INTERNAL MEDICINE DERMATOLOGY P2600 Atrophying scamous papulous dermatitis (Degos disease): Nine years of follow-up Jose´ Luis Iribas, MD, Hospital Dr. Jose´ M. Cullen, Santa Fe, Argentina; Adriana Albertengo, MD, Hospital J. B. Iturraspe, Santa Fe, Argentina; Jose´ Gabriel Casas, MD, Hospital Britanico, Ciudad Autonoma de Buenos Aires, Buenos Aires, Argentina Background: Degos disease was described in 1942 as atrophying scamous papulous dermatitis. It is a rare disease which, in most of the cases, starts as a cutaneous manifestation; it hardly ever begins with gastrointestinal or neurologic symptoms. Skin manifestation can occur only in 4% to 15% of the cases. The main cause of death is the involvement of gastrointestinal organs and the second is the neurologic as well. Its etiology is unknown, its thrombotic patogenia is possible. Nowadays, for some authors, it is considered within the lupus erythematouss (LE) spectrum and also it was described during the evolution of the systemic scleroderm, dermatomyositis, AIDS, and antiphospholipid antibody syndrome. Panniculitis lesions were described during its evolution. Case report: We present a 36-year-old woman after 9 years of evolution. Her disease started as a panniculitis in buttocks and thigs areas, and its histopathology was compatible with LE. After these lesions, she started with clinical typical Degos lesions disease, with thrombotic findings in a histopathology and dermal necrosis. During her evolution she developed polymyositis with a positive response to the steroid treatment. The last years she presented new typical lessons above right knee and bilateral involvement of ankles that developed in deep ulcers without any general symptoms. She has extensive calcinosis cutis and lipodystrophy. The patient was pregnant twice during her evolution, without any problems for her and for her children. She has been taking antiplatelet, antipaludic drugs, and pentoxifiline (a TNF inhibitor), except during her pregnancy. She is currently under medical control without any systemic complications. Commercial support: None identified.
P2602 Graft versus host diseaseelike: A new paraneoplastic syndrome Diana Garza-Salazar, MD, Hospital Universitario ‘‘Dr Jose´ E. Gonza´lez,’’ Monterrey, Nuevo Leo´n, Mexico; Alberto De la Fuente-Garcı´a, MD, Hospital Universitario ‘‘Dr Jose´ E. Gonza´lez,’’ Monterrey, Nuevo Leo´n, Mexico; Ivett Miranda-Maldonado, ´ n, Mexico; Jorge OcampoMD, Hospital Universitario, Monterrey, Nuevo Leo ´ n, Mexico; Minerva Candiani, MD, Hospital Universitario, Monterrey, Nuevo Leo ´ mez-Flores, MD, Hospital Universitario, Monterrey, Nuevo Leo´n, Mexico Go Introduction: Thymoma is the most frequent thymic malignancy; it is associated with various neoplasic syndromes, being myasthenia gravis the most frequent. In medical literature, it has been reported only seven cases of thymoma associated with acute graft versus host disease like. Case report: A 39-year-old Hispanic woman diagnosed with myasthenia gravis 10 years ago was managed with pyridostigmine. Soon after, a thymoma was diagnosed and thymectomy was performed without complications. She presented 2 years ago at another dermatology clinic with erythematous scaling patches and pruritus which was first confined to her legs an that later progressed to erythrodermia. A diagnosis of psoriasis was established and treatment was initiated with antihystaminics and topical steroid, with poor response to treatment. Later on, her plaques developed hyperpigmentation and sclerodermoid changes. Six months before her first visit to our department, she suffered from generalized hair loss, oral mucous ulcerations, and episodes of self-limited diarrhea. Laboratory tests evidenced normocitic normocromic anemia, elevated levels of alkaline phosphatase and also lactose dehydrogenase. A skin biopsy specimen was taken and showed typical changes of chronic graft versus host disease. Based on her background history, clinical presentation, and histopathology, a diagnosis of thymoma associated with chronic graft versus host diseaseelike was established. Discussion: Thymoma tumors have an incidence of 0.15 cases per 100000 people, predominantly affecting males between their fourth and sixth decades of life. Graft versus host disease presents in patients with bone marrow transplant. There are many cell populations responsible for this disease, being T-lymphocytes the most relevant. The presence of autoimmunity in patients with thymoma should be considered because this organ plays a major role in T-lymphocytes maturation. There have been only seven case reports of graft versus host disease associated with thymoma, which presented with acute cutaneous and gastrointestinal symptoms. Recently AIRE (autoimmune regulator gene) has been described and could help in understanding the scenario of these autoimmune diseases. In this case, the presentation was a chronic form of graft versus host disease. To our knowledge, this is the first case described of a chronic presentation. Commercial support: None identified.
