| Unfamiliarity of physicians with chilblains (perniosis) gives rise to unnecessary hospital admissions with expensive laboratory and radiologic evaluations and, at times, hazardous therapy. Seven cases of chilblains were seen in san francisco from November 1986 through January 1987. The patients presented with pruritic, at times painful, purple acral patches or plaques on the fingers, toes, and nose after exposure to a cool or a cold, damp environment. Histologic examination in two cases revealed a perivascular lymphocytic infiltrate with endothelial swelling of the subcutaneous fat and of the upper and lower dermal plexus. ( info) |
12/20. anorexia nervosa associated with acromegaloid features, onset of acrocyanosis and Raynaud's phenomenon and worsening of chilblains. patients with anorexia nervosa may develop many physical and endocrinological complications. We wish to report two patients who developed soft tissue swelling of their hands and worsening of their peripheral vascular disease, evidenced by the appearance of acrocyanosis and Raynaud's phenomenon and more severe perniosis, following the onset of their anorexia nervosa. ( info) |
13/20. Necrobiotic pernio. A 21 year old man complained of pain and burning sensation on the helices of both ears, precipitated by cold weather. Examination showed tender erythematous nodules. Biopsies of both ears showed the characteristic vascular and inflammatory changes of pernio. In addition, in the left ear, there were foci of necrotic collagen surrounded by palisaded histiocytes. The presence of necrobiosis in pernio has not been reported previously. In this case, necrobiosis would appear to have a vascular basis. ( info) |
14/20. Rowell's syndrome. Report of a case. We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti-Sj-T antibodies found in the original Rowell's syndrome cases has been recently claimed. A review of the literature suggests that most of the cases of Rowell's syndrome described thus far in fact may be cases of coincidental association of lupus erythematosus and erythema multiforme. ( info) |
15/20. skin lesions from hypersensitivity to cold during chronic myelomonocytic leukaemia. A patient with chilblain lupus and chronic myelomonocytic leukaemia (CMML) is reported. The possible mechanisms of this association which appears specific to CMML are briefly discussed. ( info) |
16/20. Chronic pernio. A historical perspective of cold-induced vascular disease. Chronic pernio, or cold-induced vascular disease, is an uncommon syndrome with a clinical resemblance to vasculitis or peripheral cholesterol embolization. We studied a case of chronic pernio in which the angiogram revealed extensive vascular changes indistinguishable from vasculitis; pathologic studies of previously reported cases have revealed a primary vasculopathy. The pathophysiology of chronic pernio may be cold-induced vessel damage leading to a secondary dermopathy. Our patient's condition improved once the correct diagnosis was established and appropriate therapy was instituted. ( info) |
17/20. Pernio. A possible association with chronic myelomonocytic leukemia. Florid pernio occurred in an elderly man with chronic myelomonocytic leukemia (CMML). Previous case reports showed four elderly men with pernio and a hematologic disorder. Retrospective examination of the reported hematologic features of these cases enabled us to identify them as cases of CMML. Pernio preceded the diagnosis of the leukemia in all cases. We suggest that pernio may occur as an initial manifestation of CMML. The pathogenesis of pernio in association with CMML remains unclear. ( info) |
18/20. Unusual causes of arterial insufficiency. Most patients with vascular insufficiency in the legs suffer from atherosclerosis. Thus, it is easy to miss the diagnosis in occasional patients with disease due to other causes. Mechanical and functional disorders which affect major arteries or arterioles are described. ( info) |
| Several dermatologic abnormalities have been described in anorexia nervosa, but only rare associations have been made with perniosis. We recently saw two teenage girls and one woman with anorexia nervosa who had symptoms of perniosis. We suggest that altered thermoregulation and a hyperreactive peripheral vascular response to cold in anorexia nervosa may predispose these patients to perniosis. ( info) |
20/20. Chilblain lupus erythematosus of Hutchinson responding to surgical treatment: a report of two patients with anti-Ro/SS-A antibodies. We report two patients with chilblain lupus erythematosus of Hutchinson (CL) who responded to surgical treatment. One of them was a 72-year-old woman (case 1), and the other a 62-year-old man (case 2). We attempted to treat these patients by excising the lesions and subsequently performing full-thickness free skin grafting, using skin from the abdominal region. No recurrence was seen in the operated area 7 years (case 1) and 3 years (case 2) after surgery. However, lesions persisted in the areas not operated upon, and in the areas where lesions had not been adequately excised. These results suggest that surgical removal of local factors reduces the rash in these cases. In addition, both patients were serologically positive for the anti-Ro/SS-A antibody suggesting that local expression of the Ro/SS-A antigen may be involved in the pathogenesis of the skin lesions. To our knowledge, full thickness free skin grafting has not been used previously to treat CL-associated skin lesions, and is promising as a treatment for patients who do not respond to conventional means. ( info) |