Case report 419
Compartment syndrome because of acute hemorrhagic edema of infancy: a case report and literature review Miri Dotana,d, Elhanan Nahumb,d, Daniel Weiglc,d and Efraim Bilavskya,d Acute hemorrhagic edema of infancy (AHEI) is a benign, self-limiting vasculitis that usually resolves completely without any sequelae or a need for active therapy. To our knowledge, compartment syndrome because of AHEI has not been reported. Chart data for a single case were reviewed and reported in a retrospective study. A 19month-old male presented with petechial rash and swelling of the left lower leg. AHEI was diagnosed clinically and confirmed by skin biopsy. On the basis of the clinical appearance, compartment syndrome of the foot was suspected. Measurements of compartmental pressures in the foot were well above the commonly cited ranges and a fasciotomy was performed. Following the operation, there was a marked clinical improvement in the limb perfusion. The child was discharged on the 20th day with marked clinical improvement; both active and passive
leg movements were intact. We suggest that pediatric orthopedic surgeons should be familiar with this entity and its rare complication. Level of evidence: V – case c 2014 Wolters report. J Pediatr Orthop B 23:419–421 Kluwer Health | Lippincott Williams & Wilkins.
Introduction
Medical history showed an in-vitro fertilization pregnancy, term cesarean section delivery because of fetal distress and neonatal depression by Apgar score 3/5. He required mechanical ventilation for 3 h in the neonatal intensive care unit without neurological or any other sequelae. The parents reported an upper respiratory tract infection 1 week before his current presentation.
Acute hemorrhagic edema of infancy (AHEI) is a leukocytoclastic small-vessel vasculitis. Although most consider AHEI to be a separate entity from Henoch–Schonlein purpura (HSP), others believe that they are different clinical presentations of the same disease [1]. The syndrome is more common in boys 6–24 months of age and is associated with a history of recent respiratory illness [2]. Unlike HSP, AHEI is mostly confined to the skin and rarely involves internal organs. Complete resolution without sequelae is expected, with or without treatment. In this case report, we present a rare case of AHEI that resulted in compartment syndrome of the lower limb.
Case report A 19-month-old previously healthy boy presented to our institutional emergency department with low-grade fever (maximum 38.21C) and restlessness, combined with swelling of the left calf and ankle. On admission, the child was alert, irritable, and nontoxic. Blood pressure was 110/46 mmHg, heart rate was 172 bpm, rectal temperature was 38.21C, and weight was 9.5 kg. Positive findings included a swollen left calf and ankle with induration, and tenderness on palpation, more prominent on the anterolateral aspect. There was also mild swelling of the right calf and ankle. A petechial rash was noted on the plantar aspect of both feet and on the hard palate. The rest of the physical examination, including a complete neurological examination, was unremarkable. c 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins 1060-152X
Journal of Pediatric Orthopaedics B 2014, 23:419–421 Keywords: acute hemorrhagic edema of infancy, compartment syndrome, fasciotomy Departments of aPediatrics C, bPediatric Intensive Care, cPediatric Orthopedic Unit, Schneider Children’s Medical Center of Israel, Petach Tikva and dSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel Correspondence to Miri Dotan, BMedSc, Department of Pediatrics C, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, Petach Tikva 49202, Israel Tel: + 972 3 925 3775; + 972 54 5844818; fax: + 972 3 925 3801; e-mail: [email protected]
Complete blood count showed a hemoglobin level of 11.2 g/dl (normal range 10.5–14 g/dl); the white blood cell count was 16 760/mm3 with 76% neutrophils, 16% lymphocytes, 4% monocytes, and 1% eosinophils. Mild thrombocytosis of 576 000/ml was also noted. Levels of serum electrolytes, kidney function tests, and liver enzymes were within the normal range. C-reactive protein was 2.83 mg/dl (normal range 0–0.5 mg/dl). Radiographic films of the left calf indicated no pathological findings. Because of fever, petechial rash, and the musculoskeletal findings, the child was treated empirically with intravenous ceftriaxone, cloxacillin, and clindamycin. He also received intravenous fluids. After several hours of hospitalization, there was a gradual increase in the circumference of the left lower leg, the limb was cooler compared with the right, and there was a decrease in the peripheral pulses, indicating compromised tissue perfusion. His rash advanced with edema and new purpuric lesions developed on both legs. C-reactive protein increased to 15.79 mg/dl. At this point, the child was admitted to the pediatric intensive care unit. Fundus examination did not indicate pathological findings; abdominal ultrasound and echocardiogram were normal and a skin biopsy was performed. DOI: 10.1097/BPB.0000000000000060
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
420 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
