Journal of the American Academy of Dermatology
852 Brief communications
4. Moon NF. Synovial hemangioma of the knee joint: a review of previously reported cases and inclusion of two new cases. Clin Orthop 1973;90:183-90. 5. Enzinger FM, Weiss SW. Soft tissue tumors. 2nd ed. St Louis: CV Mosby, 1988:514-8.
Phaeohyphomycosis caused by Exserohilum rostratum mimicking hemorrhagic herpes zoster John M. Tieman, MD, and Bonnie B. Furner, MD San Antonio, Texas Fig. 2. Columnar cells suggestive of synovial cells (arrowheads) line the synovialhemangioma. (Hematoxylin-eosin stain; X450.) Trauma preceded 35% of the cases. The lesions were exclusively unilateral, with the knee as the most common site (97%). Clinical presentation included pain in 95%, recurrent joint effusions in 88%, limitation of motion in 56%, a palpable mass in 50%,musclewasting in 50%, cutaneous changes in 21%, and leg length discrepancy in 10%. Cutaneous changes included superficial vascular involvement, such as bluish mottled skin, telangiectases, vascular nevi, and cutaneous hemangiomas in 29 of 137 cases. Synovial hemangiomas can be subclassified into intraarticular, juxtaarticular, and mixed types.?The tumor is lined by the synovial membrane in the intraarticular synovial hemangioma, whereas the juxtaarticular type doesnot truly involve the synovialmembrane. The mixed form combines features of both intraarticular and juxtaarticular tumors. The most common is the intraarticular type. The tumor ischaracterized histologically by numerous large vascular spaces lined by endothelial cells and separated by an edematous, myxoid, or focally hyalinized stroma.!Inflammatory cellsare occasionallypresent.The synovium overlyingthe tumor is sometimes thrown into villous projections. Confinementby the synoviummay not be apparent.5 Surgical excision is the treatment ofchoice.Preoperativediagnosticstudies include radiography, angiography, phlebography, computed tomography, and, in tumors that involve the joint space, arthroscopy.' REFERENCES I. Bouchut ME. Tumeur erectile de I'articulation du genou. Gaz Hop Paris 1856;29:379. 2. Atkinson TJ, WolfS, Anavi Y, et al, Synovial hemangioma of the temporomandibular joint: report of a case and review of' the literature. J Oral MaxiUofac Surg 1988;46:804-8. 3. Paley D, Jackson RW. Synovial haemangioma ofthe knee joint: diagnosis by arthroscopy. Arthroscopy 1986;2:174-7.
Subcutaneous phaeohyphomycosis most commonly appearsas nodules or plaquesinan immunocompromised host.'? We report a caseof a patient whopresentedwith hemorrhagic vesicles in a zosteriformpattern at the site of previous intravenousline infiltration. Case report. A 74-year-old man with steroid-dependent chronic obstructive pulmonary disease was admitted to the hospital for exacerba tion of his pulmonary disease and upper gastrointestinal bleeding. On hospital day 14, an intravenous line infiltrated in his left forearm and 3 days later multiple vesicles developed at that site. Examination of the skin revealed multiple hemorrhagic vesicles 2 to 4 mm in diameter with areas of erosion and crusting in a dermatomal distribution on the extensor aspect of the left forearm, elbow, and, to a lesser extent, the volar aspect of the forearm (Fig. 1). A Tzanck smear was negative. A skin biopsy specimen revealed a subepidermal vesicle that contained numerous neutrophils and extravasated erythrocytes. Throughout the dermis there was a moderately dense mixed inflammatory cell .infiltrate within which were pigmented hyphal elements (Fig. 2). Fungal cultures revealed the causative organism to be Exserohilum rostratum. There was no evidence of systemic involvement. The patient was treated with oral ketoconazole, 200 mgtday, and topical c1otrimazolecream. Within 1 week no new vesicleswere noted and after 6 weeks of therapy the left arm was completely clear.