Results: AGA patients showed significant higher systolic BP values (139.43 vs 107.80mm Hg; P\.0001), diastolic BP values (87.65 vs 67.48mm Hg; P \.0001) and aldosterone levels (249.55 vs 155.14pg/mL; P ¼.002) versus controls, respectively. A positive relationship was observed between blood pressure and higher aldosterone values in women with AGA (r ¼ 0.337, P ¼.033 for systolic blood pressure and r ¼ 0.210, P ¼ .194 for diastolic BP). Conclusion: A higher prevalence of hypertension in women with AGA has been found. The elevated aldosterone values in these patients may contribute, alongside other mechanisms, to the development of AGA and may also explain the higher prevalence of hypertension. Aldosterone antagonists, have been used for a long time for female AGA and may exert a dual beneficial effect in hypertensive AGA patients, controlling BP and preventing alopecia progression, especially if taken in early stages.
Nephrogenic systemic fibrosis: Report of two cases Yann Charli-Joseph, MD, Instituto Nacional de Ciencias Me´dicas y Nutricio´ n Salvador Zubira´n, Mexico City, Distrito Federal, Mexico; Ana RuelasVillavicencio, MD, Instituto Nacional de Ciencias Me´dicas y Nutricio´ n Salvador Zubira´n, Mexico City, Distrito Federal, Mexico; Linda Garcı´a-Hidalgo, MD, ´ n Salvador Zubira´n, Mexico Instituto Nacional de Ciencias Me´dicas y Nutricio City, Distrito Federal, Mexico; Rocı´o Orozco-Topete, MD, Instituto Nacional de Ciencias Me´dicas y Nutricio´n Salvador Zubira´n, Mexico City, Distrito Federal, Mexico Nephrogenic systemic fibrosis (NSF) is an emerging disorder exclusive of patients with renal disease, but its precise etiology is yet unknown. So far, only 315 cases have been reported. Thickening of the skin coincides with progressive multisystemic tissue collagen deposition. There is no effective standard treatment, but correction of glomerular function can alleviate the disease. Recently, a clear epidemiologic association with gadolinium chelateebased contrast agents (GdCA) has been reported. We describe the features of two patients recently diagnosed with NSF: patient A is a 38-year-old woman with end-stage renal disease (ESRD) secondary to eclampsia, renal transplantation failure, and currently on hemodialysis. She acquired primary cutaneous cryptococcosis and was submitted to MRI in order to rule out osteomielitis. Ten days later, she developed confluent thickened erythematous patches in distal limbs. A biopsy specimen revealed septal granulomatose paniculitis, extensive dermal fibrosis, and an increased number of CD341 spindle cells, consistent with NSF. Patient B is a 50-year-old man with ESRD with associated hyperparathyroidism and cardiomiopathy secondary to hypertension under treatment with peritoneal dialysis, erythropoietin, calcium and iron. Two weeks after a cardiac MRI performed to study progressive dyspnea he developed symmetric, indurated, edematose yellowish patches in both arms and legs. A punch biopsy specimen was consistent with NSF. The clinicopathologic characteristics of these patients are consistent with literature reports on NSF. Both cases reaffirm the association with Gd-CA, which in the presence of ESRD have an increased half-life, and are thought to transmetallate (free gadolinium ion is released from the chelate in exchange for endogenous metals) with subsecuent binding of gadolinium to human tissue and promotion of fibrosis. Furthermore, our findings support the requirement of co-factors to generate a suitable environment for fibrosis (erythropoietin, proinflammatory conditions such as infection, or metals that facilitate transmetallation). Finally, the importance of recognizing this disease and avoiding GdCA in patients with ESRD cannot be overemphasized.
Commercial support: None identified.
Commercial support: None identified.
P2601 Blood pressure levels in women with androgenetic alopecia Salvador Arias-Santiago, MD, San Cecilio Clinical Hospital, Granada, Spain; Husein Husein-El-Ahmed, MD, San Cecilio Clinical Hospital, Granada, Spain; Jose Aneiros-Ferna´ndez, MD, San Cecilio Hospital, Granada, Spain; Marı´a Teresa Gutie´rrez-Salmero´n, MD, San Cecilio Hospitai, Granada, Spain; Ramo´n NaranjoSintes, MD, San Cecilio Hospital, Granada, Spain Introduction: Androgenic alopecia (AGA) is the most common type of alopecia both in men and in women, but its prevalence and pathogenic mechanisms have been mainly investigated in men. Few studies have analyzed the relationship between AGA in women and cardiovascular disease. There is reported to be an elevated prevalence of hypertension among men with AGA, and it has been proposed that both phenomena may be explained by the presence of hyperaldosteronism. However, no data on blood pressure (BP) and aldosterone levels in women with AGA have been published to date. The objective of this study was to evaluate aldosterone levels and the presence of systolic and diastolic hypertension in women with early-onset AGA and in healthy controls. Methods: This case-control study included 40 women with AGA and 40 healthy controls from the Dermatology Department of San Cecilio Hospital, Granada (Spain).
J AM ACAD DERMATOL