- Email: [email protected]
Nasal root island pedicle flap for lateral nasal sidewall defect: A case report
4710
4706
Multiple symmetric lipomatosis Mariano Marini, MD, Sanatorio G€ uemes; Sergio Lacossegliaz, MD, Sanatorio G€ uemes; Guillermina Giardini, MD, Sanatorio G€ uemes; Victoria Campana, MD, Sanatorio G€ uemes Multiple symmetric lipomatosis (MSL), also known as Madelung’s disease, is a rare condition of the adipose tissue of unknown etiology, associated with alcoholism, metabolic syndrome, and peripheral neuropathy. Brodie first described it in 1846, and since then only 300 case reports have been published. The disease is characterized by nonencapsulated fat masses symmetrically distributed in the head, the neck, the trunk, the abdominal wall and the proximal parts of the upper limbs. It is common in middle-aged white men of the Mediterranean area. We are reporting a 56-year-old man, cigarette and alcohol user, with history of chronic obstructive pulmonary disease, pulmonary tuberculosis diagnosed in 2010 and allergy to iodine, who was attended at our service because he presented with multiples symmetric masses of different size that had appeared seven years ago in the occipital region, neck, trunk and proximal parts of upper and lower limbs. They had increased their size during the last six months. At the physical examination they revealed to be soft, mobile and painless. Laboratory showed abnormal liver enzymes and dyslipidemia. Biopsy of the mass, soft tissue ultrasound and computing tomography requested, all coincided in diagnosis of lipoma. Taking into consideration the history and clinical features of the patient and the reports of the complementary studies, we arrived at the diagnosis of MSL type I. In conclusion, MSL is a rare disease found mainly in chronic alcoholic men with metabolic syndrome. Although at present there is no effective treatment, it is recommended to abandon alcohol and lose weight to avoid progression of the disease. Surgical therapy is reserved for symptomatic tumors.
Nail dystrophy presenting as an early sign of primary systemic amyloidosis Juliana Feres, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Jo~ao Carlos Lopes Sim~ao, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Maurıcio Eiji Almeida Santos, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Marco Andrey Cipriani Frade, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo A 48-year-old woman presented with a one year history of progressive dyspnea and anasarca at cardiology department. During investigation, overall cardiac enlargement was noted, with the echocardiogram showing infiltrative restrictive cardiomyopathy and MRI that strongly suggested cardiac amyloidosis. There were no other systemic or cutaneous findings, except for a three years history of nail changing. All the digits and her toenails had a thin nail plate, onychorrhexis and splitting. A lateral longitudinal biopsy of the left third finger showed an abnormal dermis, expanded by a fissured amorphous eosinophilic material. These deposits were Congo red positive with apple-green birefringence under polarized light, consistent with amyloid. Laboratory investigation ended without criteria for the diagnosis of multiple myeloma, but showed a hyper production of lambda serum light chains (kappa: 6.58; lambda ¼ 308 and ratio 0.02). The final diagnosis was primary systemic amyloidosis (lambda chain). Patient started treatment at the hematology department, but died after 6 months, because of cardiac injury. Commercial support: None identified.
Commercial support: None identified.
5426 Nail changes in alopecia areata: Case report and review of the literature Khatiya Chelidze, BS, Khatiya Chelidze; Shari Lipner, MD, Weill Cornell Medicine A 54-year-old man was referred to our nail clinic with ridging of his fingernails. He was diagnosed with alopecia areata (AA) one year prior to the nail changes. His hair loss progressed rapidly, with loss of all scalp, facial and body year within a month. Physical examination was significant for severe ridging and trachyonychia of all his fingernails, without toenail involvement. A nail biopsy was performed. Histologic examination revealed abnormal keratinization of the nail plate with superficial matrical dyshesion and premature dyskeratosis. Nail changes are often associated with AA. There is abnormal keratinization of the nail matrix, but the pathophysiology of these changes is incompletely understood. Nail abnormalities are present in 14-19% of adult patients with AA and may be even more common in the pediatric population. The risk of nail changes has been shown to correlate with the severity of disease; ie, alopecia totalis (AT) and alopecia universalis (AU) are the most closely associated variants. The most common nail changes associated with AA are pitting (15%) and trachyonychia (8%-14%). Other reported changes include red spotted lunulae, longitudinal ridging, punctate leukonychia and pigmented longitudinal bands. Interestingly, abnormalities of the toenails are rarely seen. The onset of nail changes has been noted to precede, follow, and coincide with the onset of hair loss variably. Due to the association of AU and AT with nail changes, authors have argued that the presence of nail changes may be a poor prognostic factor. Nail changes associated with AA should be confirmed with a biopsy to guide diagnosis and treatment. Whether nail changes resolve with treatment of AA is unclear; clinical trials on patients with AA are required to better understand optimal therapies.
