User Tools

Site Tools


grover

Grover's disease (transient acantholytic dermatosis)

Introduction

  • a common acquired itchy mainly truncal rash characterised by acantholysis
  • described by Grover in 1970

Epidemiology

  • most often affects Caucasian men over 50 years of age with sundamaged skin
  • said to occur in 0.1% of the total population with a male-to-female ratio of 2.4:1 and average age = 61yrs 1)
  • risk factors include sun-exposure, sweating, fever, B symptoms of malignancy, and being hospitalised or bedridden.
  • a Grover-like rash has been reported during the febrile phase of COVID-19.
  • increasingly reported with the use of BRAF-inhibitors
  • may be more common in winter - some suggest it is 4x more common in winter than in summer
  • asteatotic eczema occurs five times as often among patients with TAD as in controls
  • some believe it arises against a backdrop of a xerotic epidermis with decreased sweat production rather than being caused by sweating and heat as previously postulated

Clinical features

  • often starts quite suddenly after being excessively hot or febrile in bed
  • most common sites affected are central back, mid chest, and upper arms and is usually very itchy
  • may affect legs
  • small, discrete, sometimes crusted, erythematous papules and papulovesicles with occasional vesicles, crusting, and erosions
  • duration is variable although spontaneous self-resolution in 2-4 weeks is typical
  • it is often relapsing and seasonal - 46% recurred, 11% persistent while only 43% resolved without recurrence

Biopsy histology

  • 5 classical patterns which can occur separately or simultaneously:
    • pemphigus-vulgaris like (47%)
    • Darier-like (18%)
    • Hailey-Hailey-like (8%)
    • spongiotic-like (9%)
    • pemphigus foliaceous–like (9%)
    • mixed (9%)
  • All variants share the common distinguishing features of acantholysis and dyskeratosis

DDx

  • Galli-Galli
    • presents similarly to Grover disease in predominantly adult males as small, slightly keratotic, variably colored papules; however, focally, a reticulated pattern, as seen in Dowling-Degos disease, should be appreciated
    • Galli-Galli can affect a larger portion of the body, including the hands, groin, and lower extremities
    • histology shows similar elongation and interdigitating downgrowths of the rete ridges found in Dowling-Degos disease, and absence of the various acantholytic patterns seen in Grover disease
    • mainly appears between adolescence and middle age
    • presents with hyperpigmented macules in the skin folds
    • thought to be an allelic variant of Dowling-Degos disease (DDD) - a AD with variable penetrance hereditary condition or spontaneous mutation with frameshift or nonsense KRT5 gene mutations lead to haploinsufficiency of keratin 5, a protein that plays an important role in cell-cell adhesion, epidermal differentiation, and melanosome uptake
    • 1st described in 1982
  • Epidermolysis bullosa with mottled pigmentation – as with Galli-Galli, is caused by keratin 5 mutation; has additional features to Galli-Galli of palmoplantar hyperkeratosis, bullous lesions.
  • Darier disease
    • an AD genetic disorder (ATP2A2, found on chromosome 12q23-24.1. This gene codes for the SERCA enzyme or pump (SarcoEndoplasmic Reticulum Calcium-ATPase)) classified as a hereditary acantholytic dermatosis
    • typically presents with persistent, greasy, scaly crusted papules in a seborrhoeic distribution and in skin folds which usually starts from adolescence
  • pemphigus vulgaris
  • pemphigus foliaceous
  • benign familial pemphigus (Hailey-Hailey disease)

Mx

  • control fever
  • avoid sun / sweating
  • vitamin D
  • antihistamines for relief of pruritus
  • 50% of patients respond to topical corticosteroids
  • sustained remission has been described after a course of systemic corticosteroids
  • skin biopsy may be needed for diagnosis
  • chronic severe disease may require isotretinoin or topical adapalene
grover.txt · Last modified: 2022/07/15 07:26 by gary1

Donate Powered by PHP Valid HTML5 Valid CSS Driven by DokuWiki