Paraneoplastic transient acantholytic dermatosis (Grover’s disease) along Blaschko lines

ARTICLE

Auteur(s) : Nadia Garçon¹, Allan Karam¹, Gilles Lemasson², Jean-Philippe Metges³, Laurent Misery¹

¹Department of Dermatology, University Hospital of Brest, 5 avenue Foch, 29200 Brest, France
²Department of pathology, University Hospital of Brest, 5 avenue Foch, 29200 Brest, France
³Department of oncology, University Hospital of Brest, 5 avenue Foch, 29200 Brest, France

A 73-year-old man with no prior history had a rectal carcinoma (T4 N2 M0), discovered by colonoscopy in the presence of anaemia. In November 2005, he presented with a pruritic rash on the back that had begun a few weeks before his hospitalisation. The dermatological examination found red to purple maculopapules with brownish crusts along the Blaschko lines (figure 1). The rest of the examination was normal. The histopathological examination showed a parakeratotic hyperkeratosis and a dyskeratotic acantholysis. Direct and indirect immunofluorescence showed no antibody deposits. No herpes infection was found by PCR in samples of cutaneous lesions.

Based on the clinical and histopathological features, the diagnosis of transient acantholytic dermatosis along the lines of Blaschko was made. No topical treatment was prescribed.

The rectal carcinoma was treated with surgery (rectosigmoid resection with colorectal anastomosis and protective ileostomy and partial cystectomy) and chemotherapy (6 doses of FUFOL folinic acid and 5-flurouracil 370 mg/m² from December to May 2006).

In January and May 2006, the evolution was marked by a regression of the dermatosis.

After 6 months, the patient was in complete remission for the rectal adenocarcinoma. There was a post-inflammatory pigmentation on the skin, but no sign of evolving Grover’s disease. The histopathological exam did not find any dyskeratosis.

The cutaneous lesions relapsed at the same time as the cancer in September 2006.

After an improvement in his condition, the patient finally received a second surgery in January 2007, but there was a tumour break with a high risk of contamination of the pelvis. A multidisciplinary staff decided against any complementary treatment. Nevertheless, the cutaneous lesions improved again. In June 2008, he was diagnosed with a relapse of cutaneous lesions on the upper part of the back. Their whorl-like appearance was compatible with Blaschko lines; the histopathological examination showed dyskeratosis and acantholysis. As no complementary treatment was given and the patient had no pain or digestive symptoms, further examinations have not been conducted. However, we suspect that this recurrence of the lesions may indicate a relapse of the neoplasia.

To our knowledge, this is the first case report of a paraneoplastic transient acantholytic dermatosis along Blaschko lines. The main differential diagnosis could be linear Darier’s disease. The histopathological data are not specific enough to differentiate Darier’s disease from Grover’s disease with any certitude [1]. Happle [3] and Fantini [4] also discussed this differential diagnosis. Although the diseases share associated factors like heat, sun exposure and sweating, Darier’s disease is hereditary [2], usually not pruritic, is localised on seborrheic zones and begins in childhood or adolescence [1]. Considering the absence of a personal or family history, the chronological evolution, the absence of a clear genetic hereditary disease through a paraneoplastic mechanism and the topography of the lesions only on the back, Darier’s disease seemed unlikely.

This particular disposition may be due to the post-zygotic mutations of genes coding for desmosomal proteins, resulting in a genetic mosaicism. These abnormalities may become manifest by the cellular stress caused by tumoral mediators or by a cross-immunisation against both the antigens from cancer cells and the antigens from epidermal cells in some Blaschko lines. Grover’s disease can be linked to many different hemopathies or solid tumours [5], but the mechanism of these associations remains unclear. In our case, the paraneoplastic characteristic was retained because of the chronological evolution of the dermatosis: it appeared at the diagnosis of the cancer, improved after treatment of the cancer and finally relapsed parallel to it.

Acknowledgements

References

2 Powell J, Sakuntabhai A, James M, et al. Grover’s disease, despite histological similarities to Darier’s disease, does not share an abnomality in the ATP2A2 gene. Br J Dermatol 2000; 142: 658.

3 Happle R. Linear Darier or Grover’s disease? J Am Acad Dermatol 2003; 49: 1200-1.

4 Fantini F, Kovacs E, Scarabello A. Unilateral transient acantholytic dermatosis (Grover’s disease) along Blaschko lines J. Am Acad Dermatol 2002; 47: 319-20.

5 Schneider I, Mojzes J. La maladie de Grover : une maladie faiblement corrélée à des tumeurs malignes viscérales. Ann Dermatol Venereol 1993; 120: 65-71.