Paraneoplastic Dermatomyositis with Cutaneous and Myopathic Disease Responsive to Adrenocorticotropic Hormone Therapy.

Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy. The authors present a case report of paraneoplastic dermatomyositis associated with triple negative, BRCA-1 positive, invasive intraductal carcinoma of the breast, whose myopathic and cuteanous symptoms were recalcitrant to high-dose corticosteroid therapy. Herein, the authors describe the first reported case of the use of an injectable adrenocorticotropic hormone agonist gel in a patient with myopathic paraneoplastic disease that achieved clinical resolution of both myopathic and cutaneous symptoms, but subseuqently developed significant hyperpigmentation of her face suspected to be secondary to a chemotherapeutic-induced pigmentary change which was augmented by adrenocorticotropic hormone therapy.

A Case of Lichen Planus Pigmentosus with Facial Dyspigmentation Responsive to Combination Therapy with Chemical Peels and Topical Retinoids.

Lichen planus pigmentosus is a photodistributed dyschromia of unknown etiology described clinically as hyperpigmented gray-blue or brown-black macules or patches in a photodistributed pattern. Although there has been some debate, lichen planus pigmentosus is considered by many to be a separate diagnostic entity from ashy dermatosis or erythema dyschromicum perstans, which shares similar characteristics. Various treatment strategies have been applied to help resolve or improve the appearance of lichen planus pigmentosus lesions; however, an optimal treatment method is yet to be elucidated. The authors present a case of an 18-year-old Hispanic man with lichen planus pigmentosus whose skin findings responded dramatically to a combined regimen of daily topical azelaic acid foam and tretinoin cream with twice-monthly chemical peels using glycolic acid and Jessner's solution. The authors have noted a sparcity of therapeutic literature for lichen planus pigmentosus, and hope to aid clinicians in therapeutic management strategy for this patient subset.

Treatments for microcystic adnexal carcinoma--A review.

INTRODUCTION: Microcystic adnexal carcinoma (MAC) is a rare malignant cutaneous neoplasm presenting as a slow-growing, indurated nodule, papule or plaque. Clinically, the lesion can blend into the surrounding skin, obscuring borders and consequently delaying diagnosis histologically. Surgical and histologic techniques that emphasize examination of all margins may optimize management through early diagnosis and prevention of recurrences. OBJECTIVE: This review aims to assess the current surgical and histology techniques that result in lower rates of tumor recurrence and, consequently, better clinical outcomes. METHODS: A literature search of the PubMed database was conducted to identify studies examining wide local excision (WLE), Mohs micrographic surgery (MMS), radiotherapy (RT) and chemotherapy in the treatment of MAC. RESULTS: WLE had a high likelihood of positive margins and local recurrence. MMS was found to have the lowest recurrence rates. Definitive RT could be considered for elderly patients or those who are poor surgical candidates, as large surgical defects may be required to obtain free margins with either WLE or MMS. Chemotherapy was found to be ineffective. CONCLUSION: Complete margin evaluation with MMS permits complete tumor removal with subsequently low recurrence rate.

Epidermodysplasia verruciformis and cutaneous human papillomavirus DNA, but not genital human papillomavirus DNAs, are frequently detected in vulvar and vaginal melanoma.

Vulvovaginal melanomas are rare and their etiology is unknown. Genital mucosal human papillomavirus (HPV) 16 has been identified in both cutaneous and mucosal melanoma, suggesting that it might play a role in the pathogenesis or progression of melanoma. In this study, we investigated the prevalence of HPV DNA by using a broad spectrum of degenerate and type-specific primers for genital-mucosal, epidermodysplasia verruciformis-associated (EV), and cutaneous HPV types in 6 vulvar and 3 vaginal melanomas. The patients were mostly postmenopausal women (8/9), had a mean age of 67 years (range, 44-85 years), and had mucosal lentiginous (7) or nodular (2) melanomas. In the adjacent skin/mucosa, mucosal melanosis was found in 5, lichen sclerosus or a lichenoid mucositis in 4, and blue nevi in 2 women. With nested polymerase chain reaction techniques followed by direct sequencing, HPV DNA was identified in 6 of 9 (67%) melanomas; these were either cutaneous (HPV 3) (4/9) or epidermodysplasia verruciformis-associated types (HPV 38, Z95969, AJ00151) (4/9). Epidermodysplasia verruciformis-associated HPV (type 15) was found solely in 1/10 (10%) normal vulvar controls. Genital-mucosal HPV types were not detected either by degenerate nested polymerase chain reaction or type-specific probes for HPV 16. We propose that the above findings are not coincidental but may represent a molecular record of HPV involvement in pathogenesis or progression of melanoma, which is consistent with the strong but poorly defined association of cutaneous HPV types with nonmelanoma skin cancers. The theory that HPV may act as a cofactor in melanoma development deserves further clinical and experimental investigations.