5410 Nasal root island pedicle flap for lateral nasal sidewall defect: A case report Jean-Philip Lacroix, MD, McGill University; Manish Khanna, MD, McGill University Background: Various modifications of island pedicle flaps have been described to address difficult reconstructive situations. It is a very tissue-efficient flap that provides outstanding clinical result. It must be located in areas with elastic and spongy subcutaneous tissue with a rich blood supply. We describe a superiorly based nasal root island pedicle flap to repair defects of the lateral nasal sidewall. Objective: Our purpose is to describe a novel single-stage island pedicle flap for repairs of lateral nasal sidewall. Methods: We provide illustrative example with descriptive technique and method of placing the flap. FIGURE 1. A. 1.6 cm 3 1.2 cm defect of the left lateral nasal sidewall after Mohs excision of a squamous cell carcinoma. B. Intraoperatively, an island pedicle flap is designed with a muscular pedicle based on the rich blood supply of the dorsal nasal artery and the supratrochlear artery. C. Postoperative defect reconstructed with the superior-based nasal root island pedicle flap. FIGURE 2. A. Early cosmetic result 2 weeks postoperatively. B. C. Cosmetic result with no revision 6 months postoperatively. Conclusion: The nasal root island pedicle flap for repair of upper lateral nasal sidewall defect is a new application of a one-stage procedure that yields excellent functional and cosmetic result. Knowledge of the limitations and the anatomy of the flap are crucial for a good outcome. Commercial support: None identified.
Commercial support: None identified.
JUNE 2017
J AM ACAD DERMATOL
AB175
4706
Multiple symmetric lipomatosis Mariano Marini, MD, Sanatorio G€ uemes; Sergio Lacossegliaz, MD, Sanatorio G€ uemes; Guillermina Giardini, MD, Sanatorio G€ uemes; Victoria Campana, MD, Sanatorio G€ uemes Multiple symmetric lipomatosis (MSL), also known as Madelung’s disease, is a rare condition of the adipose tissue of unknown etiology, associated with alcoholism, metabolic syndrome, and peripheral neuropathy. Brodie first described it in 1846, and since then only 300 case reports have been published. The disease is characterized by nonencapsulated fat masses symmetrically distributed in the head, the neck, the trunk, the abdominal wall and the proximal parts of the upper limbs. It is common in middle-aged white men of the Mediterranean area. We are reporting a 56-year-old man, cigarette and alcohol user, with history of chronic obstructive pulmonary disease, pulmonary tuberculosis diagnosed in 2010 and allergy to iodine, who was attended at our service because he presented with multiples symmetric masses of different size that had appeared seven years ago in the occipital region, neck, trunk and proximal parts of upper and lower limbs. They had increased their size during the last six months. At the physical examination they revealed to be soft, mobile and painless. Laboratory showed abnormal liver enzymes and dyslipidemia. Biopsy of the mass, soft tissue ultrasound and computing tomography requested, all coincided in diagnosis of lipoma. Taking into consideration the history and clinical features of the patient and the reports of the complementary studies, we arrived at the diagnosis of MSL type I. In conclusion, MSL is a rare disease found mainly in chronic alcoholic men with metabolic syndrome. Although at present there is no effective treatment, it is recommended to abandon alcohol and lose weight to avoid progression of the disease. Surgical therapy is reserved for symptomatic tumors.
