10.5826/dpc.1201a05
text/xml
2160-9381
1656
en
Purnima Malhotra
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
juvenile bullous pemphigoid
Diffuse Juvenile Bullous Pemphigoid Managed Successfully With a Short Course of Cyclophosphamide
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-02-02
2021-01-24
2022-02-02
2022-02-09
e2022005
Dermatol Pract Concept
Purnima Malhotra
2022/02/02
10.5826/dpc.1201a05
e2022005
Department of Pathology, Atal Bihari Vajpayee Institute of Medical Sciences (ABVIMS) and Dr. Ram Manohar Lohia Hospital, New Delhi, India
2160-9381
juvenile bullous pemphigoid
en
e2022005
Nemeth AJ, Klein AD, Gould EW, Schachner LA. Childhood Bullous Pemphigoid: Clinical and Immunologic Features, Treatment, and Prognosis. Arch Dermatol. 1991;127(3):378–386. doi:10.1001/archderm.1991.01680030098014. PMID: 1998369
Diffuse Juvenile Bullous Pemphigoid Managed Successfully With a Short Course of Cyclophosphamide
https://dpcj.org/index.php/dpc/article/download/1656/1466
                    Dermatology Practical & Conceptual


            Diffuse Juvenile Bullous Pemphigoid
         Managed Successfully With a Short Course of
                     Cyclophosphamide
     Seema Rani1, Diksha Aggarwal1, Kabir Sardana1, Savitha Bathula1, Purnima Malhotra2


1 Department of Dermatology, Atal Bihari Vajpayee Institute of Medical Sciences (ABVIMS) and Dr. Ram Manohar Lohia Hospital,
  New Delhi, India
2 Department of Pathology, Atal Bihari Vajpayee Institute of Medical Sciences (ABVIMS) and Dr. Ram Manohar Lohia Hospital,
  New Delhi, India



Key words: immunobullous, bullous pemphigoid, cyclophosphamide, juvenile bullous pemphigoid
Citation: Rani S, Aggarwal D, Sardana K, Savitha B, Malhotra P. Diffuse juvenile bullous pemphigoid managed successfully with a short
course of cyclophosphamide. Dermatol Pract Concept. 2022;12(1):e2022005. DOI: https://doi.org/10.5826/dpc.1201a05
Accepted: April 18, 2021; Published: January 2022
Copyright: ©2022 Rani et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted
noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Seema Rani, Associate Professor, Department of Dermatology, Atal Bihari Vajpayee Institute of Medical Sciences
(ABVIMS) and Dr. Ram Manohar Lohia Hospital, New Delhi, India. E-mail: drseemashekhar@gmail.com




Introduction                                                             an erythematous base that involved 90% of the body sur-
                                                                         face areaalong with involvement of head and neck areas
Adolescent bullous pemphigoid (BP) is a very rare entity—                (Figure1A). Nails and mucosa were unaffected. Bulla spread
with generalized (erythrodermic) variant being even rarer—               sign was positive and Nikolsky sign was negative. Tzanck
and management of such difficult BP cases can be challenging.            smear showed eosinophils and neutrophils. The total leu-
                                                                         kocyte count was 20,000 per mm3 with 77% eosinophils
                                                                         in differential leukocyte count and an absolute eosinophil
Case Presentation
                                                                         count of 15,400 per mm3. The serum immunoglobulin (Ig) E
A 17-year-old girl presented with a 1-month history of                   level was normal. Histopathology revealed focal clefting at
intense pruritus that was followed by the development of                 the dermoepidermal junction and perivascular mononuclear
multiple fluid-filled lesions. These lesions ruptured spon-              inflammatory infiltrate with numerous eosinophils in the
taneously in 3 to 4 days and left behind painful erosions.               superficial dermis (Figure 1B). Direct immunofluorescence
There was no history of drug intake, trauma, recent vac-                 revealed linear deposits of IgG and C3 along the basement
cination, fever,or photosensitivity. Notably, she had an                 membrane zone. Based on clinical presentation and histo-
intellectual disability; however, the psychiatric consultation           pathological and immunological findings, a diagnosis of
was normal. Cutaneous examination revealed multiple                      diffuse adolescent BP was made. The BP disease area index
tensevesicles, both clear and hemorrhagic, and bullae over               score was 145.



