scholarly journals Purpura Fulminans in a 20-Year-Old Female

2021 ◽  
Vol 79 (4) ◽  
pp. 369-371
Author(s):  
Francisca Alves ◽  
Maria Manuel Brites ◽  
Inês Coutinho
Keyword(s):  
Acute Infection ◽  
Purpura Fulminans ◽  
Protein S ◽  
Lower Limbs ◽  
Acute Tonsillitis ◽  
Systemic Corticosteroids ◽  
Clinical Scenarios ◽  
The Face ◽  
Accompanying Symptoms ◽  
Partial Deficiency

A previously healthy 20-year-old female presented with extensive retiform purpura located at the face, upper and lower limbs, one week after an episode of acute tonsillitis. Despite the exuberance of the cutaneous findings and progression to skin necrosis she had no accompanying symptoms. Laboratory investigation revealed a heterozygous protein C mutation (exon 9, c.1332G> C, p.Trp444Cys), accounting for a partial deficiency of this anticoagulant protein. The patient was started on broad spectrum antibiotics, anticoagulation and systemic corticosteroids, with no lesional progression and complete resolution of cutaneous ulceration within 6 months. This is a singular case of purpura fulminans, since two different causative factors precipitated the events. The previous tonsillitis reported by the patient is significant, because the serum concentration of protein S may also decrease after an infectious event - post-infectious purpura fulminans. This case illustrates that purpura fulminans due to autoantibodies against protein S, although rare, should be considered, especially in the absence of a severe acute infection. It also illustrates how in a given patient different independent factors can act simultaneously, triggering potentially devastating clinical scenarios.

scholarly journals Idiopathic purpura fulminans with anti-PS antibodies in children: a multicenter case series and systematic review

2021 ◽  
Author(s):  
Alexandre THERON ◽  
Olivier Dautremay ◽  
Elodie Boissier ◽  
Amel ZERROUKHI ◽  
Julien BALEINE ◽  
...  
Keyword(s):  
Systematic Review ◽  
Treatment Options ◽  
Diagnostic Delay ◽  
Purpura Fulminans ◽  
Case Series ◽  
Protein S ◽  
Fresh Frozen Plasma ◽  
Lower Limbs ◽  
Coagulation Disorder ◽  
Fresh Frozen

Idiopathic purpura fulminans (IPF) is a rare but severe pro-thrombotic coagulation disorder that can occur after chickenpox or HHV6 infection. IPF leads to an autoantibody-mediated decrease in the protein S plasma concentration. We conducted a retrospective multicenter study involving IPF patients from 13 French pediatric centers and a systematic review of literature-published cases. Eighteen patients were included in our case series, and thirty-four as literature review cases. The median age was 4.9 years and the diagnostic delay after the first signs of viral infection was 7 days. The lower limbs were involved in 49 (94%) patients with typical lesions. A recent history of VZV or HHV6 infection was present in 41 (78%) and 7 (14%) of cases, respectively. Most of the patients received heparin (n=51, 98%) and fresh frozen plasma transfusions (n=41, 79%); other treatment options were immunoglobulin infusion, platelet transfusion, corticosteroid therapy, plasmapheresis, and coagulation regulator concentrate infusion. The antithrombin level and platelet count at diagnosis appeared to be associated with severe complications. Given the rarity of this disease, the creation of a prospective international registry is required to consolidate these findings.

Purpura fulminans due to protein S deficiency following chickenpox

1992 ◽  
Vol 127 (1) ◽  
pp. 30-32 ◽  
Author(s):  
W.G. PHILLIPS ◽  
J.R. MARSDEN ◽  
F.G. HILL
Keyword(s):  
Purpura Fulminans ◽  
Protein S ◽  
Protein S Deficiency ◽  
S Deficiency

Malignant Pyoderma

1981 ◽  
Vol 89 (6) ◽  
pp. 917-922 ◽  
Author(s):  
John R. Salassa ◽  
R.K. Winkelmann ◽  
Thomas J. McDonald
Keyword(s):  
Chronic Inflammation ◽  
External Ear ◽  
Systemic Corticosteroids ◽  
The Face ◽  
Acute And Chronic Inflammation ◽  
Histopathologic Findings

Malignant pyoderma is a rare inflammatory pyoderma of unknown cause with characteristic destructive and necrotizing ulcers limited to the face, neck, and upper trunk. The typical lesions are small, purulent, periauricular ulcers. Enlargement and coalescence result in extensive lesions that may ultimately destroy portions of the external ear. The histopathologic findings are acute and chronic inflammation with necrosis. Treatment consists of long-term systemic corticosteroids. We present two new cases and alert otolaryngologists to the seriousness of this disease.

