Victoria Werth MD, Professor, Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA on October 1, 2010
Would strongly consider antiphospholipid syndrome. aPTT can be prolonged because of the autoantibodies. The mottled skin findings are consistent and she could be have pulmonary emboli as a cause her pleuritic chest pain. Would check a panel: aPTT, RVVT, lupus anticoagulant, anti-phospholipid antibody, to rule this out.
If positive, she needs evaluation for pulmonary emboli, and further treatment for this and antiphospholipid syndrome.
Khalid el-Aboud MD, Medical Director and Consultant Dermatologist, King Faisal Hospital, Makkah, Saudi Arabia on October 1, 2010
Among the differential diagnoses, I want to draw the attention of the authors to the possibility of factitial disorder in this case. The general clue is that, when the pattern of presentation does not fit with common diseases, one may think about self-induced changes. This patient might have a Secrétan's syndrome.
Secrétan's disease is an injury that is self-inflicted either for secondary gain or as a conversion reaction and present with limb edema that might be associated with vascular changes. It is best treated with conservative care and psychiatric counseling. It was first described in 1901 as "hard edema and traumatic hyperplasia of the dorsum of the metacarpus." Lower limb involvement can occur in Secretion's disease.
Needless to say that other differential diagnoses to be considered in this case include:
Connective tissue diseases like SLE, dermatomyositis, scleroderma
Blood diseases like cold agglutinin diseases, antiphospholipid syndrome.
Disease related to the cold exposure like cryoglobulinemia and frost bite.
Reynaud's disease
Vascular diseases like Buerger disease
Exanthema related to viral infection.
For further reading about Secrétan's disease please see the following references. Moretta DN , Cooley RD Jr. Secrétan's disease: a unique case report and literature review. Am J Orthop (Belle Mead NJ). 2002 Sep; 31(9):524-7.
Winkelmann RK , Barker SM. Factitial traumatic panniculitis. J Am Acad Dermatol. 1985 Dec; 13(6):988-94.
Abnousi F , Chou LB. Secretan's disease of the foot: a case report and review. Foot Ankle Int. 2008 Feb; 29(2):248-50.
Michael LaCombe MD, Internal Medicine Physician, Maine General Medical Center, Augusta, ME, USA on October 2, 2010
Lacking: Blood pressure measurements in both arms; chest
auscultation for pleural friction rubs; examination of digital nail folds for thrombi;
d-dimer assay; echocardiogram for PA systolic pressures and evidence of RV pressure
overload
Most likely APL syndrome with multiple pulmonary
emboli
el-Azhary, Rokea A., M.D., Ph.D, Professor of Dermatology, Mayo School of Medicine, Rochester, Minnesota, USA on October 2, 2010
The clinical cutaneous pattern is linear scleroderma. Have seen it before and looks early on very much like a vascular process. Can't explain the pleuritic chest pain, unless it is related to the sclerodermatous tightening process.
Julianne Mann MD, Dermatology resident, Oregon Health and Science University, Portland, OR, USA on October 3, 2010
Thank you to everyone who has posted.
Some additional information: the patient was managed while in house by the family medicine service who reported no friction rubs. Blood pressures in both arms were normal. There was one tiny splinter hemorrhage in one fingernail plate, nailfolds were normal.
We too strongly considered anti-phospholipid antibody syndrome. Anti-cardiolipin antibodies and lupus anticoagulant were ordered but for some reason were not performed while the patient was in house, so she will be having these done this coming week.
MRI of the L upper extremity yesterday was normal.
Yoon Cohen, University of New England College of Osteopathic Medicine, Portland, Maine, USA on October 5, 2010
I'm just wondering if we have ruled out erythromelalgia if I may share my thoughts on this case.
While it commonly presents in the distal extremities and bilaterally, there are cases reported that it could present unilaterally, particularly in secondary erythromelalgia. For example with myeloproliferative disorder. In that case symptoms could precede diagnosis of the myeloproliferative disorder by about 2.5 years [1] so she does not necessarily have abnormal CBC today. Dramatic relief with aspirin or NSAIDs could be typical of this type according to the reference.
Thank you for letting me participate in the discussion.
Raed Alsmadi MD, Consultant Dermatologist, Amman, Jordan on October 6, 2010
Very good case I think a rheumatologist should be consulted. I think this can be a vascular manifestation of Behcet's disease where we can have pulmonary lesion include thrombosis in the superior vena cava, or mediastinal vein, aortic or pulmonary arterial aneurysm, pulmonary infarct, hemorrage, pleural effusion, focal or diffuse pulmonary fibrosis as caused by vasovasorum vasculitis of pulmonary vessels. Also the upper extremities have arterial occlusion.
Bashir Zendah MD, Senior Registrar, Department of Dermatology, Tripoli Medical Centre, Tripoli, Libya on October 9, 2010
Good case to do a skin biopsy and exclude urticaria vasculitis and its associations.
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