Nail dystrophy presenting as an early sign of primary systemic amyloidosis Juliana Feres, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Jo~ao Carlos Lopes Sim~ao, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Maurıcio Eiji Almeida Santos, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo; Marco Andrey Cipriani Frade, MD, Ribeir~ao Preto Medical School, University of S~ao Paulo A 48-year-old woman presented with a one year history of progressive dyspnea and anasarca at cardiology department. During investigation, overall cardiac enlargement was noted, with the echocardiogram showing infiltrative restrictive cardiomyopathy and MRI that strongly suggested cardiac amyloidosis. There were no other systemic or cutaneous findings, except for a three years history of nail changing. All the digits and her toenails had a thin nail plate, onychorrhexis and splitting. A lateral longitudinal biopsy of the left third finger showed an abnormal dermis, expanded by a fissured amorphous eosinophilic material. These deposits were Congo red positive with apple-green birefringence under polarized light, consistent with amyloid. Laboratory investigation ended without criteria for the diagnosis of multiple myeloma, but showed a hyper production of lambda serum light chains (kappa: 6.58; lambda ¼ 308 and ratio 0.02). The final diagnosis was primary systemic amyloidosis (lambda chain). Patient started treatment at the hematology department, but died after 6 months, because of cardiac injury. Commercial support: None identified.
Commercial support: None identified.
5426 Nail changes in alopecia areata: Case report and review of the literature Khatiya Chelidze, BS, Khatiya Chelidze; Shari Lipner, MD, Weill Cornell Medicine A 54-year-old man was referred to our nail clinic with ridging of his fingernails. He was diagnosed with alopecia areata (AA) one year prior to the nail changes. His hair loss progressed rapidly, with loss of all scalp, facial and body year within a month. Physical examination was significant for severe ridging and trachyonychia of all his fingernails, without toenail involvement. A nail biopsy was performed. Histologic examination revealed abnormal keratinization of the nail plate with superficial matrical dyshesion and premature dyskeratosis. Nail changes are often associated with AA. There is abnormal keratinization of the nail matrix, but the pathophysiology of these changes is incompletely understood. Nail abnormalities are present in 14-19% of adult patients with AA and may be even more common in the pediatric population. The risk of nail changes has been shown to correlate with the severity of disease; ie, alopecia totalis (AT) and alopecia universalis (AU) are the most closely associated variants. The most common nail changes associated with AA are pitting (15%) and trachyonychia (8%-14%). Other reported changes include red spotted lunulae, longitudinal ridging, punctate leukonychia and pigmented longitudinal bands. Interestingly, abnormalities of the toenails are rarely seen. The onset of nail changes has been noted to precede, follow, and coincide with the onset of hair loss variably. Due to the association of AU and AT with nail changes, authors have argued that the presence of nail changes may be a poor prognostic factor. Nail changes associated with AA should be confirmed with a biopsy to guide diagnosis and treatment. Whether nail changes resolve with treatment of AA is unclear; clinical trials on patients with AA are required to better understand optimal therapies.
5410 Nasal root island pedicle flap for lateral nasal sidewall defect: A case report Jean-Philip Lacroix, MD, McGill University; Manish Khanna, MD, McGill University Background: Various modifications of island pedicle flaps have been described to address difficult reconstructive situations. It is a very tissue-efficient flap that provides outstanding clinical result. It must be located in areas with elastic and spongy subcutaneous tissue with a rich blood supply. We describe a superiorly based nasal root island pedicle flap to repair defects of the lateral nasal sidewall. Objective: Our purpose is to describe a novel single-stage island pedicle flap for repairs of lateral nasal sidewall. Methods: We provide illustrative example with descriptive technique and method of placing the flap. FIGURE 1. A. 1.6 cm 3 1.2 cm defect of the left lateral nasal sidewall after Mohs excision of a squamous cell carcinoma. B. Intraoperatively, an island pedicle flap is designed with a muscular pedicle based on the rich blood supply of the dorsal nasal artery and the supratrochlear artery. C. Postoperative defect reconstructed with the superior-based nasal root island pedicle flap. FIGURE 2. A. Early cosmetic result 2 weeks postoperatively. B. C. Cosmetic result with no revision 6 months postoperatively. Conclusion: The nasal root island pedicle flap for repair of upper lateral nasal sidewall defect is a new application of a one-stage procedure that yields excellent functional and cosmetic result. Knowledge of the limitations and the anatomy of the flap are crucial for a good outcome. Commercial support: None identified.
Commercial support: None identified.
JUNE 2017
J AM ACAD DERMATOL
AB175