As the swelling and skin tension of the left lower limb increased, an ultrasound and Doppler were performed and showed severe soft tissue swelling with normal arterial and venous circulation. At this point, on the basis of the clinical appearance of the foot, a compartment syndrome of the foot was suspected. The patient was brought to the operating room and administered general anesthesia. Measurements of compartmental pressures in the foot were taken using arterial line manometry connected directly to the needle. These were shown to be well above the commonly cited ranges of compartment syndrome [3] (see below), and the diagnosis of compartment syndrome of the foot was established. Fasciotomy of all nine foot compartments was performed utilizing two parallel dorsal incisions for the dorsal and all interossei compartments and one longitudinal incision on the medial aspect of the foot for the plantar compartments. Intracompartmental pressures decreased immediately with clinical improvement in tissue circulation. The pre fasciotomy compartmental pressures in the interossei, dorsal, plantar medial, and plantar lateral compartments were 65, 70, 58, and 60 mmHg, respectively. Immediately after fasciotomy, these compartmental pressures were 35, 37, 28, and 44 mmHg, respectively. The surgical wounds were treated by frequent wet saline dressing changes. Primary closure of all wounds was performed on day 4 after surgery. Following the operation, there was a marked clinical improvement in the limb perfusion. Intravenous steroid treatment with methylprednisolone 2 mg/kg per day was also initiated. After 3 days of hospitalization and because of the fact that no organism was found on blood culture, antibiotic treatment was changed to cefuroxime. The next day, the skin biopsy that was performed earlier showed leukocytoclastic vasculitis consistent with the diagnosis of AHEI. On the fourth day of hospitalization, severe swelling of the right hand was noted. Intracompartmental pressures were elevated, but because of the lack of clinical suspicion of compartment syndrome and spontaneous improvement within 1 day, no intervention was performed. The child was discharged on the 20th day with marked clinical improvement; both active and passive leg movements were intact, and the rash had subsided. On the follow-up examination after discharge, the child was afebrile, with no clinical signs of wound infection at the site of fasciotomy; he had full range of motion of the left leg. At 4 years of age, the child was diagnosed with familial Mediterranean fever (FMF) because of recurrent episodes of fever and abdominal pain. The child was found
to be positive for M694V and V726A mutations of the MEFV gene and treatment with colchicine was started.
Discussion We report a case of AHEI who presented with a rare complication of compartment syndrome of the lower leg that required a fasciotomy. To the best of our knowledge, this is the first time such a complication has been described in the literature. AHEI (Finkelstein’s disease) is an acute leukocytoclastic vasculitis that most American authors identify as a variant of HSP. However, in the European (non-English) medical literature, it is believed to be a distinct clinical entity [4]. In 2009, Fiore et al. [2] published a systematic review of the literature that found a total of 287 patients in articles published since 1912. This relatively low number may be the result of true low incidence, underdiagnosis, or confusion with other disorders [4]. AHEI is more common in boys, 6–24 months of age, and often follows a history of recent respiratory illness with or without a course of antibiotics [2]. The onset of AHEI is acute and may be associated with fever, and tender symmetric edematous areas over the cheeks, ears, and extremities, with relative sparing of the trunk. Subsequently, purpura develops in the areas of pre-existing edema. It has been suggested that the distribution of the purpura is gravity dependent [4]. Unlike HSP, the course of AHEI is benign and complete recovery usually occurs within 2–3 weeks. Recurrent episodes have rarely been reported. Histological study showed leukocytoclastic vasculitis, but in contrast to HSP, perivascular deposits of immunoglobulin A are detected in only 10±35% of AHEI patients [4]. In addition to HSP, the differential diagnosis of AHEI includes meningococcemia, erythema multiforme, and drug eruption. However, the main differences between AHEI and HSP are that AHEI patients are younger (usually not older than 2 years), fever is common, the distribution of the rash includes the face and auricles, edema is almost always present, perivascular deposits of immunoglobulin A are infrequent, and renal involvement and long-standing complications are rare [5]. An involvement of body systems other than skin was reported in no more than 25 patients; this included the kidneys (glomerulonephritis, hematuria, and proteinuria), bowel (isolated abdominal pain, intestinal bleeding, intussusception), joints (arthralgias, arthritis), and genitals (testicular torsion) [2]. Severe soft tissue involvement was never reported before in children with AHEI. Compartment syndrome is a clinical condition characterized by elevated pressure within a confined fascial space, causing circulatory compromise, ischemia, and ultimately tissue necrosis [6].
