Clinical Challenge: Itchy rash on the trunk - MPR

Clinical Challenge: Itchy rash on the trunk

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  • Grover

  • Grover

  • Grover

A 60-year-old white patient, admitted to the hospital for a congestive heart failure exacerbation, complains of extremely itchy papules on his chest and back. The eruption started a few days after admission, after which the patient been vigorously scratching.

Since hospital admission the patient has been receiving intravenous furosemide. His home medications include furosemide, potassium chloride, simvastatin, metoprolol and lisinopril. He has been using all of these medications for many months.

Grover's disease, also commonly called transient acantholytic dermatosis, consists of erythematous papules and papulovesicles that favor the trunk. The lesions can be extremely pruritic and are commonly crusted and excoriated.Grover's disease most commonly affects men in the 50-year-old to 60-year-old...

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Grover’s disease, also commonly called transient acantholytic dermatosis, consists of erythematous papules and papulovesicles that favor the trunk. The lesions can be extremely pruritic and are commonly crusted and excoriated.

Grover’s disease most commonly affects men in the 50-year-old to 60-year-old age group. While the etiology of the eruption is uncertain, known risk factors for development or exacerbation of the disease include heat, sweating, friction, sunlight and hospitalization.

Asteatotic eczema is a common association. The disease may be acute or chronic, but it tends to persist. The eruption may clear spontaneously within weeks, but it frequently follows a relapsing course over months to years.  The persistent nature of the disease has led some authors to propose renaming the disease “persistent and recurrent acantholytic dermatosis” which is more accurate than the currently favored term “transient acantholytic dermatosis.” 

Histologically, Grover’s disease may appear in four different patterns: Hailey-Hailey-like, Darier’s-like, pemphigus-like, and spongiotic with acantholysis. The Darier’s-like disease pattern is most common. Although Grover’s disease mimics these other diseases histologically, it is clinically distinct. 

Hailey-Hailey disease, also called benign familial pemphigus, is an autosomal-dominantly inherited disease due to mutations in the gene ATP2C1.  The onset of the skin eruption is in the late teens to twenties and consists of erosions in the neck, groin and axilla that can become malodorous and develop vegetations. 

Darier’s disease, also called ‘keratosis follicularis,’ is autosomal dominantly inherited disease due to mutations in the gene ATP2A2. The onset of the disease is frequently puberty. Patients develop crusted keratotic papules in a seborrheic distribution in addition to nail dystrophy, whitish papules in the oral mucosa and palmoplantar papules.

The history and clinical presentation in this case is not consistent with a morbilliform exanthem. 

Diagnosis & Treatment

Clinical diagnosis is based on the presence of pruritic and crusted erythematous papules and papulovesicles favoring the trunk in an elderly male. A biopsy can support the diagnosis.                        

Treatment is difficult. Removing any underlying exacerbating factors is important. As many as 50% of patients respond to treatment with topical steroids. Other therapies that have been reported to be successful include systemic steroids, systemic retinoids and phototherapy.                     

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.

References

  1. Bolognia J, Jorizzo JL, Rapini RP. Dermatology. Chapters 40 and 58. St. Louis, Mo.: Mosby/Elsevier, 2008.
  2. James WD, Berger TG, Elston DM et al. Andrews’ Diseases of the Skin: Clinical Dermatology. Chapters 21 and 27. Philadelphia: Saunders Elsevier, 2006.