Dermatology Online Journal

Unilesional pemphigus vulgaris of the scalp
Gerardo Ferrara¹, Cesare Massone², Iris Zalaudek², Giuseppe Argenziano³
Dermatology Online Journal 15 (10): 9

1. Anatompic Pathology Unit, Gaetano Rummo General Hospital, Benevento, Italy. gerardo.ferrara@libero.it
2. Department of Dermatology, Medical University of Graz, Graz, Austria
3. Department of Dermatology, Second University of Naples, Naples, Italy

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Figure 1	Figure 2
Figure 1. The keratotic lesion of the scalp before (left) and after (right) the curettage and the biopsy at its base. Figure 2. The cytopathologic finding of acantholytic cells on Diff-Quik (left) and Papanicolaous (right) stained smears (x400)

A 57-year-old man was seen for a three-month history of an itchy, keratotic plaque of the scalp, (Fig. 1, left); clinical examination was otherwise unremarkable. The lesion was curetted followed by a punch biopsy at its base (Fig. 1, right).

The curetted material was smeared onto glass slides and stained with Diff-Quik (Fig. 2, left) and with Papanicolaou stains (Fig. 2, right). Cytopathologic examination disclosed many single keratinocytes with polygonal contours, dense cytoplasms, and enlarged vesicular nuclei with small to medium-sized nucleoli. Some synctitial clusters were seen as well (Fig. 2, left); a minority of cells showed small pyknotic nuclei (dyskeratotic cells; Fig. 2, top right).

Figure 3	Figure 4
Figure 3. The histologic finding of suprabasilar clefts (x25) with floating acantholytic keratinocytes (insets, x250) Figure 4. Direct immunofluorescence of hair plucked from the lesion: intercellular IgG deposits within the outer root sheaths of the hair (x40) with the typical 'network' pattern of pemphigus vulgaris (inset, x400)

The histologic picture was characterized by a 'naked' epidermal basal layer and a cleaved adnexal epithelium; keratinocytes floating within the clefts showed enlarged nuclei and round cytoplasmic contours (acantholytic cells) (Fig. 3). These histopathological features were consistent with pemphigus vulgaris (PV). However the immunoblotting technique for anti-desmoglein 1 and anti-desmoglein 3 circulating antibodies proved negative. Direct immunofluorescence of hair plucked from the lesion was subsequently performed: intercellular IgG deposits within the outer root sheaths of the hair were found with the typical 'network' pattern of PV (Fig. 4).

Systemic steroid therapy (deflazacort 90 mg/day p.o.) was administered. After one month, a complete remission of the disease was obtained. After one further month, a 50 percent reduction of the corticosteroid dosage every two weeks was begun. Ten months after the diagnosis, on a very low corticosteroid dosage (deflazacort 6 mg on alternate days) the patient had no evidence of disease.

Pemphigus vulgaris is an autoimmune blistering disease of the skin and the mucous membranes in which autoantibodies against desmosomal glycoproteins (desmoglein 3 more frequently than desmoglein 1) are responsible for a loss of keratinocyte cell-to-cell adhesion [1]. The typical clinical picture of PV is a widespread mucocutaneous bullous eruption. At its onset, however, the disease can appear as deceptively banal: mucosal (oral, genital) erosions are common. Exudative lesions of the skin folds (axilla, umbilicus, groin) and/or of the nail folds can be also found; proliferative lesions ('acanthomata') [2] can be present because of an epidermal damage-repair cycle. In this early phase, the diagnosis of PV can be difficult because of the possible absence of circulating autoantibodies [3, 4].

Dermatologists should be aware of all these potential pitfalls because the earlier the diagnosis of PV the lower the effective dosage of immunosuppressive drugs. The first diagnostic step can be Tzanck cytopathologic examination [5], a simple, quick, and inexpensive test in which acantholytic cells can be easily found as polygonal cells with deeply stained cytoplasms and enlarged vesicular nuclei. Conventional histopathology shows a characteristic, albeit not pathognomonic, suprabasilar acantolysis with a 'tombstone' basal layer and a dermal infiltrate with eosinophils. Direct immunofluorescence on snap-frozen biopsy specimens taken in and around the affected areas shows the diagnostic 'network' pattern, with IgG (and, less frequently, C3, IgM, and IgA) depositions within the intercellular spaces of keratinocytes.

The scalp is commonly involved in disseminated PV. However, the clinical onset of PV as a single lesion of the scalp is distinctly unusual. The clinical picture we have shown is rather non-specific because other inflammatory and neoplastic diseases of the scalp can be hyperkeratotic. A Tzanck cytopathologic examination can effectively assist the clinical evaluation of these lesions.

PV of the scalp causes anagen effluvium, i.e., loss of hair with their outer root sheaths [6]. Since acantholysis takes place along the outer root sheaths, direct immumofluorescence on plucked hair can disclose pathognomonic peripilar immunoglobulin depositions [7].

Systemic corticosteroids remain the mainstay of therapy for PV. However, most of the deaths of PV patients are now caused by complications of therapy. Adjuvant and/or alternative therapies must be thus investigated [8]. Among these, topical corticosteroids [9] and topical tacrolimus [10] have been proposed for (localized) pemphigus foliaceus and may be effective for early localized PV.

References

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