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Compartment syndrome because of AHEI Dotan et al. 421
On reviewing the literature, two cases of HSP and compartment syndrome were found. However, in one case, the child also had a bleeding diathesis (severe factor XIII deficiency) and the compartment syndrome was attributed to a massive hematoma secondary to the continuous bleeding after several attempts of intravenous cannulation [7]. In the second case, the compartment syndrome developed after the removal of an arterial line that might have triggered the event [8]. To our knowledge, there are no reported cases of AHEI involving compartment syndrome. We believe that the edema in this case was so severe that it resulted in increased pressure, leading to the compartment syndrome. Another interesting point in this case is the further diagnosis of the child with FMF. FMF is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, or arthritis [9]. FMF was associated with several vasculic diseases including HSP [10]. Children with FMF tend to have a more severe course of HSP. Thus, the link that exists between FMF and the severity of HSP could also explain the severe and extraordinary complication of this seemingly benign disease in our described case. In conclusion, despite the fact that most cases of pediatric compartment syndrome are traumatic or postoperative in nature [6], orthopedic surgeons should be
aware of other medical etiologies such as vasculitis for compartment syndrome.
Acknowledgements Conflicts of interest
There are no conflicts of interest.
References 1
AlSufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol 2009; 48:617–622. 2 Fiore E, Rizzi M, Ragazzi M, Vanoni F, Bernasconi M, Bianchetti MG, Simonetti GD. Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol 2008; 59:684–695. 3 Whitesides TE, Haney TC, Morimoto K, Harada H. Tissue pressure measurements as a determinant for the need of fasciotomy. Clin Orthop Relat Res 1975; 113:43–51. 4 Garty BZ, Ofer I, Finkelstein Y. Acute hemorrhagic edema of infancy. Isr Med Assoc J 2002; 4:228–229. 5 Cunningham BB, Caro WA, Eramo LR. Neonatal acute hemorrhagic edema of childhood: case report and review of the English-language literature. Pediatr Dermatol 1996; 13:39–44. 6 Bae DS, Kadiyala RK, Waters PM. Acute compartment syndrome in children: contemporary diagnosis, treatment, and outcome. J Pediatr Orthop 2001; 21:680–688. 7 Alioglu B, Ozsoy MH, Tapci E, Karamercan S, Agras PI, Dallar Y. Successful use of recombinant factor VIIa in a child with Schoenlein–Henoch purpura presenting with compartment syndrome and severe factor XIII deficiency. Blood Coagul Fibrinolysis 2013; 24:102–105. 8 Abidin AS, Jalaluddin MY. Left hand compartment syndrome: a rare complication of Henoch Schonlein purpura. J Paediatr Child Health 2008; 44:385. 9 Aksu K, Keser G. Coexistence of vasculitides with familial Mediterranean fever. Rheumatol Int 2011; 31:1263–1274. 10 Flatau E, Kohn D, Schiller D, Lurie M, Levy E. Scho¨nlein–Henoch syndrome in patients with familial Mediterranean fever. Arthritis Rheum 1982; 25:42–47.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Compartment syndrome because of acute hemorrhagic edema of infancy: a case report and literature review Miri Dotana,d, Elhanan Nahumb,d, Daniel Weiglc,d and Efraim Bilavskya,d Acute hemorrhagic edema of infancy (AHEI) is a benign, self-limiting vasculitis that usually resolves completely without any sequelae or a need for active therapy. To our knowledge, compartment syndrome because of AHEI has not been reported. Chart data for a single case were reviewed and reported in a retrospective study. A 19month-old male presented with petechial rash and swelling of the left lower leg. AHEI was diagnosed clinically and confirmed by skin biopsy. On the basis of the clinical appearance, compartment syndrome of the foot was suspected. Measurements of compartmental pressures in the foot were well above the commonly cited ranges and a fasciotomy was performed. Following the operation, there was a marked clinical improvement in the limb perfusion. The child was discharged on the 20th day with marked clinical improvement; both active and passive
leg movements were intact. We suggest that pediatric orthopedic surgeons should be familiar with this entity and its rare complication. Level of evidence: V – case c 2014 Wolters report. J Pediatr Orthop B 23:419–421 Kluwer Health | Lippincott Williams & Wilkins.
