
Intense hyperkeratosis producing a yellowish color interwoven with an erythematous border in the palmar region of a patient.
Here we describe the cases of two patients from the same family with typical clinical pictures and a history of consanguinity between the parents. The cases illustrate a rare genodermatosis that should be part of the differential diagnosis of palmoplantar keratodermas.
Case Reports

She reported that her parents had had 6 children, 3 of whom were healthy and 3 of whom suffered from the disease (her, the patient in case 2 and another sibling, who had died of another cause). Dermatological examination revealed intense transgressive whitish-yellow palmoplantar hyperkeratosis with maceration, and erythema extending to the dorsa of the feet and hands. Hyperkeratosis of the nails on all the fingers and toes was also present (Figures 1 and 2). Laboratory tests were normal. A clinical diagnosis of Mal de Meleda was made. However, the patient refused any specific treatment (systemic retinoids) and chose treatment with emollients.
Case 2: BDC, 72 years old, female, sister of patient 1, born and living in Mogi das Cruzes presented with a complaint of desquamation on her feet and hands since birth and the development of painful fissures. She also complained of excessive sweating and lesions with a fetid odor but denied any lesions on other parts of her body.


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