Discussion. Including the present case, four cases of subcutaneous phaeohyphomycosis caused by E. rostratum havebeen reported (TableI). Three of the four cases haveoccurredinimmunocompromised patients.Mostare preceded by trauma, whichpresumably creates the portal of entry for this opportunistic organism.Phaeohyphomycosis caused by E. rostratum has been treated with amphotericin B, ketoconazole, and a combinationof surgicalexcision and systemic antifungal medication.i" Our From the Division of Dermatology, The University of Texas Health ScienceCenter at San Antonio. Reprint requests: Bonnie B. Furner, MD, Division of Dermatology, Univ, of Texas at San Antonio, 7703 Floyd Curl Dr., San Antonio, TX 78284-7876. 16/4/31553
Volume 25 Number 5, Part 1
November 1991
Brief communications 853
Fig. 1. Hemorrhagic vesiclesin zosteriform distribution on posterior elbow and dorsolateral aspect of forearm.
Fig. 2. Branching fungal hyphae and spores. (Gomori-methenamine silver stains; X250.) Table I. Subcutaneous phaeohyphomycosis caused by Exserohilum rostraturn Case No. (reference)
Underlying disease/ immune status
Acute lymphocytic leukemia Cardiac transplant (prednisone, azathioprine) Immunocompetent Present case
Chronic obstructive pulmonary disease (prednisone)
Clinical presentation/ preceding trauma
Blue, necrotic macule and plaque at site of armboard Red, subcutaneous nodules on anterolateral aspect of leg (no known trauma) Erythematous plaque and nodule on leg at site of jellyfish sting Hemorrhagic vesicles at site of IV infiltration
Treatment
Amphotericin B Excision, amphotericin B, ketoconazole Excision, ketoconazole Ketoconazole
854 Brief communications
Journal of the American Academy of Dermatology
patient had complete resolution with a 6-week course of oral ketoconazole and topical clotrimazole cream. REFERENCES 1. McGinnis MR. Chromoblastomycosis and phaeohyphomycosis: New concepts, diagnosis, and mycology. JAM ACAD DBRMATOL 1983;8:1-16. 2. Estes SA, Merz WG, Maxwell LG. Primary cutaneous phaeohyphomycosis caused by Drechslera spicifera. Arch DermatoI1977;1l3:813-S. 3. Noel SB, Greer DL, Abadie SM, et al. Primary cutaneous phaeohyphomycosis.JAMAcAoDERMATOL 1988;18:102330. 4. Moneymaker CS, Shenep JL, Pearson TA, et al. Primary. cutaneous phaeohyphomycosis due to Exserohilum rostratum in a child with leukemia. Pediatr Infect Dis J 1986; 5:380-2. 5. McGinnis MR, Rinaldi MG, Winn RE. Emerging agents of phaeohyphomycosis: pathogenic species of Bipolaris and Exserohilum. J Clin Microbiol1986;24:250-9. 6. Burges GF, Walls CT, Maize JC. Subcutaneous phaeohyphornycosis caused by Exserohiium rostratum in an immunocompetent host. Arch DermatoI1987;123:1346-50.
Fig. 1. Angioma before treatment.
Successful treatment of Kasabach-Merrltt syndrome with pentoxifylline Y. de Prost," D. Teillac," C. Bodemer," O. Enjolras," C. Nihoul-Fekete," and D. de Prost?
