A 43 Year Old with Vegetative Plaques and Erosions on the Trunk

Figure 14.1

Clinical findings at the time of presentation with large vegetative plaques with deep erosions over the abdomen (a) that were significantly improved 2 weeks after treatment with a single cycle of IVIg (b)

Based on the case description and the clinical image, what is your diagnosis?

Pemphigus foliaceus

Mucosal predominant pemphigus vulgaris

Mucocutaneous pemphigus vulgaris

Cutaneous pemphigus vulgaris

Bullous pemphigoid

Biopsy for routine histology revealed suprabasilar clefting with numerous acantholytic keratinocytes with prominent extension down follicular epithelium. Biopsy for direct immunofluorescence showed deposition of IgG in the intracellular spaces (ICS) of the epidermis. Indirect immunofluorescence was positive with IgG reactivity in the ICS spaces of monkey esophagus at a final titer of 1:640. Anti-desmoglein (Dsg) 1 and Dsg3 ELISA studies revealed index values of 163.4 and 119.4, respectively (normal range <20U).

At the time of presentation, she was failing prednisone 80 mg daily. She was treated with 1 cycle of intravenous immunoglobulin (IVIg) and azathioprine was added as a steroid sparing agent. Two weeks following IVIg she showed significant clinical improvement (Fig 14.1b). Her prednisone was successfully tapered and she was controlled on azathioprine as monotherapy.

Diagnosis

Cutaneous pemphigus vulgaris

Discussion

Pemphigus vulgaris (PV) is a group of autoimmune blistering disorders mediated by autoantibodies against the desomosomal adhesion proteins Dsg1 and Dsg3. Three distinct clinical forms of PV have been described (Table 14.1):

Table 14.1

Histological and immunological findings in pemphigus variants

	Mucosal PV	Mucocutaneous PV	Cutaneous PV	Pemphigus foliaceus (PF)
H&E	Suprabasilar cleft	Suprabasilar cleft	Suprabasilar cleft	Subcorneal cleft
Site(s) involved	Mucosa	Mucosa	——	——
Site(s) involved	——	Skin	Skin	Skin
Antigenic target(s)	Dsg3	Dsg3	Dsg3	——
Antigenic target(s)	——	Dsg1	Dsg1	Dsg1

Mucosal predominant PV (mPV) is characterized clinically by mucosal erosions that most commonly affect the oral mucosa, but can also involve the nasal and genital mucosa as well. Routine histology shows suprabasilar clefting and acantholysis. Direct immunofluorescence (DIF) shows deposition of IgG at the ICS spaces throughout the epidermis. Indirect immunofluorescence (IIF) confirms circulating IgG with reactivity to the ICS spaces with monkey esophagus as the preferred substrate. The autoantibodies in mPV are limited to Dsg3 specificity alone. Interestingly, many pemphigus patients recall lesions limited to the mucosal surfaces at the onset of their disease. While some patients remain with lesions limited to the mucosa for the duration of their disease and are eventually categorized as mPV, most patients progress to mucocutaneous PV [1, 3].

Only gold members can continue reading. Log In or Register to continue