Introduction
Medical history showed an in-vitro fertilization pregnancy, term cesarean section delivery because of fetal distress and neonatal depression by Apgar score 3/5. He required mechanical ventilation for 3 h in the neonatal intensive care unit without neurological or any other sequelae. The parents reported an upper respiratory tract infection 1 week before his current presentation.
Acute hemorrhagic edema of infancy (AHEI) is a leukocytoclastic small-vessel vasculitis. Although most consider AHEI to be a separate entity from Henoch–Schonlein purpura (HSP), others believe that they are different clinical presentations of the same disease [1]. The syndrome is more common in boys 6–24 months of age and is associated with a history of recent respiratory illness [2]. Unlike HSP, AHEI is mostly confined to the skin and rarely involves internal organs. Complete resolution without sequelae is expected, with or without treatment. In this case report, we present a rare case of AHEI that resulted in compartment syndrome of the lower limb.
Case report A 19-month-old previously healthy boy presented to our institutional emergency department with low-grade fever (maximum 38.21C) and restlessness, combined with swelling of the left calf and ankle. On admission, the child was alert, irritable, and nontoxic. Blood pressure was 110/46 mmHg, heart rate was 172 bpm, rectal temperature was 38.21C, and weight was 9.5 kg. Positive findings included a swollen left calf and ankle with induration, and tenderness on palpation, more prominent on the anterolateral aspect. There was also mild swelling of the right calf and ankle. A petechial rash was noted on the plantar aspect of both feet and on the hard palate. The rest of the physical examination, including a complete neurological examination, was unremarkable. c 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins 1060-152X
Journal of Pediatric Orthopaedics B 2014, 23:419–421 Keywords: acute hemorrhagic edema of infancy, compartment syndrome, fasciotomy Departments of aPediatrics C, bPediatric Intensive Care, cPediatric Orthopedic Unit, Schneider Children’s Medical Center of Israel, Petach Tikva and dSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel Correspondence to Miri Dotan, BMedSc, Department of Pediatrics C, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, Petach Tikva 49202, Israel Tel: + 972 3 925 3775; + 972 54 5844818; fax: + 972 3 925 3801; e-mail: [email protected]
Complete blood count showed a hemoglobin level of 11.2 g/dl (normal range 10.5–14 g/dl); the white blood cell count was 16 760/mm3 with 76% neutrophils, 16% lymphocytes, 4% monocytes, and 1% eosinophils. Mild thrombocytosis of 576 000/ml was also noted. Levels of serum electrolytes, kidney function tests, and liver enzymes were within the normal range. C-reactive protein was 2.83 mg/dl (normal range 0–0.5 mg/dl). Radiographic films of the left calf indicated no pathological findings. Because of fever, petechial rash, and the musculoskeletal findings, the child was treated empirically with intravenous ceftriaxone, cloxacillin, and clindamycin. He also received intravenous fluids. After several hours of hospitalization, there was a gradual increase in the circumference of the left lower leg, the limb was cooler compared with the right, and there was a decrease in the peripheral pulses, indicating compromised tissue perfusion. His rash advanced with edema and new purpuric lesions developed on both legs. C-reactive protein increased to 15.79 mg/dl. At this point, the child was admitted to the pediatric intensive care unit. Fundus examination did not indicate pathological findings; abdominal ultrasound and echocardiogram were normal and a skin biopsy was performed. DOI: 10.1097/BPB.0000000000000060
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
420 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 5
As the swelling and skin tension of the left lower limb increased, an ultrasound and Doppler were performed and showed severe soft tissue swelling with normal arterial and venous circulation. At this point, on the basis of the clinical appearance of the foot, a compartment syndrome of the foot was suspected. The patient was brought to the operating room and administered general anesthesia. Measurements of compartmental pressures in the foot were taken using arterial line manometry connected directly to the needle. These were shown to be well above the commonly cited ranges of compartment syndrome [3] (see below), and the diagnosis of compartment syndrome of the foot was established. Fasciotomy of all nine foot compartments was