Research Letter | Dermatol Pract Concept. 2022;12(1):e2022005                                                                           1
Figure 1. (A) Multiple tense vesicles and bullae over an erythema-
tous base. (B)Subepidermal blister containing fibrin and eosinophils
peri adnexal infiltrate of eosinophils in the dermis (hematoxylin and
eosin stain, ×40).


    Oral prednisolone 70 mg along with broad-spectrum
antibiotic cover and antihistamines were started. Doxycycline
100 mg and nicotinamide tablets 500 mg were added as ste-
roid-sparing agents, but there was a progressive increase in
the severity of the eruption. As a consequence, Doxycycline
was replaced by azathioprine 50 mg twice daily. In spite of 4
weeks of azathioprine, there were 40-50 new blisters per day,
and in view of the recalcitrance, azathioprine was discontin-
                                                                        Figure 2. Healed lesions with residual pigmentary changes.
ued and cyclophosphamide 100 mg was added. After 2 weeks,
the number of new lesions gradually decreased and old lesions
started to heal. The patient was discharged on prednisolone             which treatment resistance occurs. Childhood BP usually
50 mg and cyclophosphamide 100 mg. Two weeks later the                  has a good prognosis, although, in some cases the course is
dose of cyclophosphamide and prednisolone was tapered to                less benign [2]. Our case had generalized BP, poor response
50 mg and 40 mg, respectively. During treatment patient rou-            to high doses of steroid, anti-inflammatory, antibiotics and
tine hematological investigations and urineanalysis were nor-           azathioprine. Non-availability of biologics at our center and
mal. Over the next 4 weeks, cyclophosphamide was stopped.               economic constraints resulted in the use of cyclophosphamide
Azathioprine was restarted at a dose of 25 mg and gradually             that was administered as a “rescue therapy” in view of its side
increased. Currently the patient is undergoing oral prednis-            effect profile. Our case exemplified the role of cyclophospha-
olone 15 mg and azathioprine 50 mg with a good control of               mide as a short-term bridge therapy, after which the patient
her disease without any side-effects. The patient is on regular         was adequately controlled on azathioprine. In a severe and
follow-up at a 4- to 6-week intervals with no relapse of bul-           recalcitrant case, such as this one, cyclophosphamide can be
lous lesions but with residual pigmentary changes (Figure 2).           used to bring the disease under control.
    Informed consent has been taken from patient guardian                  Informed consent: Written informed consent for publi-
(patient is minor) for the nature and side-effects of drug              cation of clinical details and clinical images was obtained
(cyclophosphamide).                                                     from the patient.


Conclusions                                                             References
BP is the commonest immunobullous disorder worldwide, but                1. Patsatsi A, Kyriakou A, Werth VP. Bullous pemphigoid in ado-
it is rare in childhood (100 cases) and extremely uncommon                  lescence. Pediatr Dermatol. 2019;36(2):184-188. DOI: 10.1111/
                                                                            pde.13717. PMID: 30569520.
in adolescence (14 cases) [1]. Childhood BP is diagnosed
                                                                         2. Nemeth AJ, Klein AD, Gould EW, Schachner LA. Childhood Bul-
according to the criteria of Nemeth, and our case satisfied                 lous Pemphigoid: Clinical and Immunologic Features, ­Treatment,
these criteria. BP is generally more responsive to treatment                and Prognosis. Arch Dermatol. 1991;127(3):378–386.
than pemphigus vulgaris, but there are refractory cases in                  doi:10.1001/archderm.1991.01680030098014. PMID: 1998369.



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