Paris, France Kasabach-Merritt syndrome (KMS) is a variant of disseminated intravascular coagulation (DIC) in which platelets and clotting factors are locally consumed within a giant hemangioma. The cause of DIC is not clear but the blood is static in the venous sinusoids and both platelets and contact factors may be activated by the abnormal endothelium.' We have successfully treated a lO-monthold infant who hadKMS with pentoxifylline (PTX). PTX is a methylxanthine derivative that, in addition to restoring normal blood flow, shows significant antithrombotic properties. 2-5 Case report. A 2-month-old girl with a large angioma (Fig. 1) of the right forearm had thrombocytopenia (7000 to 30,000 cells/mm-) and a low fibrinogen level (0.90 gm/L), together with the presence of fibrin-fibrinogen degradation products (FDP) (>48 p,g/ml). The following treatments were ineffective on the size of the angioma and the hemostatic disturbances: systemic steroids (prednisone, 2 mg/kg/day, for 2 months), ticlopidine-aspirin (10 mg/kg/day, each, for 4 months), embolization of the branches of the radial and humoral arteries, radiation therapy (3 X 0.5 Gy), compression, and lnterferon/alfa 2b (daily subcutaneous injections of 3 M.U/m2 for 2 months). From the Department of Dermatology" and the Children's Surgical Clinic,b Hopltal Necker Enfants Malades; and the Department of Hematology and Immunology, INSERM U294 Hoplta! Bichat." Reprint requests: Yves de Prost, Dept. ofDermatology, Hopital Necker, 149 rue de Sevres, 75015 Paris, France.
16/4/31417
Fig. 2. Angioma after 2 months of treatment. PTX alone was administered orally after the failure of these treatments, at 100 mg/day (body weight, 8 kg). After 4 days the platelet count was 195,OOOjm3 and FDP were below 4 p,g/m!. The angioma progressively diminished in size, with surface blanching (Fig. 2). After 7 weeksof treatment the pIatelet count was 295,000/mm3• PTX was stopped after 3 months of treatment (platelet coun t 478,000/ mrn''), Seven da yslater, the count was 168,000jmm3 and another 7 days later, it was 103,000 (at this time, the hand and arm showed increased erythema). We then resumed pentoxifylline treatment at the same dose and 10 days later, the platelet count was 176,OOOjmm3 with concomitant decrease of the volume of the angioma.
Discussion. Many therapeutic modalities have been applied in Kasabach-Merritt syndrome.f PTX therapy appears to have resolved the DIC, with secondary improvement in the angioma. Furthermore, the platelet count decreased when we stopped the treatment and quickly returned to a normal value when we resumed PTX, Several mechanisms may contribute to this result. PTX has recently been shown to modulate a number of cell functions and displaces the hemostatic balance toward a decrease in procoagulant events." PTX inhibits
852 Brief communications
4. Moon NF. Synovial hemangioma of the knee joint: a review of previously reported cases and inclusion of two new cases. Clin Orthop 1973;90:183-90. 5. Enzinger FM, Weiss SW. Soft tissue tumors. 2nd ed. St Louis: CV Mosby, 1988:514-8.
Phaeohyphomycosis caused by Exserohilum rostratum mimicking hemorrhagic herpes zoster John M. Tieman, MD, and Bonnie B. Furner, MD San Antonio, Texas Fig. 2. Columnar cells suggestive of synovial cells (arrowheads) line the synovialhemangioma. (Hematoxylin-eosin stain; X450.) Trauma preceded 35% of the cases. The lesions were exclusively unilateral, with the knee as the most common site (97%). Clinical presentation included pain in 95%, recurrent joint effusions in 88%, limitation of motion in 56%, a palpable mass in 50%,musclewasting in 50%, cutaneous changes in 21%, and leg length discrepancy in 10%. Cutaneous changes included superficial vascular involvement, such as bluish mottled skin, telangiectases, vascular nevi, and cutaneous hemangiomas in 29 of 137 cases. Synovial hemangiomas can be subclassified into intraarticular, juxtaarticular, and mixed types.?The tumor is lined by the synovial membrane in the intraarticular synovial hemangioma, whereas the juxtaarticular type doesnot truly involve the synovialmembrane. The mixed form combines features of both intraarticular and juxtaarticular tumors. The most common is the intraarticular type. The tumor ischaracterized histologically by numerous large vascular spaces lined by endothelial cells and separated by an edematous, myxoid, or focally hyalinized stroma.!Inflammatory cellsare occasionallypresent.The synovium overlyingthe tumor is sometimes thrown into villous projections. Confinementby the synoviummay not be apparent.5 Surgical excision is the treatment ofchoice.Preoperativediagnosticstudies include radiography, angiography, phlebography, computed tomography, and, in tumors that involve the joint space, arthroscopy.' REFERENCES I. Bouchut ME. Tumeur erectile de I'articulation du genou. Gaz Hop Paris 1856;29:379. 2. Atkinson TJ, WolfS, Anavi Y, et al, Synovial hemangioma of the temporomandibular joint: report of a case and review of' the literature. J Oral MaxiUofac Surg 1988;46:804-8. 3. Paley D, Jackson RW. Synovial haemangioma ofthe knee joint: diagnosis by arthroscopy. Arthroscopy 1986;2:174-7.
