Spiny keratoderma: Report of three cases

Abstract

Background Spiny keratoderma shows “music box spine” keratotic papules limited to the palms and soles and these lesions do not cause any trouble besides the cosmetic impairment and the sensation of roughness of hands and soles. Aim To present cases and review the literature on spiny keratoderma. Patients/Methods, Results Three cases of spiny keratoderma are presented. The first case is a 52‐year‐old man with protein S deficiency presented with multiple spiny keratotic papules on palms and soles for more than 30 years, referring to remove them with a razor blade. The second case is an 84‐year‐old man who was hospitalized due to a rectal adenocarcinoma diagnosed 2 months before and presented multiples papules on palms and soles that were present for more than 50 years, with worsening after being bedridden. The third case is a 59‐year‐old woman who had since birth together with relatives the same type of spiny keratotic papules on palms and soles. Conclusion The authors discuss the epidemiologic data and differential diagnosis of spiny keratoderma, as well as the related literature. Associations with systemic disease or malignancy occur in some acquired cases. It is important to distinguish from other palmoplantar keratodermas as some of that can be linked to cutaneous and internal malignancies. To date, no cases of spiny keratoderma in patients with protein S deficiency and only one case of association with rectal carcinoma have been reported.

Spiny keratoderma: A review of case reports and histopathological descriptions

“…Of the 58 articles, 10 were case series, resulting in 84 SpK cases for analysis (Table 1). The individual cases were published in the USA (32), 1,3,8,9,12,16,18,20,24,[30][31][32][33][34]37,45,47,49,53 Spain (12), 14,22,23,26,28,29,36,39,50,54,58 Saudi Arabia (7), 10 Italy (6), 2,13,19,46,55 Japan (6), 11,21,25,40,52 Brazil (4), 5,42 United Kingdom (3), 41,57 Australia…”

“…Of those, 41 cases had a reported history of systemic disease-13 with type II diabetes mellitus, 9,25,33,34,38,40,41,43,44,46,56,58 10 with hyperlipidemia, 18,25,39,43,53,55,56 7 with pulmonary disease including COPD and asthma, 7,14,18,21,44,47,52 and 3 with chronic kidney disease. 38,44,56 Other systemic conditions associated with SpK included pulmonary tuberculosis, 14 SARS-CoV-2 infection, 54 protein S deficiency, 5 Darier disease, 17 type IV hyperlipoproteinemia, 19 autosomal dominant polycystic kidney disease, 16 and sarcoidosis. 7 The average reported age was 47.9 in hereditary cases and 63.6 in sporadic cases.…”

“…Of the 58 articles, 10 were case series, resulting in 84 SpK cases for analysis (Table 1). The individual cases were published in the USA (32), 1,3,8,9,12,16,18,20,24,30–34,37,45,47,49,53 Spain (12), 14,22,23,26,28,29,36,39,50,54,58 Saudi Arabia (7), 10 Italy (6), 2,13,19,46,55 Japan (6), 11,21,25,40,52 Brazil (4), 5,42 United Kingdom (3), 41,57 Australia (3), 43,56 India (2), 48,51 Germany (2), 35,44 Israel (1), 27 Turkey (1), 4 France (1), 17 Scotland (1), 38 Austria (1), 15 South Africa (1), 7 and Canada (1) 6 . Of the 84 total cases, 33 (39.3%) were female and 51 (60.7%) were male.…”

“…The long list of various terms used to describe this rare condition creates confusion in histopathological correlation and diagnosis. SpK is typically either inherited in an autosomal dominant pattern or presents later in life in association with certain systemic diseases and malignancies 5 . The true incidence of SpK may be underreported as a result of the benign nature of the disease, especially in hereditary cases where patients may have had symptoms their whole lives and have family members with similar manifestations.…”

“…Disruption of cholesterol synthesis has been proposed to lead to hyperproliferation, supported by animal studies showing that topical application of lovastatin causes epidermal hyperplasia 18. The characteristic histopathological features of SpK include discrete columns of parakeratotic hyperkeratosis arising from depressions in the epidermis overlying focal hypogranulosis, resembling cornoid lamella of porokeratosis (Figure5) 5. There are several variants of porokeratosis, including linear porokeratosis, porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, punctate porokeratosis, and porokeratosis palmaris et plantaris disseminata (PPPD).…”