P1164Linear lichen sclerosus along the lines of Blaschko

Cho Rok Kim, MD, Samsung Medical Center, Seoul, South Korea; Dong Youn Lee,MD, PhD, Samsung Medical Center, Seoul, South Korea; Haeyoung Park, MD,Samsung Medical Center, Seoul, South Korea; Ji-Hye Park, MD, Samsung MedicalCenter, Seoul, South Korea

Lichen sclerosus et atrophicus (LSA) is a rare chronic inflammatory dermatosis withanogenital and extragenital presentations. Women in the fifth or sixth decade of lifeand children younger than 10 years of age are usually affected. Anogenital lesions cancause severe discomforts (dyspareunia, dysuria, pruritus, and painful defecation)and manifest with erosions, porcelain-white plaques, papules, and wide degrees ofsclerosis. Potent topical corticosteroids associated with skin care are thought to bethe most successful therapy; according to recent reports, calcineurin inhibitors arealso effective. We present a case of facial LSA with asymptomatic, well-demarcated,whitish to bluish, atrophic patch in a linear pattern on the forehead in a 48-year-oldKorean woman. This case showed an atypical clinical presentation, mimicking encoup de sabre, but histopathologic results comfirmed the diagnosis of LSA. To ourknowlege, this is the second case of linear LSA occured on face following the lines ofBlaschko.

AB48

cial support: None identified.

Commer

P1165Melanocytic nevus over a blue nevus

Jose Aneiros-Fernandez, MD, San Cecilio University Hospital, Department ofPathology, Granada, Spain; Francisco O’Valle, MD, San Cecilio UniversityHospital, Department of Pathology, Granada, Spain; Husein Husein-ElAhmed,MD, San Cecilio University Hospital, Department of Dermatology, Granada,Spain; In�es Aroca Siendones, MD, San Cecilio University Hospital, Granada,Spain; Salvador Arias-santiago, MD, San Cecilio University Hospital, Departmentof Dermatology, Granada, Spain

Background: The combined nevus is characterized morphologically by the presenceof two or more different types of melanin nursing care process in one lesion. Theseinjuries can cause failure to perform clinical and pathologic diagnosis withmelanoma.

Case report: An 18-year-old man presented with a lesion on the scalp that wasslightly elevated, symmetrical, well circumscribed, pigmented, and 0.8 cm inmaximum diameter. The histopathologic examination revealed papillary and retic-ular dermis forming cells melanocytic nests (nests) and cordswith little pigment thatdoes not affect the epidermis. The level of reticular dermis (middle and deep) areidentified pigmented melanocytic cells with monomorphic nuclei, spindle-lookingthat are available separately between collagen fibers and periadnexal, establishingthe diagnosis of congenital intradermal melanocytic nevus and common blue nevus(combined nevus).

Discussion: The combined nevus is 1% of melanocytic lesions, appearing in groupsof varying ages 3 to 83 years with an average of 30 years, with slight femalepreponderance. The nevus is mainly located in the chest, back, abdomen, and lessfrequently on the scalp, extremities, and buttocks. Most of these lesions are\7 mm,symmetrical, well circumscribed, and variable in color (black, blue, and brown).Histologically, the lesion is composed of at least two pigmented lesion that can bemixed, in a pattern most common congenital nevus and blue nevus, but can occurwith Spitz nevus, deep penetrating nevus, spindle cell nevus plexiform pigmenteddysplastic nevus.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P1166Polydactyly

Jose Aneiros-Fernandez, MD, San Cecilio University Hospital, Department ofPathology, Granada, Spain; Husein Husein-ElAhmed, MD, San Cecilio UniversityHospital, Department of Pathology, Granada, Spain; In�es Aroca Siendones, MD,San Cecilio University Hospital, Department of Dermatology, Granada, Spain;Jose Aneiros-Cachaza, MD, PhD, San Cecilio University Hospital, Department ofPathology, Granada, Spain; Salvador Arias-Santiago, MD, San Cecilio UniversityHospital, Department of Dermatology, Granada, Spain

Background: Polydactyly is the presence of a supernumerary toe in the hands or feet.It is the single most common malformation of the upper limbs, but sometimes it isassociated with a syndrome.

Case report: A patient a few days of age with the presence of a supernumerarythumb on the left of 1 cm 3 0.7 cm in diameter.

Conclusion: It is an inherited defect that is either autosomal dominant or recessive.The prevalence is 1.6 per 1000 newborns. They can be classified into two groups(preaxial, postaxial, and central duplication). In 82% of cases, the polydactyly is anisolated defect and its association with other malformations is minimal. However, itis advisable to look for associated malformations.

cial support: None identified.

Commer

P1167Sweet syndrome: Case report

Renata Paes Barreto DaSilva, MD, Hospital Central do Ex�ercito, Rio de Janeiro,Brazil; Amanda Nabuco, MD, Hospital Central do Ex�ercito, Rio de Janeiro, Brazil;Caroline Fattori Assed Saad, MD, Hospital Central do Ex�ercito, Rio de Janeiro,Brazil; Mariana Zangrando, MD, Hospital Central do Ex�ercito, Rio de Janeiro, Brazil

Sweet syndrome or acute febrile neutrophilic dermatosis is a disease that occursworldwide, and it is three times more prevalent in women than men, except whenassociated with malignancy where there is not a female predominance. Earlydiagnosis of the clinical symptoms is beneficial because there is a better response totreatment and also because of the association with malignancy in some cases. Thiscase study is about a 36-year-old woman presenting with fever and odynophagia of 3days’duration. After 7 days, the patient presentedwith erythematous-based pustulesand papules arranged in vesicle-like plaques that were painful and itchy developedon the sternum, breast, superior proximal limbs, and back. Histopathologic findingsrevealed edema and a diffuse infiltration of neutrophils in the papillary dermis withswollen endothelial cells. Laboratory results showed normal values of completeblood cell. The patient was treated with prednisone 60 mg per day for 4 weeks andalbendazol 400 mg per day for 3 days to prevent negative Gram germ infection. Afterthe third week, the symptoms improved and the skin lesions were in remission.Sweet syndrome’ pathogenesis is unknown; however, it is usually preceded byrespiratory or gastrointestinal infection caused by Streptococcus spp. and Yersinia,respectively. Association with diseases such as the autoimmunes would suggest thatthere is a relationship with hypersensitivity reactions. The most widely acceptedtheory is that there is an irregularity in cytokines secretions, principally interleukins1, 3, 6, and 8, granulocyte colony-stimulating factor, macrophages, and interferongamma. In addition, neoplasia and some drugs can be associated with the disease.Pathology characteristically reveals nodular dermic and diffuse neutrophils infiltra-tion with karyorrhexis and edema in the papillary dermis, which can overrun theepidermis and originate subcorneal pustules. Laboratory features includes leucocy-tosis with neutrophilia, raised levels of protein C, and hemosedimentation speedand antineutrophil antibodies. Diagnostic can be based on Su&Liu criteria: twomajor (histopathology and acute characterisc skin lesion) and four minor (infection,malignancy, drugs, fever and extra cutaneous manifestation; leukocytosis andcorticosteroid response). Systemic corticosteroids (0.5 mg to 1 mg/kg/day) for 4to 6 weeks are the primary treatment for Sweet syndrome, reducing the skin lesionsand systemic symptoms.

cial support: None identified.

Commer

FEBRUARY 2011