performed utilizing two parallel dorsal incisions for the dorsal and all interossei compartments and one longitudinal incision on the medial aspect of the foot for the plantar compartments. Intracompartmental pressures decreased immediately with clinical improvement in tissue circulation. The pre fasciotomy compartmental pressures in the interossei, dorsal, plantar medial, and plantar lateral compartments were 65, 70, 58, and 60 mmHg, respectively. Immediately after fasciotomy, these compartmental pressures were 35, 37, 28, and 44 mmHg, respectively. The surgical wounds were treated by frequent wet saline dressing changes. Primary closure of all wounds was performed on day 4 after surgery. Following the operation, there was a marked clinical improvement in the limb perfusion. Intravenous steroid treatment with methylprednisolone 2 mg/kg per day was also initiated. After 3 days of hospitalization and because of the fact that no organism was found on blood culture, antibiotic treatment was changed to cefuroxime. The next day, the skin biopsy that was performed earlier showed leukocytoclastic vasculitis consistent with the diagnosis of AHEI. On the fourth day of hospitalization, severe swelling of the right hand was noted. Intracompartmental pressures were elevated, but because of the lack of clinical suspicion of compartment syndrome and spontaneous improvement within 1 day, no intervention was performed. The child was discharged on the 20th day with marked clinical improvement; both active and passive leg movements were intact, and the rash had subsided. On the follow-up examination after discharge, the child was afebrile, with no clinical signs of wound infection at the site of fasciotomy; he had full range of motion of the left leg. At 4 years of age, the child was diagnosed with familial Mediterranean fever (FMF) because of recurrent episodes of fever and abdominal pain. The child was found
to be positive for M694V and V726A mutations of the MEFV gene and treatment with colchicine was started.
Discussion We report a case of AHEI who presented with a rare complication of compartment syndrome of the lower leg that required a fasciotomy. To the best of our knowledge, this is the first time such a complication has been described in the literature. AHEI (Finkelstein’s disease) is an acute leukocytoclastic vasculitis that most American authors identify as a variant of HSP. However, in the European (non-English) medical literature, it is believed to be a distinct clinical entity [4]. In 2009, Fiore et al. [2] published a systematic review of the literature that found a total of 287 patients in articles published since 1912. This relatively low number may be the result of true low incidence, underdiagnosis, or confusion with other disorders [4]. AHEI is more common in boys, 6–24 months of age, and often follows a history of recent respiratory illness with or without a course of antibiotics [2]. The onset of AHEI is acute and may be associated with fever, and tender symmetric edematous areas over the cheeks, ears, and extremities, with relative sparing of the trunk. Subsequently, purpura develops in the areas of pre-existing edema. It has been suggested that the distribution of the purpura is gravity dependent [4]. Unlike HSP, the course of AHEI is benign and complete recovery usually occurs within 2–3 weeks. Recurrent episodes have rarely been reported. Histological study showed leukocytoclastic vasculitis, but in contrast to HSP, perivascular deposits of immunoglobulin A are detected in only 10±35% of AHEI patients [4]. In addition to HSP, the differential diagnosis of AHEI includes meningococcemia, erythema multiforme, and drug eruption. However, the main differences between AHEI and HSP are that AHEI patients are younger (usually not older than 2 years), fever is common, the distribution of the rash includes the face and auricles, edema is almost always present, perivascular deposits of immunoglobulin A are infrequent, and renal involvement and long-standing complications are rare [5]. An involvement of body systems other than skin was reported in no more than 25 patients; this included the kidneys (glomerulonephritis, hematuria, and proteinuria), bowel (isolated abdominal pain, intestinal bleeding, intussusception), joints (arthralgias, arthritis), and genitals (testicular torsion) [2]. Severe soft tissue involvement was never reported before in children with AHEI. Compartment syndrome is a clinical condition characterized by elevated pressure within a confined fascial space, causing circulatory compromise, ischemia, and ultimately tissue necrosis [6].
