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EB10; EH40.2; EH64; EC20.03; EC20.0Y; EC20.02
8.2 Erythrodermas
Grading & Level of Importance: B
ICD-11
Synonyms
Red man syndrome. Symptoms. Various diagnoses.
Epidemiology
See specific diagnoses. See also chapter Red Face.
Definition
Inflammatory and scaly or exfoliative skin condition withredness and scaling of >90% of the skin, which either is primarily involved or spreads secondarily from a non-erythrodermic dermatosis.
Aetiology & Pathogenesis
Depends on underlying diagnosis (see classification).
Signs & Symptoms
Almost complete (>90%) redness and different degree of scaling of the skin, with or without itch. Sparing of the skin in the axila and abdominal folds or on the trunk (nappes claires). Swelling of lymph nodes as a consequence of pruritus scratching, erosions and superinfections. Due to loss of albumin and electrolytes via the exfoliative scaling edema develops. Sometimes temperature dysregulation and/or fever. Cachexia in some cases.
Localisation
Total skin, according to definition.
Classification
Primary Erythrodermas
- Sézary syndrome.
- Erythrodermic mycosis fungoides.
- Staphylococcal scalded skin syndrome (SSSS). Erythroderma due to secondary loss of function of the skin (deficiency of vitamins, electrolytes; malabsorption due to alcohol excess with exsiccation) Senile-(melano-) erythroderma (with cachexia).
- Congenital ichthyosiform erythroderma.
- Severe generalised sunburn.
- Ofuji Disease.
Secondary erythrodermas
- Psoriatic erythroderma.
- Pityriasis rubra pilaris.
- Atopic eczema.
- Drug reaction.
- Paraneoplastic.
- Generalised cutaneous mastocytosis.
- Generalised urticaria.
Laboratory & other workups
Depending on diagnosis (mostly full blood count, electrolytes, liver enzymes, lymphocyte sub-populations, histamine, IgE).
Dermatopathology
Depending on diagnosis.
Course
Can be acute, sub-acute and chronic.
Complications
Loss of fluid (exsiccation), of proteins and electrolytes (scaling), cardiac insufficiency; thermal loss.
Diagnosis
Redness and scaling of the total integument. Specific diagnosis of the underlying disorder is based on the dynamic and follow up of the dermatosis and the histologic features.
Differential diagnosis
See classification.
Prevention & Therapy
Depending on diagnosis. Because of emergency situations, there is often a necessity of an acute intervention.
Review Articles
- Inamadar, A. C. and S. Ragunatha (2019). "The rash that becomes an erythroderma." Clin Dermatol 37(2): 88-98. Lee, H. (2023). "Mycosis fungoides and Sezary syndrome." Blood Res 58(S1): 66-82.
- Lee, H. (2023). "Mycosis fungoides and Sezary syndrome." Blood Res 58(S1): 66-82.
- Cuperus, E., A. Bygum, L. Boeckmann, C. Bodemer, M. C. Bolling, M. Caproni, A. Diociaiuti, S. Emmert, J. Fischer, A. Gostynski, S. Guez, M. E. van Gijn, K. Hannulla-Jouppi, C. Has, A. Hernandez-Martin, A. E. Martinez, J. Mazereeuw-Hautier, M. Medvecz, I. Neri, V. Sigurdsson, K. Suessmuth, H. Traupe, V. Oji and S. Pasmans (2022). "Proposal for a 6-step approach for differential diagnosis of neonatal erythroderma." J Eur Acad Dermatol Venereol 36(7): 973-986.
- Tso, S., H. Moiz, F. Satchwell, T. Hari, S. Dhariwal, R. Barlow, E. Forbat, I. C. Blee, Y. T. Tan, C. Thind, A. Ilchyshyn, H. Randeva, M. M. Kwok, A. C. Y. Tso and T. M. Barber (2021). "Erythroderma (exfoliative dermatitis). Part 2: energy homeostasis and dietetic management strategies." Clin Exp Dermatol 46(6): 1011-1015.
- Tso, S., F. Satchwell, H. Moiz, T. Hari, S. Dhariwal, R. Barlow, E. Forbat, H. Randeva, Y. T. Tan, A. Ilchyshyn, M. M. Kwok, T. M. Barber, C. Thind and A. C. Y. Tso (2021). "Erythroderma (exfoliative dermatitis). Part 1: underlying causes, clinical presentation and pathogenesis." Clin Exp Dermatol 46(6): 1001-1010.
- A.C. Inamadar, S. Ragunatha: The rash that becomes an erythroderma (2019)
- Rothe, M. J., Bialy, T. L., & Grant-Kels, J. M. (2000). Erythroderma. Dermatol Clin, 18(3), 405-415.
- M.J. Rothe, T.L. Bialy, J.M. Grant-Kels: Erythroderma (2000)
- Thestrup-Pedersen, K., Halkier-Sørensen L., Søgaard H. and Zacharie H. (1998). The red man syndrome. Exfoliative dermatitis of unknown etiology: a description and follow-up of 38 patients. J Am Acad Dermatol. Jun;18(6):1307-12. doi: 10.1016/s0190-9622(88)70139-5.
- Thestrup-Pedersen, K., Halkier, S. L., Sogaard, H., & Zachariae, H. (1988). The red man syndrome. Exfoliative dermatitis of unknown etiology: a description and follow-up of 38 patients. J Am Acad Dermatol, 18(6), 1307-1312.
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