Subcutaneous phaeohyphomycosis most commonly appearsas nodules or plaquesinan immunocompromised host.'? We report a caseof a patient whopresentedwith hemorrhagic vesicles in a zosteriformpattern at the site of previous intravenousline infiltration. Case report. A 74-year-old man with steroid-dependent chronic obstructive pulmonary disease was admitted to the hospital for exacerba tion of his pulmonary disease and upper gastrointestinal bleeding. On hospital day 14, an intravenous line infiltrated in his left forearm and 3 days later multiple vesicles developed at that site. Examination of the skin revealed multiple hemorrhagic vesicles 2 to 4 mm in diameter with areas of erosion and crusting in a dermatomal distribution on the extensor aspect of the left forearm, elbow, and, to a lesser extent, the volar aspect of the forearm (Fig. 1). A Tzanck smear was negative. A skin biopsy specimen revealed a subepidermal vesicle that contained numerous neutrophils and extravasated erythrocytes. Throughout the dermis there was a moderately dense mixed inflammatory cell .infiltrate within which were pigmented hyphal elements (Fig. 2). Fungal cultures revealed the causative organism to be Exserohilum rostratum. There was no evidence of systemic involvement. The patient was treated with oral ketoconazole, 200 mgtday, and topical c1otrimazolecream. Within 1 week no new vesicleswere noted and after 6 weeks of therapy the left arm was completely clear.
Discussion. Including the present case, four cases of subcutaneous phaeohyphomycosis caused by E. rostratum havebeen reported (TableI). Three of the four cases haveoccurredinimmunocompromised patients.Mostare preceded by trauma, whichpresumably creates the portal of entry for this opportunistic organism.Phaeohyphomycosis caused by E. rostratum has been treated with amphotericin B, ketoconazole, and a combinationof surgicalexcision and systemic antifungal medication.i" Our From the Division of Dermatology, The University of Texas Health ScienceCenter at San Antonio. Reprint requests: Bonnie B. Furner, MD, Division of Dermatology, Univ, of Texas at San Antonio, 7703 Floyd Curl Dr., San Antonio, TX 78284-7876. 16/4/31553
Volume 25 Number 5, Part 1
November 1991
Brief communications 853
Fig. 1. Hemorrhagic vesiclesin zosteriform distribution on posterior elbow and dorsolateral aspect of forearm.
Fig. 2. Branching fungal hyphae and spores. (Gomori-methenamine silver stains; X250.) Table I. Subcutaneous phaeohyphomycosis caused by Exserohilum rostraturn Case No. (reference)
Underlying disease/ immune status
Acute lymphocytic leukemia Cardiac transplant (prednisone, azathioprine) Immunocompetent Present case
Chronic obstructive pulmonary disease (prednisone)
Clinical presentation/ preceding trauma
Blue, necrotic macule and plaque at site of armboard Red, subcutaneous nodules on anterolateral aspect of leg (no known trauma) Erythematous plaque and nodule on leg at site of jellyfish sting Hemorrhagic vesicles at site of IV infiltration
Treatment
Amphotericin B Excision, amphotericin B, ketoconazole Excision, ketoconazole Ketoconazole
854 Brief communications
Journal of the American Academy of Dermatology
patient had complete resolution with a 6-week course of oral ketoconazole and topical clotrimazole cream. REFERENCES 1. McGinnis MR. Chromoblastomycosis and phaeohyphomycosis: New concepts, diagnosis, and mycology. JAM ACAD DBRMATOL 1983;8:1-16. 2. Estes SA, Merz WG, Maxwell LG. Primary cutaneous phaeohyphomycosis caused by Drechslera spicifera. Arch DermatoI1977;1l3:813-S. 3. Noel SB, Greer DL, Abadie SM, et al. Primary cutaneous phaeohyphomycosis.JAMAcAoDERMATOL 1988;18:102330. 4. Moneymaker CS, Shenep JL, Pearson TA, et al. Primary. cutaneous phaeohyphomycosis due to Exserohilum rostratum in a child with leukemia. Pediatr Infect Dis J 1986; 5:380-2. 5. McGinnis MR, Rinaldi MG, Winn RE. Emerging agents of phaeohyphomycosis: pathogenic species of Bipolaris and Exserohilum. J Clin Microbiol1986;24:250-9. 6. Burges GF, Walls CT, Maize JC. Subcutaneous phaeohyphornycosis caused by Exserohiium rostratum in an immunocompetent host. Arch DermatoI1987;123:1346-50.
