ARTICLE
Auteur(s) :, Iqbal
Bukhari*
Dermatology Department, College of Medicine, King Faisal
University, Dammam, Saudi Arabia.
accepté le 2 Août 2004
Transient acantholytic dermatosis (TAD) is an acquired
papulovesicular eruption which was first described by Ralph W.
Grover in 1970 [1]. Clinically, the eruption begins with red brown
or flesh colored papules, papulovesicles or keratotic papules
affecting the trunk and extremities, associated with mild pruritus
[2]. Here a patient with TAD is presented, who had bullous lesions
similar to pemphigus vulgaris with minimal pruritus and
histopathologic findings showing a mixture of the
spongiotic-acantholytic pattern and the bullous pattern.
Case report
A 14-year-old Pakistani female presented to our dermatology clinic
with a one month history of a mildly pruritic bullous eruption
affecting the trunk and upper and lower extremities including the
palmoplantar surfaces. The patient’s problems started one month
previously with the appearance of itchy palmar lesions after
playing with chalk in school. The lesions were papular at the
beginning but later became vesicular. A few days later, she noticed
the appearance of new papulovesicular and bullous lesions on the
trunk and the extremities together with the palms and soles. The
condition progressively became more widespread over a period of 4
weeks. She was unable to use her hands and had difficulty in
walking due to the large bullae on the feet. Her past medical
history, drug history and family history was unremarkable. In the
beginning, she was seen at a private clinic and was prescribed
topical clobetasol propionate but no improvement was noticed,
instead more lesions erupted. On examination of her skin, large
bullous lesions of different sizes ranging from 2-5cm in diameter
were seen distributed on the upper and lower extremities while
papulovesicular lesions were mainly seen on the trunk ( (figure 1) ). Nikolsky sign
was easily elicited. Face, scalp, neck, mucus membranes and nails
were normal. Her basic blood investigations were within normal
limits. A biopsy of a newly formed lesion on the arm showed tense
well-circumscribed, intraepidermal, spongiotic vesicles containing
a few acantholytic cells but there was no dyskeratosis,
parakeratosis, acanthosis or œsinophils. In addition, a single-cell
tombstone layer of basal keratinocytes at the base of the blister
was not seen. The dermis was edematous with mild a perivascular
lymphohistiocytic infiltrate ( (figure 2) ). Direct
immunoflorescence testing from the periphery of a lesion on the arm
was negative. So with those clinicopathological findings our final
diagnosis was bullous variant of Grover’s disease. After managing
the large bullae, the patient was started on Fucicort cream
(betamethasone valerate 0.1% with fusidic acid 2%) twice a day for
10 days. Fortunately, lesions started to regress and healed
completely within three weeks. The patient was followed up in the
clinic at four month intervals for a period of twelve months with
no recurrence.
Discussion
Transient acantholytic dermatosis (TAD), also known as Grover’s
disease, is an acquired, monomorphous papulovesicular eruption
which was first described by Ralph W. Grover in 1970 [1]. It is a
disorder that has been reported worldwide with male to female ratio
3:1 [3-5]. Clinically, the eruption usually begins with a few
discrete erythematous, edematous, acneiform, red-brown or flesh
colored papules, papulovesicles or keratotic papules [2, 6, 7].
Their size usually ranges from 1-3 mm in diameter but can be
up to 1 cm. The condition begins on the trunk and then spreads
to the neck and thighs but the palms, soles, scalp and mucus
membranes are usually spared [2, 8, 9]. Other clinical variants of
the disorder have been described such as vesiculopustular [11],
bullous [12], nummular [1], follicular [13], herpetiform [14] and
zosteriform [15]. In most patients, there are no constitutional
symptoms but only pruritus of variable intensity [10]. In our case
the lesions were mainly bullous, similar to pemphigus vulgaris
presentation, with minimal pruritus. The pathogensis of TAD is
unknown but many factors have been associated with it such as
sunlight, excessive sweating [16], ionizing radiation [17], drugs
such as sulfadoxine-pyrimethamine and recombinant human
interleukin-4 [18, 19], nonspecific irritation and inflammation
such as asteatotic eczema, contact dermatitis and atopic dermatitis
[3]. TAD has also been associated with genitourinary tract and
hematologic malignancies [17]. In our patient there was a positive
history of irritation of the hands by chalk just prior to the
appearance of the lesions, which could be the triggering event. The
histologic features of the disease usually include acantholysis,
dyskeratosis, spongiosis with hyperkeratosis, acanthosis and
parakeratosis. The dermis may be edematous and contain a
perivascular lymphohistiocytic infiltrate [2, 11]. Besides this,
there are many histological patterns that could predominate the
picture, such as Darier’s disease pattern [20, 21], Hailey-Hailey
disease pattern [21], pemphigus vulgaris-like pattern [22],
pemphigus foliaceous-like pattern [23], spongiotic-acantholytic
pattern [20], and the bullous pattern [12]. Our patient’s
histopathologic findings were a mixture of the
spongiotic-acantholytic and the bullous patterns. In addition, the
results of both direct and indirect immunoflorescence are usually
negative in TAD and when positive they are not consistent [24].
Electronmicroscopic studies have demonstrated dissolution of
desmosomal attachment plaques which leads to the acantholysis
observed in this disease [25]. Immunohistochemistry studies have
confirmed the involvement of desmoplakin I, II, plakoglobin and
desmoglein in the acantholytic process [25-27]. TAD has a
self-limited course and most lesions clear spontaneously within
several weeks but some cases may be persistent or recurrent.
Patients should be advised to avoid excessive exposure to the sun
and strenuous execise [28]. Topical soothing baths, bath oils, zinc
oxide and low potency steroid creams may relieve itching [7].
Systemic therapy is needed when the eruption is severely pruritic,
extensive and persistent. Oral vitamin A [22], isotretinoin [22,
29], etretinate [30], prednisone [29], methotrexate [22], PUVA [31]
and Grenz irradiation [32] were all reported to be effective in
this disorder. So our final diagnosis of this case was bullous
variant of TAD because of the positive history of irritation,
bullous appearance of the lesions, mixed histopathologic pattern,
negative immunoflorescence, and the spontaneous resolution of the
lesions within weeks.
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