scholarly journals A study of 200 cases of congenital limb deficiencies

1994 ◽  
Vol 18 (3) ◽  
pp. 174-179 ◽  
Author(s):  
S. K. Jain
Keyword(s):  
Surgical Correction ◽  
Lower Limbs ◽  
Upper Limbs ◽  
Artificial Limb ◽  
Prosthetic Fitting ◽  
Congenital Limb ◽  
Partial Deficiency ◽  
Limb Deficiencies ◽  
Limb Deficiency ◽  
Congenital Limb Deficiency

An analysis of 200 patients with congenital limb deficiency who attended the Artificial Limb Centre, Pune from January 1984 to April 1990 is presented. This group is representative of the congenital limb deficient population of the country. The commonest deficiencies were transverse phalangeal total/partial deficiency and transverse forearm partial deficiency (below elbow) in upper limbs, whereas transverse metatarsal total/partial deficiency and transverse leg partial deficiency (below knee) were commonest in lower limbs. Transverse forearm partial deficiency was more common in female, while transverse leg partial deficiency was more common in male children, 16 patients did not require any treatment, 6 needed only surgical correction. Some 30 patients needed surgery before prosthetic fitting, while 148 patients required only prostheses. Some 68% of patients achieved satisfactory to excellent results; 18% showed poor rehabilitation. No definitive cause for the deformities could be isolated; however, many parents believed that possible exposure to the eclipse during pregnancy was the cause of the deficiency. The eldest child was most affected.

Protein S-Mangel durch passageren Protein S-Inhibitor bei einem Kind mit Purpura fulminans

1993 ◽  
pp. 272-276
Author(s):  
F. Bergmann ◽  
P. F. Hoyer ◽  
S. Vigano D’Angelo ◽  
A. D’Angelo ◽  
C. Oestereich ◽  
...  
Keyword(s):  
Purpura Fulminans ◽  
Protein S

Postvaricella Purpura Fulminans Caused by Acquired Protein S Deficiency Resulting From Antiprotein S Antibodies: Search for the Epitopes

2002 ◽  
Vol 24 (5) ◽  
pp. 413-416 ◽  
Author(s):  
C. Heleen van Ommen ◽  
Merel van Wijnen ◽  
Flip G. de Groot ◽  
Chantal M. A. M. van der Horst ◽  
Marjolein Peters
Keyword(s):  
Purpura Fulminans ◽  
Protein S ◽  
Protein S Deficiency ◽  
S Deficiency

scholarly journals Chest Pain: The Need to Consider Less Frequent Diagnosis

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
Pedro Magalhães ◽  
Anabela Morais ◽  
Sofia Carvalho ◽  
Joana Cunha ◽  
Ana R. Lima ◽  
...  
Keyword(s):  
Chest Pain ◽  
Takayasu Arteritis ◽  
Temporal Artery ◽  
Lower Limbs ◽  
Temporal Artery Biopsy ◽  
Acute Phase Reactants ◽  
Granulomatous Vasculitis ◽  
Positron Emission ◽  
Clinical Scenarios ◽  
Underlying Causes

Chest pain is one of the most frequent patient’s complaints. The commonest underlying causes are well known, but, sometimes, in some clinical scenarios, it is necessary to consider other diagnoses. We report a case of a 68-year-old Caucasian male, chronically hypertensive, who complained of recurrent episodes of chest pain and fever with elevated acute phase reactants. The first investigation was negative for some of the most likely diagnosis and he quickly improved with anti-inflammatory drugs. Over a few months, his symptoms continued to recur periodically, his hypertension was aggravated, and he developed headaches and lower limbs claudication. After a temporal artery biopsy that was negative for vasculitis, he underwent a positron emission tomography suggestive of Takayasu Arteritis. Takayasu Arteritis is a rare chronic granulomatous vasculitis of the aorta and its first-order branches affecting mostly females up to 50 years old. Chest pain is experienced by >40% of the patients and results from the inflammation of the aorta, pulmonary artery, or coronaries.

POSTINFECTION PURPURA FULMINANS IN A PATIENT HETEROZYGOUS FOR PROTHROMBIN G20210A AND ACQUIRED PROTEIN S RESISTANCE

1999 ◽  
Vol 16 (6) ◽  
pp. 561-564 ◽  
Author(s):  
Saad Al-Ismail ◽  
Peter Collins ◽  
Riayd Najib ◽  
Michael James-Ellison ◽  
Malachy O'Hagan
Keyword(s):  
Purpura Fulminans ◽  
Protein S ◽  
Prothrombin G20210a
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