Pemphigus vegetans: A clinical case

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Abstract

Aim. To present a clinical case of pemphigus vegetans, a rare form of dermatosis.

Materials and methods. A clinical and laboratory examination of a 32-year-old woman complaining of rash was carried out. The rash in the form of blisters, pustules, erosions and vegetating lesions was located mainly on the mucous membranes and skin folds. The list of performed examinations included a cytological study of a swab taken from the bottom of a lesion, a histological examination of skin biopsies from a lesion and an immunofluorescence study of biopsy specimens taken from an apparently non-affected skin area.

Results. The clinical manifestations of the disease and the results of the conducted histological examinations corresponded to the diagnosis of pemphigus vegetans. The diagnosis was confirmed by the presence of acantholytic cells in the swab and IgG deposits in the intercellular spaces of the epidermis. Oral therapy with prednisone at a dose of 1 mg/kg body weight per day led to regression of the rash.

Conclusion. In the investigated clinical case, the localization and nature of skin lesions were typical of pemphigus vegetans. The conducted histological and enzyme immunoassays confirmed the diagnosis, which allowed an adequate therapy to be selected. As a result, the patient demonstrated a pronounced clinical improvement. The described case is presented due to the rarity of this dermatosis.

About the authors

A. E. Karamova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: karamova@cnikvi.ru
Arfenya E. Karamova* — Cand. Sci. (Med.), Head of the Dermatology Department Россия

V. V. Chikin

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru
Vadim V. Chikin — Dr. Sci. (Med.), Senior Researcher, Dermatology Department Россия

L. F. Znamenskaya

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru
Lyudmila F. Znamenskaya — Dr. Sci. (Med.), Leading Researcher, Dermatology Department Россия

M. A. Nefedova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru
Maria A. Nefedova — Junior Researcher, Dermatology Department Россия

E. S. Mamedova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru
Egana S. Mamedova — Dermatovenerologist, Department of the Diagnosis and Therapy of Common Dermatoses Россия

