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Transient intra-epidermal bullous dermatosis

European Journal of Dermatology. Volume 15, Number 4, 288-90, July-August 2005, Clinical report

Summary

Author(s) : Iqbal Bukhari , Dermatology Department, College of Medicine, King Faisal University, Dammam, Saudi Arabia..

Summary : Transient acantholytic dermatosis is an acquired, mildly pruritic, papulovesicular disease affecting the trunk and extremities. In this report a case of the bullous variant of this disease is described, with a brief review of the literature.

Keywords : acantholysis, bullous transient acantholytic dermatosis, dyskeratosis, Grover’s disease, Hailey-Hailey disease, pemphigus vulgaris

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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.

References

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