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Grover’s Disease / Transient Acantholytic Dermatosis

Key Points

  • Grover’s disease is a pruritic condition that typically affects adult individuals and classically presents on the trunk and proximal extremities.
  • The characteristic lesion is a crusted erythematous papule, pustule, or papulovesicle.
  • Grover’s may be triggered by heat, sweating, and occlusion and may persist for years. Sun damage and xerosis may be important risk factors.
  • The strategy is to suppress the pruritus and if possible clear the cutaneous lesions. Gentle skin care and frequent emollient use may prevent recurrences.
  • Certain medications have been reported in association with Grover’s disease.

Introduction

Grover’s disease, also known as transient acantholytic dermatosis (TAD), is a common pruritic condition that typically affects adult individuals and classically presents on the trunk and proximal extremities. It may be more common in Caucasian individuals, especially in the fifth to seventh decades of life. The characteristic lesion is a crusted erythematous papule, pustule, or papulovesicle. Lesions of transient acantholytic dermatosis (TAD) often are not “transient,” and may persist for years, especially in the inframammary regions. Heat, sweating, and occlusion appear to be triggers, with patients presenting after trips to tropical or semitropical climates, with fever, and after hospitalization or bed rest. Immunosuppression may increase the risk of developing TAD, as it is seen in patients with HIV infection, in leukemia, and in patients recently receiving bone marrow transplants. History of extensive sun exposure and xerosis may also be important risk factors. Pruritus may be severe. The strategy is to suppress the pruritus and if possible to clear the cutaneous lesions. Gentle skin care and frequent emollient use may prevent recurrences.

Certain medications have been reported in association with Grover’s disease: anastrozole, vemurafenib, dabrafenib, cetuximab, mercury, D-penicillamine.

Initial Evaluation

Differential diagnosis

Seborrheic dermatitis, chest

Darier’s disease

Psoriasis

Secondary syphilis

Pemphigus foliaceus

Treatment

First-line therapy: The first-line therapy for Grover’s is to suppress pruritus and when possible, reduce inflammation and clear lesions.

First steps

  • If TAD is not pruritic, treatment may not be required.
  • A high-potency or superpotent steroid cream, such as fluocinonide 0.05% or clobetasol 0.05% cream, applied twice daily can be effective in some patients and may suffice for patients with mild disease. Occlusion with a sauna suit may enhance therapy, although care must be taken to avoid overheating and sweating.
  • Topical tretinoin gel 0.025% or tazarotene gel 0.05% or 0.1% applied once to twice daily may be effective. Often topical application must be continued to the point of irritation in order for the lesions to resolve. Superficial desquamation should be anticipated.

Ancillary steps

  • An emollient lotion containing menthol, phenol, and camphor may provide temporary relief. Thicker emollients are preferred, with caution for worsening of lesions due to occlusion by thick creams or ointments.
  • Antihistamines (e.g., doxepin 10–25 mg, hydroxyzine 10–50 mg, or diphenhydramine 25–50 mg as a single evening dose) may help control the pruritus.
  • Avoidance of sweating may prevent exacerbations.

Subsequent steps

  • Topical calcipotriene may also be helpful.
  • Photochemotherapy (PUVA) is effective in some cases; an initial brief exacerbation may occur, however, before clearing.
  • For patients with extensive or severely pruritic disease, begin low-dose isotretinoin 20–40 mg/day. Response may take more than a month. The dose can be tapered gradually as the condition improves; however, often 4–6 months of low-dose isotretinoin (10 mg/day) may be required to induce a remission.
  • Systemic steroids can provide temporary relief, and can induce remissions. Relapses may occur. Systemic steroids should be reserved for the most severely pruritic patients, and an alternative treatment, as noted above, that will allow transition off the systemic steroids should be started if the systemic steroids are required for longer than one month in a dose exceeding 10 mg/day.
  • Other systemic therapies reported in the literature with potential efficacy for TAD include methotrexate, and etanercept.

Pitfalls

  • TAD, despite its name, may not be transient, and may persist for years.
  • Failure to improve with the suggested treatments should raise the consideration of secondary infection; infection of Grover’s disease with herpes simplex virus, Staphylococcus aureus, and scabietic infestation have been reported in the literature.
  • The important differential diagnosis of Darier’s disease and pemphigus foliaceus should be considered in unusual or extensive presentations.
  • The potential side effects of topical and systemic retinoids and topical steroids should be monitored.

When to refer to a dermatologist

  • When the diagnosis of Grover’s is not clear.
  • If pruritus associated with Grover’s cannot be mitigated.
  • If infection of Grover’s disease is suspected.
  • For persistent cases in which photochemotherapy may be helpful adjunctive treatment.

Clinical Case

Case 1

  • 49-year-old Caucasian male
  • No significant past medical history except for childhood eczema and allergic rhinitis
  • Recently returned from a one-month hiking trip to Australia
  • Presents for management of severe pruritic eruption that started on his trip
  • Denies any new cosmetic, plant, or insect exposures
  • Currently using diphenhydramine cream for relief of pruritus
  • Reports development of new lesions since returning from his trip.

Initial evaluation

  • Very healthy-appearing male
  • Monomorphous erythematous crusted papules scattered across the chest and back
  • Diagnosis: Grover’s disease (transient acantholytic dermatosis)
  • Fluocinonide 0.05% ointment once daily and frequent emollient use is recommended.
  • A prescription for hydroxyzine 25-50 mg at bedtime is also given to suppress nocturnal pruritus.
  • Patient is counseled to avoid sweating and occlusion, when possible.
  • Follow-up in four weeks

Follow-up evaluation

  • There are fewer lesions but the patient reports ongoing pruritic lesions. He admits to not using emollients
  • Ongoing fluocinonide ointment applied once daily and frequent emollient use is again recommended; education on use of emollients is discussed and a handout on gentle skin care is given (See handout Skin Care in Atopic Dermatitis.)
  • Follow-up in four weeks
  • The patient is improved; the natural history of TAD is discussed, as recurrences are common, and the need for ongoing emollient use is reinforced.

References

Gupta M, Huang V, Linette G, Cornelius L (2012) Unusual complication of vemurafenib treatment of metastatic melanoma: exacerbation of acantholytic dyskeratosis complicated by Kaposi varicelliform eruption, JAAD, 148:966-968.

Norman R and Chau V (2011) Use of etanercept in treating pruritus and preventing new lesions in Grover disease, JAAD, 64: 796-798.

Quirk CJ and Heenan PJ (2004) Grover’s disease: 34 years on, Aust J Derm, 45:83-88.