All of them had been submitted to melanoma surgery between a range of time of 18 years (1987-2005). At the time of the blood draw, 91 patients were AJCC stage I-II and clinically disease-free by conventional physical and imaging examinations, 5 were AJCC stage III, and 4 were AJCC stage IV. The MAMs studied were: tyrosinase (TyrOH), MAGE-3, MART-1, p97 and MUC-18. Results: We documented the presence of at least one MAM in 23 cases (23%). Our data showed that stage III and IV are not necessarily associated with the simultaneous expression of 3-4 markers. We documented a strong association of MUC-18 positivity and disease progression, no correlation has been found for the other MAMs. Conclusion: MUC-18 is well-known to be a MAM associated with tumor progression. In our study, it is the only marker associated with more advanced disease stage, both alone and in combination with any of the other MAMs. Commercial support: None identified.

Commercial support: None identified.

MELANOMA AND PIGMENTED LESIONS P2200 Biopsy site selections based on confocal examination of pigmented lesions on the face and scalp Sanjay Mandal, MBBS, MD, Memorial Sloan-Kettering Cancer Center, New York, NY, United States; Alon Scope, MD, Memorial Sloan-Kettering Cancer Center, New York, NY, United States; Cristiane Benvenuto-Andrade, MD, Memorial SloanKettering Cancer Center, New York, NY, United States; Allan Halpern, MD, MS, Memorial Sloan-Kettering Cancer Center, New York, NY, United States Distinguishing melanoma from other pigmented lesions on the face and scalp can be a clinical challenge. Larger lesions may require multiple biopsies to establish the diagnosis and plan definitive treatment. However, biopsy site selection based on clinical appearance alone may be inconclusive and fail to reveal the worst pathology within the lesion. In vivo reflectance confocal microscopy (RCM) enables visualization of tissue morphology noninvasively and thus may be used to predict the most appropriate biopsy site. The study aim was to evaluate the potential of RCM for predicting the results of histopathologic analysis of biopsies from facial pigmented lesions. Eleven patients, each contributing one pigmented lesion on the face or scalp, were included in this study. Each lesion was evaluated by RCM at one or multiple sites. RCM images were analyzed by a dermatologist experienced with RCM (SG). Previously described RCM findings, such as presence of bright dendritic cells in the suprabasal epidermis, presence of junctional irregular shaped bright cells and disarray of normal epidermal architecture were used to suggest areas suspicious for melanoma. RCM was considered positive if findings were suspicious for melanoma. At least one biopsy was taken from each lesion at a site imaged with RCM. If the lesion was excised in toto, the most suspicious RCM findings were compared with the final histopathologic diagnosis. A correlation between positive/negative results was done between RCM and histopathology. Results: Four melanoma in situ, 2 invasive melanoma, 1 solar lentigo, 1 lesion showing features of lentigo and pigmented seborrheic keratosis, 1 lesion showing features of actinic keratosis and seborrheic keratosis, 1 dysplastic nevus, and 1 lichenoid keratosis were included in this series. Of the 6 melanomas, 18 sites were suggestive of melanoma on RCM, and 14 of those sites were histologically confirmed. In the non-melanoma lesions, two of the 19 sites that were analyzed by RCM were suggestive for melanoma but read as benign on histopathology.

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Conclusion: In vivo RCM may be useful in identifying appropriate biopsy sites on pigmented lesions on the face and scalp areas. Further evolution of this technique may obviate the need for repeated biopsies on this cosmetically sensitive area and lead to better histopathologic diagnosis.

The ‘‘ugly duckling’’ sign is sensitive for melanoma detection Alon Scope, MD, Dermatology Service, Memorial Sloan-Kettering Cancer Center, New York, NY, United States; Stephen Dusza, MPH, Memorial Sloan-Kettering Cancer Center, New York, NY, United States; Allan Halpern, MD, Memorial SloanKettering Cancer Center, New York, NY, United States; Ashfaq Marghoob, MD, Memorial Sloan-Kettering Cancer Center, New York, NY, United States In 1998, Grob et al. suggested the ‘‘ugly duckling’’ sign for melanoma detection, based on the observation that nevi in the same individual tend to resemble one another, and that melanoma is often an ‘‘ugly duckling,’’ different from the other nevi. However, the usefulness of this sign for melanoma screening was never analyzed. The aim of this study was to investigate whether the ‘‘ugly duckling’’ sign is sensitive for melanoma detection. Baseline images of the backs of 12 patients were obtained from a database of standardized patient images provided by MoleSafe, a New-Zealand based teledermatology company. All cases had at least 8 atypical moles on the back, and in 5 cases, 1 of the lesions was a histologically confirmed melanoma. For all cases there were 1-year follow-up dermoscopic images demonstrating stability of all benign nevi. An overview image of the back supplemented with close-up clinical images of the lesions was shown to study participants. The images were evaluated by 34 participants, including 8 pigmented lesion experts, 13 general dermatologists, 5 dermatology nurses, and 8 non-clinical medical staff. Participants were asked to identify one or more lesions on the back that differed from other nevi. Sensitivity of the ‘‘ugly duckling’’ sign for melanoma diagnosis was defined as the number of melanomas identified as different divided by the total number of melanomas. Specificity was defined as the number of nevi not identified as different divided by the total number of nevi. A lesion was considered a generally apparent ‘‘ugly duckling’’ if perceived as different by at least two-thirds of participants. The sensitivity of the ‘‘ugly duckling’’ sign for melanoma detection was 0.86 for the whole group; 0.89 for experts, 0.85 for general dermatologists, 0.88 for nurses and 0.85 for non-clinicians. The specificity was 0.87 for the group; 0.93 for the experts, 0.90 for general dermatologists, 0.79 for nurses, and 0.83 for nonclinicians. All 5 melanomas (100%) and only 4 of 140 nevi (2.9%) were generally apparent ‘‘ugly ducklings.’’ Limitations of the study are that assessment was done on selected images of patients with multiple atypical moles and that the study focused on melanocytic lesions and did not address other benign lesions. In conclusion, in the present set of cases, the ‘‘ugly duckling’’ sign was sensitive for melanoma detection across various skill groups. Melanoma was generally apparent as an ‘‘ugly duckling.’’

Commercial support: None identified.

Commercial support: None identified.

FEBRUARY 2007

J AM ACAD DERMATOL

AB143