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P8176Facial Becker nevus associated with fibrous dysplasia and facialasymmetry
Sue Ann Chan, MBChB, Birmingham Skin Centre, Birmingham, United Kingdom;Amirtha Vani Rajasekaran, MBChB, Birmingham Skin Centre, Birmingham, UnitedKingdom
Introduction: Becker nevus is a pubertal onset epidermal nevus, more common inmales, associated with hyperhidrosis, hypertrichosis, and melanosis triggered bycirculating androgens.We report an interesting case of Becker nevus associated withfibrous dysplasia of maxillary bone and facial asymmetry.
Case report: A 32-year-old man was referred by his General Practitioner withreduced density of hair growth over his left side of his face since puberty. Hegenerally shaves his right side of his face twice a day, while barely requiring to shavehis left side of the face tomaintain symmetry. Interestingly, he gives history of fibrousdysplasia of his right maxilla, requiring dental transplant of his UR5 (upper right 5)and UR8 at age 20. He had childhood asthma and eczema and is hypertensive. Hedoes not report any family history of genetic disorders. He is married with 1 childwho has 3 cherry hemangiomas but otherwise very well. His regular medicationsinclude lisinopril and furosemide. Examination revealed increased hair growth onthe right side of the face with hyperhidrosis. A mild facial asymmetry was also notedwith more prominent nasolabial fold on the right side. There is no sensorineuraldeficits. Eyelashes, eyebrows, and scalp hair were symmetrical. This patient wasdiscussed in depth at the West Midlands Regional Clinical Meeting and a possiblediagnosis of Becker nevuswas postulated. Punch biopsies were taken from each sideof his face for comparison. Biopsy from the left cheek was normal while the biopsytaken from the right cheek revealed mild epidermal acanthosis, prominent hairfollicles and smooth muscle hypertrophy of the arrectores pilorum aswell as smoothmuscle bundles in the dermis. There was also mild perivascular chronic inflamma-tion in the superficial dermis. These findings confirm a diagnosis of a Becker nevuson the right side of the face.
Discussion: Interestingly, our patient and General Practitioner’s concern waspredominantly the lack of hair growth on the left side of his face rather than thehypertrichosis on his right side. It was very difficult to ascertain the focus ofpathology in the initial stages—the lack of hair on the left side or the hypertrichosison the right side. We reinforce the importance of adapting an open approach whenmanaging dermatologic patients to obtain the correct diagnosis and treatment planfor our patients.
AB44
cial support: None identified.
CommerP7717Facial lumps and bumps
Ha Do, MD, MS, Indiana University, Dermatology Department, Indianapolis, IN,United States; Daniel West, MD, Indiana University, Dermatology Department,Indianapolis, IN, United States; Jessica LeBlanc, MD, Indiana University,Dermatology Department, Indianapolis, IN, United States; Yongxue Yao, MD,PhD, Indiana University, Dermatology Department, Indianapolis, IN, UnitedStates
Various dermatologic conditions can give rise to or are associated with facial lumpsand bumps. We present 3 interesting facial lumps and bumps cases with greatclinical photographs, histopathology photos, and clinical pearls to highlight the keyteaching points for each case. We include both common presentations for rareconditions and rare presentations for rare conditions. The first case is a 61-year-oldfemalewith history of sarcoidosis presented with secondarymiliary osteoma cutis ofthe cheeks. These lesions are considered as metaplastic ectopic ossification inpreviously active facial cutaneous sarcoid lesions. The second case is a 60-year-oldmale with previously undiagnosed Birt-Hogg-Dube syndrome who presented for anunrelated problem. The facial papules led a series of questions that increased thesuspicion for the diagnosis, and biopsy of the papules allowed confirmation andinitiation of proper clinical follow-up. The third case is a 54-year-old female whodemonstrated an unusual presentation of localized central facial papules that turnedout to be nodular amyloidosis. Amyloidosis teaching points are reviewed.
cial support: None identified.
