Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review
Abstract
:1. Introduction
2. Case Report
3. Discussion
4. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Authors | Sex | Patient Age at Diagnosis | Clinical Features | Mucosal Involvement | Histopathology/DIF | IIF/ ELISA | Treatment | Follow-Up |
---|---|---|---|---|---|---|---|---|
Current case | M | 9 | Erosions on the upper and lower lips, on the surface of the tongue, on the hard palate, on the soft palate and symmetrically on the cheek mucous membranes. Limited lesions on the back | Oral | Acantholysis and intact basal layer. Intercellular IgG and C3 | Anti-DSG 3 (174.4 U/mL) * | Prednisone 1 mg/kg daily, Azathioprine 2 mg/kg daily | Clinical improvement in 1 month. Still on prednisone and azathioprine after 18 months |
Virtuoso, J. [2] | M | 10 | Erosive, bleeding painful lesions of oral mucosa, sialorrhoea, weight loss, dry cough | Oral | Intraepidermal acantholysis. Intercellular IgG and C3 | Anti-DSG 3 | Prednisolone 2 mg/kg daily, Azathioprine 0.5 mg/kg daily | Tapered off drugs after 8 months; no maintenance therapy |
Wananukul, S. [3] | F | 12 | Vescicobollous lesions at trunk, extremities and scalp. Extent of involvement 30% | Oral | Suprabasal separation containing acantholytic cells. Intercellular IgG | 1:160 | Prednisolone 5 mg/kg daily, Azathioprine 2 mg/kg daily | Tapered off drugs in 7 months; no lesions for 3 years; no maintenance therapy |
Wananukul, S. [3] | F | 11 | Vescicobollous lesions at scalp, face, trunk and extremities. Extent of involvement 15% | Oral and genital | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | 1:640 | Prednisolone 3 mg/kg daily, Azathioprine 2 mg/kg daily | Tapered off drugs in 12 months, lesion-free for 15 months |
Fuertes, I. [4] | F | 1.5 | Trunk | Oral | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | Not done | Prednisolone 6 mg/kg daily for 1 month, Azathioprine 1–4 mg/kg day for 4 months, Cyclosporin 5–6.5 mg/Kg/day, Dapsone, Prednisone 20–40 mg/Kg/day, Rituximab | Infection |
Gupta, N.T. [17] | M | 7 | Generalized scaling and redness associated with pedal oedema and tightness of both upper and lower extremities. Flaccid blisters on scalp, face and both lower extremities associated with photosensitivity | No | Suprabasal separation containing acantholytic cells. IgG and C3 at the basement membrane zone | Anti-DSG 1 and 3 | Prednisolone 2.5 mg/kg daily, Azathioprine 25 mg daily | Complete remission. Still on prednisolone 5 mg daily and azathioprine 50 mg daily |
Bean, S.F. [18] | M | 11 | Generalized bullous eruption | Erosions in mucosa | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | IgG 1:1280 | Prednisone, Azathioprine (dosage not specified) | More than 3 years, multiple relapses |
Kanwar, A.J. [19] | M | 14 | Vesiculobullous lesions at scalp, face, trunk and extremities. Extent of involvement 50% | Oral (anus at 9 months after diagnosis) | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | 1:160 | Prednisolone 3 mg/kg daily, Azathioprine 2 mg/kg daily | Still on prednisolone 20 mg (alternate days) and azathioprine (1 mg/Kg/day) at 2 years; intralesional corticosteroids at vegetation |
Harangi, F. [20] | M | 10 | Genital and trunk lesions | Oral (anus at 9 months after diagnosis) | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | Not done | Prednisolone 1.5–2 mg/kg daily, Azathioprine 2 mg/kg daily for 4 months | Complete remission for 4 years |
Kong, H.H. [21] | F | 10 | Trunk (20% of body surface) | Oral | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | Not done | Prednisone 1.6–2 mg/kg daily, Azathioprine 2 mg/kg daily for 4 months, Mycophenolate mofetil 1–2 g/day, Intravenous immunoglobulin, Rituximab | Infection |
Koturoglu, G. [22] | F | 11 | Trunk, extremities | Oral | Suprabasal separation containing acantholytic cells; intercellular IgG and C3 | Not done | Prednisolone 1.5–2 mg/kg daily, Azathioprine 2 mg/kg daily for 6 months | Partial remission on maintenance therapy |
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Vezzoli, P.; Parietti, M.; Carugno, A.; Di Mercurio, M.; Benaglia, C.; Zussino, M.; Cavalli, R.; Sena, P.; Berti, E. Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review. J. Clin. Med. 2022, 11, 6858. https://doi.org/10.3390/jcm11226858
Vezzoli P, Parietti M, Carugno A, Di Mercurio M, Benaglia C, Zussino M, Cavalli R, Sena P, Berti E. Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review. Journal of Clinical Medicine. 2022; 11(22):6858. https://doi.org/10.3390/jcm11226858
Chicago/Turabian StyleVezzoli, Pamela, Michele Parietti, Andrea Carugno, Marco Di Mercurio, Chiara Benaglia, Martina Zussino, Riccardo Cavalli, Paolo Sena, and Emilio Berti. 2022. "Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review" Journal of Clinical Medicine 11, no. 22: 6858. https://doi.org/10.3390/jcm11226858