TY - JOUR
T1 - Bullae and purpura revealing protein C consumption
AU - Valentini, Piero
AU - Piastra, Marco
AU - Bersani, Iliana
AU - Pietrini, Domenico
AU - De Carolis, Maria Pia
AU - Conti, Giorgio
AU - Vitale, Francesca
PY - 2011
Y1 - 2011
N2 - We present two patients’ clinical images showing severe
foot lesions characterized by bullous appearance over a
diffusely purpuric skin, with serious fingertip involvement.
Figure 1a and b presents a 4-year-old child suffering from
Streptococcus pyogenes septic shock while Fig. 1c and d
shows an 18-month-old child with group C Neisseria
meningitidis septic shock. Both cases were complicated by
severe DIC (with admission protein C level equal to 8 and
2%, respectively) followed by massive rhabdomyolysis and
myoglobinuria; low-dose fenoldopam maintained renal
function in the first case, whereas hemodiafiltration was
necessary in the second patient. Both children survived after
receiving high-level hemodynamic and ventilatory support.
Cutaneous anomalies characterized by the presence of
purpura and bullae may be due to several causes with different
pathophysiology, clinical presentation, and location
distinguishing each clinical form. Differential diagnoses
include DIC, sepsis, Henoch-Scho¨nlein purpura, necrotizing
fasciitis, systemic lupus erythematosus, hemorrhagic
erysipelas, bullous amyloidosis, ecthyma gangrenosum,
thrombocytopenia, gloves and socks syndrome, and herpetiform
dermatitis [1–4]. In our cases, cutaneous lesions
resulted from massive ischemic processes involving lower
limb terminal districts. Distal perfusion was improved by
treating the thrombotic process through protein C concentrate
[5] and antithrombin massive replacement; intravenous
pentoxifylline and low-dose prostacyclin were given
due to their antiinflammatory and rheologic properties.
Eventually, only a few distal phalanges were partially lost in
both patients. Massive rhabdomyolysis and myoglobinuria
were treated conservatively for the meningococcal sepsis
patient, whereas a 6-day course of continuous veno-venous
hemodiafiltration was needed for the Streptococcus pyogenes-
affected child.
AB - We present two patients’ clinical images showing severe
foot lesions characterized by bullous appearance over a
diffusely purpuric skin, with serious fingertip involvement.
Figure 1a and b presents a 4-year-old child suffering from
Streptococcus pyogenes septic shock while Fig. 1c and d
shows an 18-month-old child with group C Neisseria
meningitidis septic shock. Both cases were complicated by
severe DIC (with admission protein C level equal to 8 and
2%, respectively) followed by massive rhabdomyolysis and
myoglobinuria; low-dose fenoldopam maintained renal
function in the first case, whereas hemodiafiltration was
necessary in the second patient. Both children survived after
receiving high-level hemodynamic and ventilatory support.
Cutaneous anomalies characterized by the presence of
purpura and bullae may be due to several causes with different
pathophysiology, clinical presentation, and location
distinguishing each clinical form. Differential diagnoses
include DIC, sepsis, Henoch-Scho¨nlein purpura, necrotizing
fasciitis, systemic lupus erythematosus, hemorrhagic
erysipelas, bullous amyloidosis, ecthyma gangrenosum,
thrombocytopenia, gloves and socks syndrome, and herpetiform
dermatitis [1–4]. In our cases, cutaneous lesions
resulted from massive ischemic processes involving lower
limb terminal districts. Distal perfusion was improved by
treating the thrombotic process through protein C concentrate
[5] and antithrombin massive replacement; intravenous
pentoxifylline and low-dose prostacyclin were given
due to their antiinflammatory and rheologic properties.
Eventually, only a few distal phalanges were partially lost in
both patients. Massive rhabdomyolysis and myoglobinuria
were treated conservatively for the meningococcal sepsis
patient, whereas a 6-day course of continuous veno-venous
hemodiafiltration was needed for the Streptococcus pyogenes-
affected child.
KW - Bullae and purpura
KW - Bullae and purpura
UR - http://hdl.handle.net/10807/3716
U2 - 10.1007/s15010-011-0150-4
DO - 10.1007/s15010-011-0150-4
M3 - Article
SN - 0300-8126
SP - 599
EP - 600
JO - Infection
JF - Infection
ER -