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Compartment syndrome because of AHEI Dotan et al. 421
On reviewing the literature, two cases of HSP and compartment syndrome were found. However, in one case, the child also had a bleeding diathesis (severe factor XIII deficiency) and the compartment syndrome was attributed to a massive hematoma secondary to the continuous bleeding after several attempts of intravenous cannulation [7]. In the second case, the compartment syndrome developed after the removal of an arterial line that might have triggered the event [8]. To our knowledge, there are no reported cases of AHEI involving compartment syndrome. We believe that the edema in this case was so severe that it resulted in increased pressure, leading to the compartment syndrome. Another interesting point in this case is the further diagnosis of the child with FMF. FMF is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, or arthritis [9]. FMF was associated with several vasculic diseases including HSP [10]. Children with FMF tend to have a more severe course of HSP. Thus, the link that exists between FMF and the severity of HSP could also explain the severe and extraordinary complication of this seemingly benign disease in our described case. In conclusion, despite the fact that most cases of pediatric compartment syndrome are traumatic or postoperative in nature [6], orthopedic surgeons should be
aware of other medical etiologies such as vasculitis for compartment syndrome.
Acknowledgements Conflicts of interest
There are no conflicts of interest.
References 1
AlSufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol 2009; 48:617–622. 2 Fiore E, Rizzi M, Ragazzi M, Vanoni F, Bernasconi M, Bianchetti MG, Simonetti GD. Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol 2008; 59:684–695. 3 Whitesides TE, Haney TC, Morimoto K, Harada H. Tissue pressure measurements as a determinant for the need of fasciotomy. Clin Orthop Relat Res 1975; 113:43–51. 4 Garty BZ, Ofer I, Finkelstein Y. Acute hemorrhagic edema of infancy. Isr Med Assoc J 2002; 4:228–229. 5 Cunningham BB, Caro WA, Eramo LR. Neonatal acute hemorrhagic edema of childhood: case report and review of the English-language literature. Pediatr Dermatol 1996; 13:39–44. 6 Bae DS, Kadiyala RK, Waters PM. Acute compartment syndrome in children: contemporary diagnosis, treatment, and outcome. J Pediatr Orthop 2001; 21:680–688. 7 Alioglu B, Ozsoy MH, Tapci E, Karamercan S, Agras PI, Dallar Y. Successful use of recombinant factor VIIa in a child with Schoenlein–Henoch purpura presenting with compartment syndrome and severe factor XIII deficiency. Blood Coagul Fibrinolysis 2013; 24:102–105. 8 Abidin AS, Jalaluddin MY. Left hand compartment syndrome: a rare complication of Henoch Schonlein purpura. J Paediatr Child Health 2008; 44:385. 9 Aksu K, Keser G. Coexistence of vasculitides with familial Mediterranean fever. Rheumatol Int 2011; 31:1263–1274. 10 Flatau E, Kohn D, Schiller D, Lurie M, Levy E. Scho¨nlein–Henoch syndrome in patients with familial Mediterranean fever. Arthritis Rheum 1982; 25:42–47.
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
