記住我
Sir,
Pemphigus vulgaris (PV) is an autoimmune bullous dermatosis, developing due to autoantibodies against desmogleins 1 and 3.[1] Clinically, it manifests with painful mucosal erosions and flaccid bullae with erosions distributed mostly over the trunk, groin, armpits, scalp, and face.[2] We hereby present an unusual case of PV where the patient presented with multiple pustules having a targetoid appearance.
A 32-year-old lady presented with pustules on the extremities of 1-month duration and recurrent oral erosions for 6 months. She had consulted dentists, ear-nose-throat specialists, and physicians for oral lesions; however, the erosions kept recurring. Recently, she developed multiple pustules on the extremities, which healed in around 10–14 days with post-inflammatory hyperpigmentation, but newer lesions kept appearing. There was no history of weight loss or changes in bowel or bladder habits. An examination showed a well-built lady with palatal erosions and targetoid lesions (pustules on an erythematous base) over the extremities. [Figure 1]a, [Figure 1]b and [Figure 2] Nikolsky sign was negative. We kept clinical differentials of IgA pemphigus, paraneoplastic pemphigus, PV, erythema multiforme, Rowell syndrome and subcorneal pustular dermatosis. Histopathology showed intra-epidermal blisters in suprabasal location, with acantholytic cells within the blister cavity. [Figure 3]a Direct immunofluorescence (DIF) showed epidermal deposition of IgG in the intercellular spaces in a “fishnet” pattern. [Figure 3]b Considering histopathology and DIF, the case was diagnosed as PV. Assessment of autoantibodies against desmoglein 1 and 3 could not be done. Gram stain and culture from the pus did not show any organism. Routine blood investigations, lactate dehydrogenase, and protein electrophoresis were within normal limits. Serology for antinuclear antibody, computed tomography of the chest, abdomen and pelvis and mammogram were negative. She was treated with prednisolone (1 mg/kg/day for 6 weeks followed by tapering over 3 months) and azathioprine (50 mg twice daily). The lesions have healed with post-inflammatory hyperpigmentation [Figure 4], and new lesions have stopped appearing.
Figure 1: (a and b) Targetoid lesions on an erythematous base over the extremities
Figure 3: Photomicrograph showing suprabasal blister and acantholytic cells in the blister cavity (a) [hematoxylin and eosin, 400 ×] and direct immunofluorescence showing intercellular deposits of IgG (b) [100 ×]
Figure 4: Post-treatment resolution of the lesions with hyperpigmentationPV can present with atypical and rare manifestations like crusted plaques on the face and scalp, foot ulcers, dyshidrosiform dermatitis, macroglossia, nail dystrophy, paronychia, subungual hematomas and targetoid pustules (consistent with our case). There have been isolated reports of patients with PV presenting with pustules as reported by Rimal et al.[3] and Yang et al.[4] Another case of PV presenting with true target lesions coalescing into an annular configuration has also been reported.[5] Our case was unique because of targetoid pustules as predominant lesions, localization of the pustules on the extremities, pustules having no tendency to spread and form bigger erosions (unlike classical PV), and negative Nikolsky sign. These unusual features make the presentation unique; however, it is not clear if such a presentation represents a subset of PV with a different course and prognosis. Similar pustules predominant presentation has been described in pemphigus foliaceous too.[6]
Treatment of PV should be initiated as early as possible in order to achieve and maintain disease remission and prevent complications.[7] Corticosteroids form the mainstay of therapy in developing and resource-poor countries. It is prudent to start oral prednisolone at a dose of 1 to 2 mg/kg/day, followed by gradual tapering when remission has been achieved. Steroid-sparing immunosuppressives like azathioprine, mycophenolate mofetil, and cyclophosphamide are almost universally used. Rituximab appears to be the most preferred treatment for PV, and both lymphoma protocol and the protocol for rheumatoid arthritis have been used successfully.[7]
Other therapeutic options include cyclophosphamide 1 to 3 mg/kg/day orally or intravenously, dexamethasone-cyclophosphamide pulse therapy, methotrexate (10–20 mg/week), dapsone (50–200 mg/day), cyclosporine (3–5 mg/kg/day), intravenous immunoglobulins (0.4 g/kg/day for 5 days), plasmapheresis and immunoadsorption.[8]
With updates in the understanding of the pathomechanism of the disease, future therapeutic options are being investigated systematically. Some of them include modified chimeric antigen receptor (CAR) therapy to target Dsg3-specific B-cells, anti-CD154 monoclonal antibody to prevent the production of anti-Dsg3 IgG, B-cell activating factor (BAFF) inhibitor, A proliferation-inducing ligand (APRIL) inhibitor, p38 mitogen-activated protein kinase signaling pathway inhibitor, c-Myc and epidermal growth factor receptor inhibitors and Bruton's tyrosine kinase (BTK).
To conclude, as we delve deep into the molecular targets for the management of PV, dermatologists should be aware of the atypical clinical presentations of this elusive immunobullous disorder.
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
Nil.
Conflicts of interest
There are no conflicts of interest.
References 
留言 (0)