Dear Editor,
A three-and-a-half-month-old male child presented with fluid-filled lesions and redness over the body since six weeks. The mother noticed red raised lesions initially on his soles, perianal, and genital regions, acute in onset, which gradually spread over the trunk, and extremities which was associated with the formation of multiple blisters, and excessive itching. There was no history of fever or any infection. Birth and developmental history were normal. The child was vaccinated till date. Mucocutaneous examination revealed multiple well-defined urticarial plaques with tense bullae, vesicles and erosions on the face, scrotal, perianal region, bilateral upper and lower limbs. Multiple crusted lesions with urticarial plaques were present on the scalp with no loss of hair [Figures 1 and 2]. Oral and genital mucosae were normal. Systemic examination was normal. The following investigations were done - Hb- 11.2, TLC- 19,000, DLC- N-24 L-62 M-2 E-12, LFT, RFT - Normal, G6PD level- 18.22 units/gm (normal levels 6.6–17.2). Biopsy showed intraepidermal bullae with eosinophils [Figure 3]. Direct immunofluorescence (DIF) from perilesional skin showed a linear deposition of IgG and C3 at the dermoepidermal junction [Figure 4].
Figure 1.
Multiple crusted lesions with urticarial plaques were present on trunk, upper limbs and the scalp with no loss of hair
Figure 2.
Multiple crusted lesions with urticarial plaques surmounted with bullae on both lower limbs
Figure 3.
Biopsy showed intraepidermal bullae with eosinophils (H and E, 10x)
Figure 4.
Direct immunofluorescence from perilesional skin showed a linear deposition of IgG and C3 at the dermoepidermal junction
Since the lesion that was taken for biopsy was a bit old, there were intra-epidermal bullae with no acantholytic cells, but DIF findings were suggestive of bullous pemphigoid (BP).
Based on the history, clinical examination, skin biopsy and DIF findings, a diagnosis of BP was made. Oral prednisolone 2 mg/kg body weight and antihistamine for 15 days were advised. After one month, a dose of 1 mg/kg of intravenous immunoglobulin (IVIG) was given. The patient was asked to follow up after one month. The patient showed improvement, and at follow up, most of the lesions had already healed and subsided [Figure 5].
Figure 5.
Post-treatment picture showing that most of the lesions have healed and subsided
BP in infancy occurs within hours to days after vaccinations. Infants have acral involvement with or without generalized blistering.[1] The widespread involvement predominates in infants.
Mucous membrane involvement and involvement of the hands and feet in infants under one year old appear to be more common in childhood BP. BP in children is relatively rare. More than sixty cases are reported in literature. The number of new case reports in infants has increased in the last decade. While just a few children with the condition have been studied immunologically, antibodies against the 180-kDa antigen (BP180) appear to be involved in the etiology of the disease, similar to adult BP.[1]
Treatment of choice includes systemic corticosteroids of 1 to 2 mg/kg/day of prednisone. Dapsone at a dose of 2–4 mg/kg/day to induce remission.[2] For resistant cases, intravenous immunoglobulin (IVIG), intravenous pulse methylprednisolone, oral mycophenolate mofetil, cyclosporine, sulfapyridine, and erythromycin have been tried.[1]
In circumstances where clinical or biopsy findings are inconclusive, immunofluorescence aids in cinching the diagnosis. This case is being presented because of its rarity and the need for immunofluorescence study.[3]
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Department of dermatology, SSIMS Davangere.
Conflicts of interest
There are no conflicts of interest.
Acknowledgement
I would like to express my heartfelt gratitude to my beloved parents, Dr. Nagarathana Kololgi and Dr. Prakash Kololgi, for their unwavering support and guidance throughout the process of writing this article for publication. Your encouragement and wisdom have been invaluable, and I am truly grateful for your constant belief in me.
References
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