1. ERYTHEMA GYRATUM REPENS &
ERYTHEMA MARGINATUM RHEUMATICUM
DR STEFI RANI
DVL PG 2nd Yr
REFERENCE
1.ROOKS
2.IADVL
2. Erythema gyratum repens
● Erythema gyratum repens is a rare, distinctive, figurate eruption consisting of
mobile, concentric, often palpable, erythematous, wave‐like bands, which give a
‘wood‐grain’ appearance to the skin.
● A peripheral scale or collarette may be present. Lesions often commence on the
limbs and the complete torso may be affected.
● There is severe pruritus, and sometimes ichthyosis and bullae may develop within
the erythema.
● The lesions migrate from day to day, usually changing position by about 1 cm
daily.
3. Epidemiology
Age
It usually occurs in patients older than 40 years, usually in the seventh decade
with a mean age of 63 years, but it has been reported to occur from age 16 to 75
years.
Sex
The male to female ratio is 2 : 1.
Ethnicity
It has been reported to occur predominantly in white persons.
4. Associated diseases
● Erythema gyratum repens is considered in the majority of patients (80%) to be a
paraneoplastic dermatosis and may precede the diagnosis of malignancy.
● The most frequent underlying malignancy in descending order is carcinoma of the
lung (47%) followed by oesophagus, breast, stomach, kidney, cervix, pharynx,
urinary bladder, uterus , cervix, pancreas, prostate and haematological neoplasia.
● Treatment of the cancer may result in clearance of the eruption, and tumour
recurrence or metastases can precipitate a recurrence.
5. ● Erythema gyratum repens has also been described in the absence of an underlying
malignancy associated with pityriasis rubra pilaris , psoriasis, ichthyosis, mycobacterial
infections, connective tissue diseases (lupus erythematosus, Sjögren syndrome,
rheumatoid arthritis and scleroderma) and the hypereosinophilic syndrome.
● An erythema gyratum repens‐ like eruption may be a manifestation of another
cutaneous disease, diagnosis being made on histology (lupus erythematosus,
immunobullous disorder , mycosis fungoides, erythrokeratoderma variabilis,
urticarial vasculitis and neutrophilic dermatosis).
● Close clinicopathological correlation is required as there is an underlying
malignancy present even if this cannot be diagnosed at presentation.
6. Pathophysiology
● The histological features are not diagnostic. There is a superficial and
occasionally deep perivascular lymphohistiocytic infiltrate in the papillary dermis
associated with hyperkeratosis, parakeratosis, acanthosis and spongiosis.
● Granular deposits of IgG and C3 have been found at the basement membrane
zone of both involved and uninvolved skin, supporting the concept that this
dermatosis has an immunological pathogenesis
7. Clinical features
● Regular waves of erythema spread over the body to produce a series of
concentric, figurate bands in a pattern resembling the grain of wood.
● The characteristic feature is the way the rings, swirls or waves appear within
existing lesions to form a concentric pattern of sequential eruptions, with day
to day migration of the leading edge by about 1 cm. Scaling usually present at
the trailing edge, and itching is prominent.
● This is in contrast to the more common ‘annular erythemas’ of the EAC
pattern, in which each lesion is usually a distinct ring or arc with variable but
usually without prominent scaling, and not in a concentric arrangement.
● Hyperkeratosis of palms occurs in about 10% and has been reported in both
paraneoplastic and idiopathic cases.
8.
9. Differential diagnoses
Include EAC, erythema multiforme, necrolytic migratory erythema, subacute cutaneous
lupus erythematosus, lupus erythematosus gyratum repens, erythema chronicum
migrans, tinea corporis, erythrokeratoderma variabilis, subacute annular (Lapière)
variant of psoriasis and pityriasis rubra pilaris.
Disease course and prognosis
This is dependent on the underlying disease.
10. Investigations
Useful investigations include a full blood count, antinuclear antibody profile, chest X‐ray,
mammogram, cervical smear, prostate‐specific antigen, CT scan of the thorax, abdomen and
pelvis and, if indicated, endoscopy or colonoscopy.
Management
Treatment ladder
First line
• Identify the underlying disease
Second line
• Treat the underlying disease
11. Erythema marginatum rheumaticum
● Erythema marginatum rheumaticum is an annular and sometimes polycyclic,
serpiginous, erythematous eruption. Together with carditis, migratory polyarthritis, chorea
and subcutaneous nodules it is one of the Duckett Jones major criteria for the diagnosis
of rheumatic fever.
● However, erythema marginatum occurs in about 10% of patients with rheumatic fever.
