DOI: 10.1111/pde.

13348

Pediatric
Dermatology

Itchy bumps with new-onset bullae

Syed H. Mahmood BS1,2 | Anthony Van Vreede MD2 | David Mehregan MD2
1
Wayne State University School of Medicine, Detroit, MI, USA
2
Department of Dermatology, Wayne State University School of Medicine, Dearborn, MI, USA

Correspondence
Syed H. Mahmood, BS, Wayne State University School of Medicine, Detroit, MI, USA.
Email: shmahmoo@med.wayne.edu

Editor: Antonio Torrelo MD

A 13-year-old African American boy presented to the clinic with a 2-

month history of itchy bumps on the arms, legs, and trunk (Figure 1).
One week before he had been diagnosed with a scabies infection
and was treated with permethrin, but potassium hydroxide and min-
eral preparation tests were negative on the day of presentation.
Physical examination revealed flat-topped, violaceous and gray
papules with a fine white reticular pattern. A punch biopsy was
obtained (Figure 2). The biopsy showed a dense bandlike lymphocy-
tic infiltrate in the papillary dermis, sawtooth pattern of rete ridges,
degeneration of the basal cell layer, and an acanthotic epidermis. FIGURE 3
The patient was prescribed topical triamcinolone and then a pred-
nisone taper and hydroxyzine therapy as the condition worsened,
but he was not compliant with treatment.

FIGURE 4

Nine weeks later, he presented with xerosis, vesicles, and bullae in

different stages of healing on the arms and legs, in areas that the origi-
nal rash had and had not affected. Examination of these areas showed
FIGURE 1 tense bullae with negative Nikolsky and Asboe–Hansen signs and large
erosions with beefy underlying granulation tissue with no visible der-
mis or purulent discharge (Figure 3). The bilateral lower legs and dorsal
feet were more affected than the upper extremities and face, but the
oral mucosa was not involved. Hepatitis panel, enzyme-linked
immunosorbent assay (ELISA) for the human immunodeficiency virus,
and rapid plasma regain tests were negative. Complete blood count,
comprehensive metabolic profile, and fasting lipids were within normal
limits. A biopsy from a bulla was obtained (Figure 4).

FIGURE 2 1 | WHAT IS THE DIAGNOSIS?

Pediatric Dermatology. 2018;35:141–142. wileyonlinelibrary.com/journal/pde © 2018 Wiley Periodicals, Inc. | 141

142 | Pediatric
Dermatology
1.1 | Diagnosis: Lichen Planus Pemphigoides
2 | DISCUSSION
Lichen planus pemphigoides (LPP), which Kaposi first described in
1892,1 is an autoimmune disease characterized by the appearance of
bullous lesions in individuals with lichen planus (LP). The disease is
rare in childhood, with only 17 cases previously reported.2-5 The
mean age of presentation for childhood cases is 10.5 years, with a
male:female ratio of 2:1.2-5 On average, the time to develop bullous
2-5
lesions from the onset of LP is 6.8 weeks.
The diagnosis of LPP can be made according to clinical appearance,
light microscopy, and direct immunofluorescence of peribullous skin.
Clinically, patients have typical LP lesions, although oral involvement is
less common. Patients later develop bullae on the extremities. Lesions
on the trunk are less common. Histopathologic examination of the
papules shows typical features of LP, whereas that from bullous
lesions shows features of LP in association with a subepidermal split.
Direct immunofluorescence reveals linear deposition of immunoglobu-
lin (Ig)G or C3 in the basement membrane zone.2
Our patient initially exhibited the typical rash of LP, with polygo-
nal, scaly, flat-topped, violaceous papules with a fine, white, reticular
pattern on the surface. A biopsy showed a dense, bandlike, lympho-
cytic infiltrate in the papillary dermis with a sawtooth pattern of rete
ridges, degeneration of the basal cell layer, and a hyperkeratotic epi-
dermis consistent with LP (Figure 2). The patient later developed
tense bullae, and a biopsy of peribullous skin showed a subepidermal
vesicle and degeneration of the basal cell layer (Figure 4). Direct
immunofluorescence showed linear C3, fibrinogen, and weak IgG
deposition along the basement membrane zone.
It is likely that the pathogenesis of LPP involves epitope spreading,
a process in which sequestered antigens are exposed to the immune
system because of damage to a protective layer, leading to the devel-
opment of an autoimmune response.4 In the case of LPP, the dense,
bandlike, lymphocytic infiltrate in the papillary dermis of the initial LP
lesions disrupts the dermoepidermal junction, unmasking antigens that
then become the target for immunologic reactions.6 In LPP, the target
of the immune response is the NC16A domain of bullous pemphigoid
(BP) antigen 2.7 This antigen is also targeted in BP, but Zillikens and
colleagues8 found that the specific domains within NC16A responsible
for antigenic response may differ, indicating that the diseases are dis-
tinct. In our patient, ELISA demonstrated circulating IgG against the
MAHMOOD ET AL.
BP180 antigen at 175 U (normal <9 U), whereas IgG against the
BP230 antigen was within normal limits.
LPP is distinct from BP and bullous LP (BLP). In LPP, bullae
appear on skin with LP and on normal skin, distinguishing the dis-
ease from BLP, in which bullae only appear on LP lesions.
Histopathology appears identical in BLP and LPP, so direct
immunofluorescence (negative in BLP) is necessary to differentiate
the two. Bullous pemphigoid does not have typical LP lesions, thus
allowing one to differentiate BP from LPP using a clinical examina-
tion.
Systemic steroids and dapsone are first-line treatment for LPP.5
Methotrexate also has been used for treatment.2 Our patient was
found to have glucose-6-phosphate dehydrogenase deficiency, so
treatment with dapsone was not initiated. Consideration of other
systemic therapy was to be discussed, but the patient missed his fol-
low-up appointments.
ORCID
Syed H. Mahmood http://orcid.org/0000-0002-7321-4072
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