CommerJ AM ACAD DERMATOL
P7567GardnereDiamond syndrome
Nathan Cleaver, DO, St. Joseph Mercy Hospital, Ypsilanti, MI, United States;David Altman, MD, St. Joseph Mercy Hospital, Ypsilanti, MI, United States; StuartGildenberg, MD, St. Joseph Mercy Hospital, Ypsilanti, MI, United States
A 30-year-old white female presented with a 5-day history of complaints of low backpain with lower extremity numbness and tingling. She stated her symptoms beganabruptly while watching television. There was no history of trauma or injury to herlow back. The patient’s past medical and surgical history was unremarkable. Thepatient admitted to significant personal stress in her life. On physical examination,the patient was emotionally labile and tearful. She had clustered purpuric patches inmultiple stages of healing scattered on the central lower back. Upon palpation, thepatient claimed pain of 10/10. On the same visit, another resident physicianperformed the same examination independently, but elicited only minimaltenderness upon palpation. Following an inconclusive neurologic and hematologicwork-up, the diagnosis of GardnereDiamond syndromewasmade. In 1955, Gardnerand Diamond first described a condition in which purpura was produced byhypersensitivity to extravasated erythrocytes. In 1968, Ratnoff and Agle proposedthe name psychogenic purpura, as lesions often occurred in association withpsychiatric problems such as depression, anxiety, histrionic or conversion person-ality disorder. GardnereDiamond syndrome (autoerythrocyte sensitization syn-drome, psychogenic purpura) is characterized by the sudden onset of painful,swollen bruises of variable sizes in a particular area of the body. The lesions are oftenprecipitated by emotional stress, and are primarily seen in middle-aged women,although it has been less commonly reported in males and children. In manypatients, this will recur, often remitting for months to years with relief of emotionalstressors. The lesions tend to spontaneously resolve in about 2 weeks, with nofatalities or serious complications reported in association with the disease. Therehave been no therapeutic interventions that have proved beneficial other thanpsychotherapy to date.
cial support: None identified.
CommerP7986Giant cell tumor of the hand: A case report
Manuel Valdebran, MD, Instituto Dermatol�ogico y Cirug�ıa de Piel ‘‘Dr. HubertoBogaert D�ıaz,’’ Santo Domingo, Dominican Republic; Angel Taveras, MD, InstitutoDermatol�ogico y Cirug�ıa de Piel ‘‘Dr. Huberto Bogaert D�ıaz,’’ Santo Domingo,Dominican Republic; Eduardo Sanchez, MD, Instituto Dermatol�ogico y Cirug�ıa dePiel ‘‘Dr. Huberto Bogaert D�ıaz,’’ Santo Domingo, Dominican Republic; FernandaNanita Estevez, Instituto Dermatol�ogico y Cirug�ıa de Piel ‘‘Dr. Huberto BogaertD�ıaz,’’ Santo Domingo, Dominican Republic; Nery Ram�ırez, MD, InstitutoDermatol�ogico y Cirug�ıa de Piel ‘‘Dr. Huberto Bogaert D�ıaz,’’ Santo Domingo,Dominican Republic; Rossy Nu~nez, MD, Instituto Dermatol�ogico y Cirug�ıa de Piel‘‘Dr. Huberto Bogaert D�ıaz,’’ Santo Domingo, Dominican Republic
Background: Giant cell tumor (GCT) of phalanx is a rare entity accounting forapproximately 2% to 5% of all giant cell tumors. Metaphyseal region of themetacarpals and phalanges is the site of origin for most of these tumors. ThoughGCT is not a sarcoma, those located in the finger appear to behavemore aggressivelyoften requiring extensive en bloc excision.
Case report: A 23-year-old female presented to our institution with a tender swellingof her left middle finger, which has been present for only 1.5 months. Physicalexamination was otherwise normal, regional nodes were not palpable. Routineserological testing and a chest radiographs were inconspicuous. Radiographs of thehand revealed expansile multiloculated lytic lesion involving the entire middlephalanx with cortical break but preserved articular spaces. The lesion wascompletely removed by en bloc resection of the third finger of her left hand.Biopsy of the specimen showed amesenchymal lesion characterized by proliferationof fibroblasts, histiocytes and abundant multinucleated giant cells.
Discussion: When GCT occur in the hand, they frequently cause severe bonydestruction and extend into the surrounding soft tissues. This case was uniqueconsidering the comparative rarity of a giant cell tumor arising from the phalanges ofthe finger. A review of the literature demonstrates a paucity of such cases. Because ofthe higher risks for local recurrence, treatment should be aggressive.
cial support: None identified.
CommerMAY 2014
