Acrokeratosis verruciformis of Hopf - Case report

Abstract

A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). After three days of prednisone treatment, the skin eruption resolved. RND is a rare cutaneous manifestation of seropositive RA, characterized by asymptomatic, symmetrical erythematous plaques with a pseudo-vesicular appearance. Histology characteristically reveals a dense, neutrophilic infiltrate with leucocitoclasis but without other signs of vasculitis. Lesions may resolve spontaneously or with RA treatment. This case illustrates an uncommon skin manifestation of active rheumatoid arthritis.

Mixed Variant of Acrokeratosis verruciformis of Hopf: A rare entity

“…AKV was first described by Hopf in 1931. 1 The probable etiology is a mutation in the ATP2A2 gene located on chromosome 12q24, a heterozygous pro602Leu mutation, the same gene affected in Darier disease (DD). 4 This mutation has an autosomal dominant pattern of inheritance but incomplete penetrance so family history may not be always present.…”

“…4 This mutation has an autosomal dominant pattern of inheritance but incomplete penetrance so family history may not be always present. 1 Since this is a dominant mutation, the disease follows a chronic course without remission. 1 The lesions can rarely undergo malignant transformation to squamous cell carcinoma.…”

“…1 Since this is a dominant mutation, the disease follows a chronic course without remission. 1 The lesions can rarely undergo malignant transformation to squamous cell carcinoma. 1 Clinically, AKV often occurs in childhood as multiple reddish-brown or skin colored, flat-topped papules mainly on the dorsum of the hands and feet and may involve knees, elbows, forearms or lower legs.…”

“…It mostly presents in early childhood, but onset may be delayed as late as the fifth decade. 1 It affects both the sexes with male to female ratio of 5:1.3. 2 Superficial ablation is the treatment of choice along with other treatment modalities including retinoic acid, cryotherapy and CO 2 laser.…”

Usage of unsaturated esters of retinol to defeat Hopf’s acrokeratosis verruciformis in hands and feet of a youg man

“…Acrokeratosis verruciformis is a disorder of keratinization characterized by multiple flat-topped, skin-colored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet. Hopf first suggested the name acrokeratosis verruciformis in 1931 [1].…”