The rash appears as pink or red macules or papules that spread outwards in a circular
shape.
● As the lesions advance, the edges become raised and red, and the centre clears. The
lesions are neither pruritic nor painful, and sometimes go unnoticed by the patient. The
lesions can fade and reappear within hours and may persist intermittently for weeks to
months.
12. Introduction
● The annular rash commonly occurs over the trunk and inner aspects of the upper arms
and thighs, but rarely if ever on the face.
● The rash is painless and rarely pruritic and typically appears in crops lasting hours to
several days.
● The rash blanches on pressure, is transient and tends to migrate from one part of the
body to another.
● It is associated with rheumatic fever but only occurs in 1–18% of patients.
13. Epidemiology
Incidence and prevalence
Erythema marginatum as a manifestation of rheumatic fever is predominantly a
disease of developing countries and the incidence declines as socioeconomic
conditions improve.
Age
It is most common in children between the ages of 5 and 15 years.
Sex
There is no sex predilection.
14. Associated diseases
● These include rheumatic fever, psittacosis and angio-oedema (C1 inhibitor
defi ciency acquired or hereditary) .
● A firm diagnosis of rheumatic fever requires that two major or one major and
two minor criteria are satisfied , in addition to evidence of recent streptococcal
infection.
15. Pathophysiology
Pathology
● The histological features are non‐specifi c and include a perivascular polymorphous infi
ltrate of neutrophils and mononuclear cells in the papillary dermis and upper portion of
the reticular dermis .
● Nuclear debris suggestive of leukocytoclasia is often present but vasculitis is not seen.
● There have been reports of cases devoid of neutrophils where the infi ltrate consisted
of a sparse, superficial, perivascular, mononuclear infiltrate composed entirely of
lymphocytes and histiocytes.
16. Causative organisms
It is predominantly associated with a β‐haemolytic (group A)streptococcal
infection but has also been described in association with psittacosis.
17. Duckett Jones criteria for the diagnosis of rheumatic fever
Major criteria
• Carditis: all layers of cardiac tissue are affected (pericardium, epicardium,
myocardium, endocardium). The patient may have a new or changing murmur, with
mitral regurgitation being the most common, followed by aortic insufficiency
• Polyarthritis: migrating arthritis that typically affects the knees, ankles, elbows and
wrists. The joints are severely painful and symptoms are dramatically responsive to
anti‐inflammatory medicines.
18. • Chorea: also known as Syndenham’s chorea or St Vitus’ dance. There are abrupt,
purposeless movements. This may be the only manifestation of acute rheumatic fever
and its presence is diagnostic. May also include emotional disturbances and
inappropriate behavior.
• Erythema marginatum: a non‐pruritic rash that commonly affects the trunk and
proximal extremities, but spares the face. The rash typically migrates from central
areas to the periphery and has well‐defined borders.
• Subcutaneous nodules: usually located over bones or tendons, these nodules are
painless and firm.
19. Minor criteria
• Fever
• Arthralgia
• Previous rheumatic fever or rheumatic heart disease
• Raised acute phase reactants, e.g. leukocytosis, elevated erythrocyte sedimentation
rate and C‐reactive protein
• Prolonged P‐R interval on electrocardiogram
20. Clinical features
● The lesions of erythema marginatum typically begin as erythematous macules or
papules that spread peripherally and may merge to produce the typical
serpiginous, polycyclic annular eruption.
● They are usually non‐pruritic and primarily involve the trunk and proximal
extremities but may involve the hands and face.
● A characteristic feature is the rapid spread, which may be 2–10 mm in 12 h, and
the pattern, which may change within an hour, completely resolving and then
reappearing.
23. Complications and co‐morbidities
● Rheumatic fever is an autoimmune inflammatory process that develops as a
sequela of a streptococcal infection, most often pharyngeal infection with
rheumatogenic group A streptococci in a susceptible individual.
● The disease manifests as polyarthritis, carditis, chorea, erythema marginatum
and/or subcutaneous nodules.
● Carditis, the most serious complication, occurs in 30–45% of rheumatic fever
patients and leads to chronic rheumatic heart disease which is characterized by
progressive and permanent valvular lesions.
24. Investigations
Erythema marginatum is part of a clinical syndrome for which no specific tests exist.
However, to aid the diagnosis the following would be appropriate:
full blood count, urea and electrolytes, liver function tests, erythrocyte sedimentation
rate, C‐reactive protein and ferritin, throat swab, antistreptolysin O titre, blood cultures,
ECG and echocardiography and skin biopsy.
