Figure 7.1
Hairless cerebriform vegetating mass on the scalp covered with pustules
Based on the case description and the photograph, what is your diagnosis?
1.
Pemphigus vegetans
2.
Pemphigoid vegetans
3.
Pyodermatitis-pyostomatitis vegetans
4.
Wart
Diagnosis
Pemphigus Vegetans
In 2004 the patient presented with oral and skin bullae and erosions of 3 months duration. Oral biopsy and direct immunofluorescence were performed which showed suprabasal acantholysis and intercellular IgG and C3 deposits, respectively and were compatible with pemphigus vulgaris (PV). Treatment was started with prednisolone 1.5 mg/kg/day (120 mg/day) and azathioprine 2 g/kg (150 mg/day). After control of his condition, prednisolone was tapered gradually. He was unable to taper his prednisolone below 15 mg/day but was in complete control of his blistering at this dose, until 1 year later, when he experienced a minor relapse on the scalp, chest and oral mucosa when he was receiving prednisolone 15 mg/day and azathioprine 150 mg/day.
In the following years he never became free of scalp lesions. Only erosions and crusted lesions were seen initially. Lesions gradually showed features of tufted folliculitis, then progressed indolently to vegetating plaques. Pull test from the involved areas revealed anagen hair loss in the active phases of disease. The patient also experienced a few minor relapses involving the oral mucosa and skin besides his persistent progressive scalp lesions. Enzyme-linked immunosorbent assays were positive for anti-Dsg 3 (>200 U/ml) and negative for anti-Dsg 1 (<20 U/ml) on two occasions.
Adding Cellcept 2 g/day for 9 months, methotrexate 15 mg/week for 5 months or minocycline 100 mg/day for a few months to the 15–30 mg prednisolone did not change the progressive course of his disease. Scalp lesions neither showed any response to multiple weekly intralesional injections of triamcinolone acetonide. The patient was never off treatment from steroids. He had poor compliance to treatments with irregular follow-up visits and suffered from uncontrolled diabetes mellitus.
His condition showed only some improvement while taking prednisolone 20 mg/day, methotrexate 20 mg/day as well as a course of clindamycin and ciprofloxacin, in his last visit in January 2014.
Figure 7.2 shows the dermoscopic view of the scalp examined in his last visit.
Figure 7.2
Dermoscopy of the scalp lesion showing vegetations, sparse hair, pustules and crusts in the empty follicular ostia
Discussion
PV is a chronic autoimmune blistering disease of the skin and/or mucosa characterized by the presence of autoantibodies targeting desmoglein 3 and to a lesser extent desmoglein 1. Pemphigus vegetans (P Veg) is a rare clinical variant of PV and historically divided into Neumann and Hallopeau types, depending on the types of primary lesions and the clinical course. P Veg of Neumann type presents initially as PV with flaccid blisters and erosions. Its course is also similar to classical PV, however the erosions does evolve into vegetating verrucous excrescences especially in the intertriginous areas (axillae, groin, lip commisures). Local moisture, heat, friction, as well as secondary bacterial (Staphylococcus aureus) and fungal (Candida albicans) infections are important in the development and persistence of lesions. Management of vegetations is based on local hygiene, frequent bathing, local antiseptics and astringents, topical antibiotics, topical antifungals, oral antibiotics, and weekly intralesional triamcinolone injections. According to our experience, intralesional triamcinolone injections are usually effective. The dose of oral steroids should not be increased.
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