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ERYTHRODERMA
(EXFOLIATIVE DERMATITIS)

DERMATOLOGY LECTURE NOTES

Debabrata Bandyopadhyay,  Associate Professor & Head,  Dept. of Dermatology,
 R G Kar Medical College, Calcutta, INDIA
 
Erythroderma or exfoliative dermatitis (ED) is a syndrome characterized by generalized inflammatory erythema with scaling. ED may result from a variety of causes, most commonly from extension of pre-existing skin disorders. It may also occur due to drug reactions or as a response to systemic diseases, notably internal malignancies. In a number of cases, the cause remains unknown. The clinical picture is same irrespective of the underlying cause and may be accompanied by a number of metabolic consequences.
 
 AETIOLOGY & PATHOGENESIS

ED may result from a variety of causes:
 
Skin Diseases Systemic Disorders Drugs

Psoriasis

Leukemia

Sulphonamides

Atopic dermatitis

Hodgkin's lymphoma

Dapsone

Seborrhoeic dermatitis

Non-Hodgkin's lymphoma

Penicillins

Stasis dermatitis

Mycosis fungoides

Cephalosporins

Contact dermatitis

Sezary syndrome

Anticonvulsants

Pityriasis rubra pilaris

Myeloma

INH

Pemphigus foliaceus

Carcinoma of lung and colon

Sulphonylureas

Norwegian scabies

HIV infection

Thiazides

Dermatophytosis

Graft versus host disease

Gold

Lichen planus

 

Arsenic

Reiter's syndrome

 

Allopurinol

Sarcoidosis

 

Antimalarials
 

 

Retinoids  and many other drugs
In a significant proportion of cases, no underlying cause can be found.
The primary pathophysiologic events resulting from these causes that subsequently lead to the clinical picture of generalized scaling and erythema remain undetermined. but it is probably a consequence of liberation of and interactions among the many cytokines and cellular adhesion molecules occurring in the skin. An increase in the rate of epidermal turnover occurs and the transit time of cells through the epidermis is shortened. This leads to  profuse scaling. The resultant loss of large amount  of proteins in the scales has adverse metabolic consequences.  
 
 CLINICAL FEATURES

CUTANEOUS FEATURES:
  • Erythroderma is more commonly found in older age groups. More males are affected than females.
  • The condition usually develops slowly, but may be of acute onset.
  • Erythema is usually the initial manifestation, followed by scaling, which becomes generalized. Scales may be fine or there may desquamation in large sheets.
  • Patients usually complains of a feeling of chilliness as well as varying degrees of itching which may be intolerable.
  • Oozing and secondary infection may develop.
  • There may be thick crusts on the scalp.
  • Palmoplantar thickening may develop.
  • Varying degrees of alopecia and nail dystrophy are common.
  • There is usually no clinical difference between the cases caused by underlying diseases and those which are idiopathic, but in some cases evidence of the underlying pathology like psoriasis may still be identified.
SYSTEMIC CHANGES:

  • Lymphadenopathy occurs in majority of the cases, even in those cases without any evidence of lymphoid malignancy.

  • Hepatomegaly and splenomegaly in some cases

  • Raised body temperature due to abnormality in temperature control.

  • Tachycardia  

  • Hyperkinetic circulation, cardiac failure may occur.

  • Raised basal metabolic rate

  • Gynecomastia , its cause or significance is not known.

  • Anemia and eosinophilia  

  • Elevated IgE

  • Hypoalbuminemia and edema

  • Serum electrolyte abnormalities due to  dehydration resulting from  increased transepidermal water loss.
 PATHOLOGY
Biopsy is often non-contributory, showing non-specific features. Idiopathic and drug-induced cases commonly shows features of a subacute dermatitis with hyperkeratosis with parakeratosis, acanthosis and spongiosis. The primary pathologic processes like lymphoma or leukemia may be discernable, thus helping in the diagnosis. Repeated biopsies may be necessary to establish the underlying cause..
 
 MANAGEMENT
  • Depends on the underlying cause.
  • Withdrawal of drugs in drug-induced cases.
  • Specific treatments of underlying pathologies like like lymphoma or other malignancies.
  • Emollients
  • Maintenance of fluid balance.
  • Good, nutritious, protein-rich diet.
  • Antihistamines for pruritus
  • Judicious use of topical steroids.
  • PUVA therapy in cases caused by psoriasis or mycosis fungoides.
  • Methotrexate in psoriatic cases.
  • Systemic steroids when conservative therapy is ineffective ( not in cases caused by psoriasis) .
 COURSE AND PROGNOSIS
  • Prognosis of ED depends on the underlying disease process.
  • Drug-induced cases resolves after drug withdrawal and appropriate therapy.
  • Prognosis of cases resulting from underlying systemic disorders like lymphomas will depend on the success of treatment of those conditions.
  • ED caused by dermatoses will eventually clear with treatment, but may recur.
  • Idiopathic cases are unpredictable.They may persist for a long time often with waxing and waning of the condition.
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