Pediatric Dermatology Consult

Pediatric Dermatology Consult - July 2017

Publish date: July 13, 2017

Make the diagnosis

A 3-month-old otherwise healthy boy was referred for a 2-week history of a nonpruritic, urticariforme eruption that progressed to blisters on the trunk, hands, and feet. The patient had a 4-day history of a nonproductive cough preceding the rash. He was otherwise active with normal feeding and bowel habits. His family history was negative for dermatologic or autoimmune disease.

On examination, the patient is a well-developed, active male with widespread erythematous infiltrated papules and arcuate plaques, tense vesiculobullous lesions, and multiple erosions on the trunk and extremities. Mucous membranes and perineum were spared.

Bacterial culture of a lesion was negative. Consult from dermatology was obtained and a biopsy performed.

Histologic examination showed a subepidermal unilocular blister with serous fluid containing predominately eosinophils and lymphocytes. Direct immunofluorescence revealed linear immunoglobulin G and C3 deposition along the basement membrane.

What’s your diagnosis?

Bullous impetigo

Hand, foot, and mouth disease (HFMD)

Linear IgA dermatosis

Bullous pemphigoid

Dermatitis herpetiformis

BY JUSLEEN AHLUWALIA, MD, AND LAWRENCE F. EICHENFIELD, MD

Consider immunologic blistering diseases in the differential diagnosis of unusual vesicles and bullae in infants and children.

The patient was diagnosed with infantile bullous pemphigoid. Treatment was initiated with clobetasol 0.05% ointment twice daily to the affected areas. Despite treatment with topical corticosteroids, the patient continued to manifest bullae and urticariforme plaques on his legs and trunk. He was then started on oral prednisolone 1 mg/kg per day with gastrointestinal ulcer prophylaxis. He improved within 3 weeks of treatment.

Bullous pemphigoid (BP) is an autoimmune blistering dermatosis that is rare in childhood, and even rarer in infancy.1 It is caused by circulating immunoglobulin G antibodies directed against 180 kD proteins in the epidermal basement membrane, resulting in vesicles, bullae, and urticaria-like eruptions. Immunologic stimulations, such as vaccinations, have been noted to trigger the onset of childhood BP.1,2 Infants under the age of 12 months classically present with acral involvement, whereas older children typically have generalized involvement or localized genital BP.3 Age-dependent expression of BP antigen in specific areas of skin could explain this variance in clinical presentation.3,4

Overall, childhood BP carries a good prognosis and responds well to topical or oral steroids.1 Most patients achieve remission within a few weeks to months of treatments; however, disease course can vary with several relapses over a few years. The longest duration of disease that has been reported is 3 years.1 Second-line treatments – such as dapsone, sulfapyridine, mycophenolate mofetil, administered alone or concurrently with steroids – may be considered for those who fail to respond to conventional treatment.1,5 Intravenous immunoglobulin has reported to be efficacious and well-tolerated in a few cases of treatment-resistant childhood BP.1,5

Differentiation of infantile bullous pemphigoid from other common and uncommon vesiculobullous diseases is necessary. Bullous impetigo (A), caused by Streptococcus pyogenes or Staphylococcus aureus can present with vesicles and bullae due to staphylococcal exfoliative toxins (exfoliatin A-D) which cause separation at the superficial epidermal level. Hand, foot, and mouth disease (HFMD) (B) is an acute Coxsackie viral infection, classically involving the oral mucosa, palms, and soles, although broad variations in presentations include “eczema coxsackium,” Gianotti-Crosti–type lesions, and petechiae.6 Linear IgA dermatosis (C) is an immunobullous condition occurring most frequently in perioral and perineal regions. The disease is characterized by a linear deposition of IgA along the dermal-epidermal junction. Dermatitis herpetiformis (E) is an autoimmune, papulovesicular eruption most commonly involving extensor surfaces of extremities and is characterized by granular deposition of IgA in the papillary dermis.

This patient’s systemic steroids were successfully tapered over several months without disease recurrence. It is unknown why this immunobullous disease is usually self-limited in children, unlike BP in adults, which has significant morbidity or mortality. While uncommon, it is important to consider immunologic blistering diseases in the differential diagnosis of unusual vesicles and bullae in infants and children.

Dr. Ahluwalia is with the division of pediatric and adolescent dermatology, Rady Children’s Hospital, San Diego, and the departments of dermatology and pediatrics at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego and professor of dermatology and pediatrics at the University of California, San Diego. Neither of the physicians has relevant financial disclosures. Email them at pdnews@frontlinemedcom.com.

References

1. Pediatr Dermatol. 2016 Mar-Apr;33(2):e77-81.

2. Orphanet J Rare Dis. 2014 Dec 10;9:185.

3. J Am Acad Dermatol. 2008 Jan;58(1):41-8.

4. Arch Dermatol. 1991 Mar;127(3):378-86.

5. Pediatr Dermatol. 2015 Sep-Oct;32(5):723-6.

6. Pediatrics. 2013 Jul;132(1):e149-57.

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