Raynaud Phenomenon and Behçet Disease: Diagnosis With Technetium Tc 99m Methylene Diphosphonate Bone Scan and Treatment With Continuous Sympathetic Block

Abstract

Although Darier disease (DD) may have papillomatous appearance, to our knowledge, no human papillomavirus (HPV) has been reported to be involved. Arotinoid ethylester (p-[(E)-2-(5,6,7,8-tetrahydro-5,5,8,8-tetramethyl-2-naphthyl) propenyl]-benzoic acid ethyl ester) is one of the third-generation, or polyaromatic, retinoids. We report here the use of arotinoid ethylester in 2 cases of DD and the detection of HPV DNA in biopsy specimens of these cases. Two men aged 60 years (case 1) and 41 years (case 2) presented with DD for 20 and 15 years, respectively. Both had crusted papules, plaques, and verrucous lesions nearly all over the body, with papillomatous vegetation pronounced in the flexures (Figure 1). Histologically, suprabasal lacunae and dyskeratosis with corps ronds and grains were consistent with the diagnosis of DD. Biopsy specimens from the vegetative lesions of the 2 cases showed papillomatous proliferation and vacuolated keratinocytes in the upper stratum malpighii, in addition to acantholysis and dyskeratosis (Figure 2). We detected DNA sequences of (1) HPV-5, HPV-36, and HPV-38 (case 1) and HPV-5 and HPV-36 (case 2) in the nested polymerase chain reaction products from the biopsy specimen of the papules and (2) HPV-5 and HPV-8 from the vegetative lesions of case 1, all using consensus primers CP65/70 and CP66/69, which are designed for epidermodysplasia verruciformis-associated HPV.¹ In another polymerase chain reaction assay using the primer MY09/11 (designed for mucous-type HPV), all the specimens were negative.²