Forum for Nordic Dermato-Venereology Nr 3, 2018 | Page 13
Nicolas Kluger – Livedo reticularis and livedo racemosa
Definition of “physiological” and “pathological”
livedos
Definition of whether the livedo is physiological and benign
or related to a condition (and therefore pathological) is made
following a meticulous examination of the livedo (Table I).
Physical examination is carried out with the patient lying
down and then standing up. The physician must first describe
the pattern, by answering the following questions:
• Are the lines of the livedo, thin and regular, or thick and
irregular?
• Does the livedo show closed unbroken circles (resembling a
fishing net) or open broken circles (resembling lightning)?
• On palpation, is the livedo infiltrated into any part?
• Is there any necrotic lesion on any part of the livedo?
• Are there any triggering factors?
• Is the livedo acute, transient or permanent?
• Is it worsened by cold exposure or standing?
• What was the age of onset of the livedo (early or late)?
• Where is the livedo localized on the body (lower limbs,
face, trunk, buttocks, upper limbs)?
• Is the distribution generalized or patchy?
• Are there any other cutaneous symptoms that could arise
suspicion for a pathological livedo: purpura, nodules,
atrophic lesions, blue toe, etc.
Cutis marmorata, livedo reticularis or livedo race-
mosa?
One of the main issues regarding the diagnosis of “livedo”
is the confusing terminology used in the medical literature,
which can easily mislead the physician (1–3, 7).
“Cutis marmorata” is an English medical term referring to
physiological livedo reticularis. Nowadays, it should be avoid-
ed for such purpose. However, the term “cutis marmorata” is
still used in the newborn condition, cutis marmorata telangi-
ectatica congenita (or Van Lohuizen syndrome). Briefly, this is
a peculiar necrotic and atrophic livedo of newborns, that can
be localized (on the limb, head, etc.) or diffuse. Its evolution
is variable, ranging from regression to stability or scarring. Its
diagnosis is clinical and it may be associated with various other
genetic conditions (macrocephalia, homocystenuria, trisomy
21, Cornelia de Lange syndrome, etc.) (8).
Fig. 2. Examples of physiological livedo reticularis appearing during
the consultation.
“Livedo reticularis” describes a livedo that has thin and regu-
lar lines, closed, unbroken circles, in other words the typical
“fishing net pattern” (Fig. 2). Livedo reticularis may be either
physiological or pathological.
“Livedo racemosa”, a term first used first by Ehrmann in 1907
(9), describes a livedo with irregular lines, open, broken cir-
cles, resembling “forked lightning” (Fig. 3). Livedo racemosa
is always pathological and the underlying disorder must be
actively managed.
Lastly, the term “retiform purpura” describes a peculiar pur-
pura with stellated borders (Fig. 4). This is related to a vessel
occlusion and subsequent haemorrhage secondary to a period
of ischaemia. The causes of retiform purpura overlap the causes
of an infiltrated and necrotic livedo racemosa (10).
Full clinical and physical examination
It is mandatory to avoid unnecessary exploration and to de-
fine the livedo. Personal and familial history should be fully
Table II. Signs of a “pathological” livedo
•
•
•
•
•
•
•
Thick, irregular lines
Open circles
Painful livedo
Infiltrated livedo
Necrotic livedo
Localization on the trunk, buttocks and face
Other cutaneous symptoms: purpura, papules, nodules, blue toe, etc.
Forum for Nord Derm Ven 2018, Vol. 23, No. 3
Fig. 3. Non-infiltrated livedo racemosa of the elbow.
E ducational R eview
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