Development of scalded skin syndrome in an adult patient with acquired epidermolysis bullosa after long-term treatment with methotrexate

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Abstract

A clinical case of a patient with epidermolysis bullosa acquisita, which is a rare autoimmune disease ― 0.17–0.26 cases per 1 million population, with the development, most likely against the background of long-term cytostatic therapy with methotrexate, develops staphylococcal scalded skin syndrome is presented.

Staphylococcal scalded skin syndrome is a lesion that appears in 0.09–0.56 cases per 1 million and is predominantly observed in newborns and children under 5 years of age, and cases in adults are usually associated with immunosuppression, human immunodeficiency virus / acquired immunodeficiency syndrome, severe renal failure or malignant neoplasms.

The article presents literature data on the etiology and pathogenesis of the presented diseases, features of differential diagnosis, description of clinical and laboratory criteria, on the basis of which diagnoses are made.

This clinical case is of interest to dermatologists, as it describes the scalded skin syndrome, which is rare in everyday practice, in a patient with acquired epidermolysis bullosa, which, despite ongoing therapy, has a risk of death of up to 65%.

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About the authors

Olga Yu. Olisova

The First Sechenov Moscow State Medical University (Sechenov University)

Email: olisovaolga@mail.ru
ORCID iD: 0000-0003-2482-1754
SPIN-code: 2500-7989

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Nataliа P. Teplyuk

The First Sechenov Moscow State Medical University (Sechenov University)

Email: teplyukn@gmail.com
ORCID iD: 0000-0002-5800-4800
SPIN-code: 8013-3256

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Dmitry V. Ignatiev

The First Sechenov Moscow State Medical University (Sechenov University)

Email: dmitrywork@list.ru
ORCID iD: 0000-0001-8751-3965
SPIN-code: 6743-7960

MD

Russian Federation, Moscow

Ekaterina R. Dunaeva

The First Sechenov Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: dunaevaer@gmail.com
ORCID iD: 0000-0002-5458-4991
SPIN-code: 3551-6329

MD

Russian Federation, Moscow

References

  1. Dermatovenerology. National leadership. Short edition. Ed. by Yu.S. Butov, Yu.K. Skripkin, O.L. Ivanov. Moscow: GEOTAR-Media; 2020. 896 p. (In Russ).
  2. Goldsmith LA, Katz SI, Gilchrist BA, et al. Fitzpatrick’s dermatology in clinical practice. Ed. by N.N. Potekaev, A.N. Lvov. Transl. from English A.V. Mishchenko, et al. 2nd revised and updated. Moscow: Panfilov Publishing House; 2018. (In Russ).
  3. Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: Diagnosis and management in children and adults. J Eur Acad Dermatol Venereol. 2014;28(11):1418–1423. doi: 10.1111/jdv.12541
  4. Liy-Wong C, Pope E, Weinstein M, Lara-Corrales I. Staphylococcal scalded skin syndrome: An epidemiological and clinical review of 84 cases. Pediatr Dermatol. 2021;38(1):149–153. doi: 10.1111/pde.14470
  5. Zaslavsky DV, Ravodin RA, Tatarskaya OB, et al. Erythroderma: Current issues of diagnosis and treatment. Pediatr. 2014;5(1):97–102. (In Russ). doi: 10.17816/PED5197-102
  6. Samtsov AV, Belousova IE. Bullous dermatoses. Saint Petersburg: Costa; 2012. 142 p. (In Russ).
  7. Miyamoto D, Gordilho JO, Santi CG, Porro AM. Epidermolysis bullosa acquisita. An Bras Dermatol. 2022;97(4):409–423. doi: 10.1016/j.abd.2021.09.010
  8. Koga H, Prost-Squarcioni C, Iwata H, et al. Epidermolysis bullosa acquisita: The 2019 update. Front Med (Lausanne). 2018;5:362. doi: 10.3389/fmed.2018.00362
  9. Patel S, Cadwell JB, Lambert WC. Comparison of adult vs paediatric staphylococcal scalded skin syndrome inpatients: A retrospective database analysis? Br J Dermatol. 2021;184(4):767–769. doi: 10.1111/BJD.19691
  10. Ross A, Shoff HW. Staphylococcal scalded skin syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022.

Supplementary files

Supplementary Files
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1. JATS XML
2. Fig. 1. Patient L., 47 years old, with a combination of two rare dermatoses (acquired epidermolysis bullosa and staphylococcal scalded skin syndrome): Against the background of partial erythroderma, hemorrhagic spots and erosions up to 7 cm in diameter, irregular in shape, with sharp borders, many of which are covered with hemorrhagic crusts, are observed, as well as marked dryness of the skin (а–с).

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3. Fig. 2. The same patient. There are hemorrhagic spots and multiple erosions from 0.5 to 7–8 cm in diameter, with sharp boundaries, with serous-hemorrhagic discharge and hemorrhagic crusts, against the background of severe dryness of the skin (а–с).

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4. Fig. 3. The same patient. Direct immunofluorescence reaction (a ― there is a distinct fixation of IgG in the basement membrane of the epidermis), and histological examination of a skin biopsy (b ― hyperkeratosis and parakeratosis are noted, flattening of the epidermal papillae; cell exocytosis. In the dermis, there is a dense lympho-leukocyte infiltrate with the phenomenon of diffuse exocytosis of cells into the epidermis. The presence of single acantholytic dystrophic keratinocytes is also noted).

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5. Fig. 4. The same patient was transferred to the intensive care unit (a, b): Multiple irregularly shaped erosive defects are noted, occupying about 40–50% of the skin, with serous-hemorrhagic discharge and numerous hemorrhagic crusts over erosive defects on the background of erythroderma.

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