Polydactyly with Ainhum in all the Extra Digits, Hyperkeratosis Palmaris et Plantaris, and Idiopathic Eosinophilia

Pseudoainhum in a patient with tuberous sclerosis complex: a case report and review of the literature

“…2,29,42 This report describes a case of pseudoainhum presenting with properties characteristic of TSC. This association, which has not been previously Disorders of abnormal keratinization Psoriasis [15][16][17] Pityriasis rubra pilaris 18 Porokeratosis of Mibelli 19 Congenital linear porokeratosis 20 Focal acral hyperkeratosis and angiodysplasia 21 Lamellar ichthyosis [22][23][24] Keratosis palmoplantaris with polydactyly and eosinophilia 25 Keratoma hereditarium dissipatum palmare et plantare (Buschke-Fischer-Brauer) 26 Keratoderma hereditarium mutilans (Vohwinkel) [27][28][29][30][31][32] Progressive symmetric erythrokeratoderma (PSEK) 4 Keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) 5 Keratosis palmoplantaris transgradiens of Siemens (mal de Meleda) 6,33 Clouston's syndrome 34 Ectodermal dysplasia syndrome (with cataracts, alopecia, sclerodactyly) 35 Papillon-Lefevre syndrome 36 Loricrin keratoderma 32 Metabolic and neurologic diseases Isolated polyneuropathy 37 Gout disease 38 Alcohol-toxic polyneuropathy 9 Erythropoietic protoporphyria 11 Plica neuropathica, hair matting, schizophrenia 10 Reynolds syndrome and breast cancer 14 Connective tissue diseases Rheumatoid arthritis 39 Discoid lupus erythematosus 40 Localized scleroderma 41,42 Bullous disease Hereditary bullous acrokeratotic poikiloderma of Wea...…”

“…Additionally, pseudoainhum is reportedly associated with a large number of diseases (Table 1). Conditions associated with pseudoainhum can be classified as keratinization abnormalities, 4–6,15–36 metabolic and neurologic disorders, 9–11,14,37,38 connective tissue diseases, 39–42 bullous dermatosis, 43–45 infections, 34,46,47 and congenital abnormalities 48,49 …”

Papillon-Lefèvre syndrome associated with pseudoainhum

“…These include infections (particularly leprosy), trauma, and cold injury. 9 Several disorders of keratinization such as Vohwinkel syndrome, 10 mal de Meleda syndrome, 11 KLICK syndrome, 12 Brauer-Buschke-Fischer syndrome, 13 Clouston's syndrome, 14 keratosis palmoplantaris with eosinophillia and polydactly, 15 focal acral hyperkeratosis and angiodysplasia, 16 pityriasis rubra pilaris, 17 porokeratosis of Mibelli, 18 psoriasis, 19 lamellar ichthyosis, 20 and progressive symmetric erythrokeratoderma. 21 Some connective tissue diseases like discoid lupus erythematosus, 22 linear scleroderma, 23 morphoea, 24 and rheumatoid arthritis.…”