Fig. 1. Angioma before treatment.
Successful treatment of Kasabach-Merrltt syndrome with pentoxifylline Y. de Prost," D. Teillac," C. Bodemer," O. Enjolras," C. Nihoul-Fekete," and D. de Prost?
Paris, France Kasabach-Merritt syndrome (KMS) is a variant of disseminated intravascular coagulation (DIC) in which platelets and clotting factors are locally consumed within a giant hemangioma. The cause of DIC is not clear but the blood is static in the venous sinusoids and both platelets and contact factors may be activated by the abnormal endothelium.' We have successfully treated a lO-monthold infant who hadKMS with pentoxifylline (PTX). PTX is a methylxanthine derivative that, in addition to restoring normal blood flow, shows significant antithrombotic properties. 2-5 Case report. A 2-month-old girl with a large angioma (Fig. 1) of the right forearm had thrombocytopenia (7000 to 30,000 cells/mm-) and a low fibrinogen level (0.90 gm/L), together with the presence of fibrin-fibrinogen degradation products (FDP) (>48 p,g/ml). The following treatments were ineffective on the size of the angioma and the hemostatic disturbances: systemic steroids (prednisone, 2 mg/kg/day, for 2 months), ticlopidine-aspirin (10 mg/kg/day, each, for 4 months), embolization of the branches of the radial and humoral arteries, radiation therapy (3 X 0.5 Gy), compression, and lnterferon/alfa 2b (daily subcutaneous injections of 3 M.U/m2 for 2 months). From the Department of Dermatology" and the Children's Surgical Clinic,b Hopltal Necker Enfants Malades; and the Department of Hematology and Immunology, INSERM U294 Hoplta! Bichat." Reprint requests: Yves de Prost, Dept. ofDermatology, Hopital Necker, 149 rue de Sevres, 75015 Paris, France.
16/4/31417
Fig. 2. Angioma after 2 months of treatment. PTX alone was administered orally after the failure of these treatments, at 100 mg/day (body weight, 8 kg). After 4 days the platelet count was 195,OOOjm3 and FDP were below 4 p,g/m!. The angioma progressively diminished in size, with surface blanching (Fig. 2). After 7 weeksof treatment the pIatelet count was 295,000/mm3• PTX was stopped after 3 months of treatment (platelet coun t 478,000/ mrn''), Seven da yslater, the count was 168,000jmm3 and another 7 days later, it was 103,000 (at this time, the hand and arm showed increased erythema). We then resumed pentoxifylline treatment at the same dose and 10 days later, the platelet count was 176,OOOjmm3 with concomitant decrease of the volume of the angioma.
Discussion. Many therapeutic modalities have been applied in Kasabach-Merritt syndrome.f PTX therapy appears to have resolved the DIC, with secondary improvement in the angioma. Furthermore, the platelet count decreased when we stopped the treatment and quickly returned to a normal value when we resumed PTX, Several mechanisms may contribute to this result. PTX has recently been shown to modulate a number of cell functions and displaces the hemostatic balance toward a decrease in procoagulant events." PTX inhibits
