Mal de Meleda: A Focused Review

Caroline Perez; Amor Khachemoune

Disclosures

Am J Clin Dermatol. 2016;17(1):63-70. 

In This Article

Clinical Patterns

Diagnostic criteria for the disease were first presented in 1969.[20] Generally, clinical signs include bilateral PPK that is diffuse and in a cuff-like (stocking and glove) pattern with sharp margins and a yellow tone. The symptoms begin soon after birth as a palmoplantar erythema and progress to the characteristic thickened, exfoliative hyperkeratosis. Ultimately, hyperkeratosis can present on the dorsal surface of the hands and feet as well (the transgrediens nature of the disease), beginning with the dorsum of the fingers in childhood and progressing to the dorsum of hands and feet in adulthood (Fig. 1). The pattern of becoming more severe with age (with greater body surface area affected) reflects the progrediens nature of the disease.[21,22] Hyperkeratosis can be complicated by hyperhidrosis, with subsequent microbial infection leading to malodorous and painful lesions.[23]

Figure 1.

Clinical features of Mal de Meleda: a diffuse, bilateral thickened hyperkeratoses with peripheral erythema on the palmar surface; b exfoliative hyperkeratoses showing sharp demarcation of lesions on the palm; and c transgrediens progression of hyperkeratoses to the dorsal surface

It has been reported that nail anomalies are the most common associated feature and include subungal hyperkeratosis, Beau's lines, onchyolysis, hyperconvexity, and koilonychias.[22,23] Patients may also have anatomical disturbances of the digits, including reports of fifth-finger dysplasia, tapering of the digits, contracture, knuckle pads, and pseudoainhum.[22,24,25] Psoriasiform lesions may be present on the knees and elbows.[5] There have also been documented oral manifestations of Mal de Meleda, with perioral erythema and lower-lip angular cheilitis as well as high, arched palate.[5,24]

In the later stages of the disease, there are rare reports of malignant melanoma arising within the areas of hyperkeratosis in Mal de Meleda.[26–28]

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