References

  1. Mergler R., Kerstan A., Schmidt E. et al. Atypical clinical and serological manifestation of pemphigus vegetans: a case report and review of the literature. Case Report Dermatol. 2017;9:121–130.
  2. Uzun S., Durdu M., Akman A. et al. Pemphigus in the mediterranean region of Turkey: a study of 148 cases. Int J Dermatol. 2006;45:523–528.
  3. Ruocco V., Ruocco E., Caccavale S. et al. Pemphigus vegetans of the folds (intertriginous areas). Clin Dermatol. 2015;33(4):471–476.
  4. Zaraa I., Sellami A., Bouguerra C. et al. Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study. J Eur Acad Dermatol Venereol. 2011;25:1160–1167.
  5. Mahé A., Flagueul B., Cissé I. et al. Pemphigus in Mali: a study of 30 cases. Br J Dermatol. 1996;134:114–119.
  6. Morelli J. G., William M., Weston M. D. Childhood immunobullous disease following a second organ transplantation. Pediatr Dermatol. 1999;3:205–207.
  7. Weston W. L., Friednash M., Hachimoto T. et al. A novel childhood pemphigus vegetans variant of intra-epidermal neutophilic IgA dermatosis. J Am Acad Dermatol. 1998;38:635–637.
  8. Wananukul S., Prongprasit P. Childhood pemphigus. Int J Dermatol. 1999;38:29–35.
  9. Sillevis Smitt J. H., Mulder T. J., Albeda F. W., Van Nierop J. C. Pempihgus vegetans in a child. Br J Dermatol. 1992;127:289–291.
  10. Базаев В. Т., Цебоева М. Б., Царуева М. С., Джанаев М. Ф. Вегетирующая пузырчатка, имитирующая рак кожи. Российский журнал кожных и венерических болезней. 2017;20(3):146–150. [Bazaev V. T., Tseboeva M. B., Tsarueva M. S., Dzhanaev V. F. Pemphigus vegetans imitating skin cancer. Russian Journal of Skin and Venereal Diseases. 2017;20(3):146–150. (In Russ.)]
  11. Joly P., Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29:432–436.
  12. Hashizume H., Iwatsuki K., Takigawa M. Epidermal antigens and complement-binding anti-intercellular antibodies in pemphigus vegetans, Hallopeau type. Br J Dermatol. 1993;129:739–743.
  13. Hashimoto K., Hashimoto T., Higashiyama M. et al. Detection of anti-desmocollins I and II autoantibodies in two cases of Hallopeau type pemphigus vegetans by immunoblot analysis. J Dermatol Sci. 1994;7(2):100–106.
  14. Ma D. L., Fang K. Hallopeau type of pemphigus vegetans confined to the right foot: case report. Chin Med J. 2009;122(5):588–590.
  15. Monshi B., Marker M., Feichtinger H. et al. Pemphigus vegetans — immunopathological findings in a rare variant of pemphigus vulgaris. J Dtsch Dermatol Ges. 2010;8:179–183.
  16. Serwin A. B., Bokiniec E., Chodynicka B. Pemphigus vegetans in a patient with lung cancer. Dermatol Online J. 2005;11:13.
  17. Nanda S., Grover C., Garg V. K., Reddy B. S. Pemphigus vegetans of hallopeau. Indian J Dermatol. 2005;50:166–167.
  18. Jansen T., Messer G., Meurer M., Plewig G. Pemphigus vegetans Eine historische Betrachtung. Hautarzt. 2001;52:504–509.
  19. Ahmed A. R. Clinical features of pemphigus. Clin Dermatol. 1983;1:13–21.
  20. Mascaro J. M. Jr. Histologicaland immunofluorescence diagnosis of autoimmune blistering diseases. In: Murrell D. F. (ed.) Blistering diseases: clinical features, pathogenesis, treatment. Berlin — Heidelberg: Springer-Verlag, 2015. P. 161–191.
  21. Самцов А. В., Белоусова И. Э. Буллезные дерматозы. СПб.: ООО «Издательско-полиграфическая компания «КОСТА», 2012. 144 c. [Samtsov A. V., Belousova I. E., Bullosa Dermatosis. Saint Petersburg: KOSTA, 2012. 144 p. (In Russ.)]
  22. Markopoulos A. K., Antoniades D. Z., Zaraboukas T. Pemphigus vegetans of the oral cavity. Int J Dermatol. 2006;45:425–428.
  23. Mendes-Bastos P., Amaro C., Fernandes C. Cobblestone mouth: an exuberant oral presentation of pemphigus vegetans. Actas Dermosifiliogr. 2015;106:72–73.
  24. Халецкая А. М. Pemphigus vegetans, связанный с сапрофитной микрофлорой полости рта. Русский Вестник Дерматологии. 1928;VI(2):148–154. [Khaletskaya A. M. Pemphigus vegetans associated with saprophytic microflora of the oral cavity. Russian Bulletin of Dermatology. 1928;VI(2):148–154. (In Russ.)]
  25. Sukakul T., Varothai S. Chronic paronychia and onychomadesis in pemphigus vegetans: an unusual presentation in a rare autoimmune disease. Case Rep Med. 2018 Jan 11;2018:5980937.
  26. Hertl M., Jedlickova H., Karpati S. et al. Pemphigus. S2 Guideline for diagnosis and treatment — guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2015;29(3):405–414.
  27. Федеральные клинические рекомендации. Дерматовенерология 2015: Болезни кожи. Инфекции, передаваемые половым путем. М.: Деловой экспресс, 2016. 768 с. [Federal clinical guidelines. Dermatovenereology 2015: Diseases of the skin. Sexually transmitted infections. Moscow: Delovoy Ekspress, 2016. 768 p. (In Russ.)]
  28. Hammers C., Schmidt E., Borradori L. Pemphigus vegetans. In: Katsambas A., Lotti T., Dessiniotu C., D’Erme A. (eds) European Handbook of Dermatological Treatments. Heidelberg: Springer, 2015. P. 701–705.
  29. Морейкис И. М., Медведева И. И. Случай вегетирующей пузырчатки с резкой задержкой хлоридов и понижением функциональной способности почек. Русский вестник дерматологии. 1927;V(6):576–582. [Moreykis I. M., Medvedeva I. I. The case of vegetative pemphigus with a sharp delay of chlorides and a decrease in the functional ability of the kidneys. Russian Bulletin of Dermatology. 1927;V(6):576–582 (In Russ.)]
  30. Kaiser J., Kaatz M., Elsner P., Ziemer M. Complete remission of drug-resistant Pemphigus vegetans treated by extracorporeal photopheresis. J Eur Acad Dermatol Venereol. 2007;21:843–844.
  31. Çetinarslan T. S., Ermertcan A. T., Temiz P., Kurutepe S. Pemphigus vegetans arising in umbilicus: Successful clearance with intralesional steroid. Indian J Dermatol Venereol Leprol. 2018;84:522.

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Copyright (c) 2019 Karamova A.E., Chikin V.V., Znamenskaya L.F., Nefedova M.A., Mamedova E.S.

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