Hereditary Palmoplantar (Epidermolytic) Keratoderma: Illustration Through a Familial Report

Virendra N. Sehgal, MD; Kabir Sardana, MD; Sonal Sharma, MD; Dharmendra Raut, MBBS

Disclosures

Skinmed. 2004;3(6) 

In This Article

An Illustration

A 21-year-old Muslim, the product of a consanguineous marriage (paternal and maternal grandmothers were sisters) developed progressive papular and erythematosquamous rash over his abdomen on precisely his third day of life. A month after his birth, the patient's mother observed fine cracks and scales over the boy's palms and soles that had a yellowish hue. The patient was also irritable, possibly due to discomfort from the palms and soles. The rashes over the hands and feet considerably increased to alter the texture of the skin and to occupy their current position. Itching and/or pain were its preeminent features. Profuse sweating of the affected part was also an attribute. The patient preferred the winter season and cherished wandering in the cold. His only elder brother had similar, much more severe complaints and had succumbed to the disease at age four years. The boy's parents had identical palms and soles, the details of which are outlined in sequence in Figure 1.

A family pedigree of palmoplantar keratoderma.

Immense thickening of the skin might virtually mask the creases, a "hallmark" in the examination of the soles of the foot. The thickening was well circumscribed and its margin was surrounded by a conspicuous erythematous (halo) border. It was waxy yellow in color. The skin was taut and had explicit fissures/cracks (Figure 2). The latter were dirty yellow and fairly deep. Similar, less-pronounced morphologic changes were elicited on the palms (Figure 3). The symmetry of the lesions was cardinal. The changes in the nails were not that pronounced. The rest of the skin surface was somewhat dry and scaly.

Soles affected by palmoplantar keratoderma showing immense and well circumscribed, waxy yellow thickening of the skin surrounded by prominent erythematous border and fissures/cracks.

Palms affected by palmoplantar keratoderma showing fissure/cracks with scaling.

Investigations, including total and differential leukocyte counts, erythrocyte sedimentation rate, random blood sugar, blood urea, serum electrolytes, and serum calcium were within norms. Hematoxylin-eosin stained sections prepared from fragile biopsy from the affected sole depicted conspicuous changes confined primarily to the epidermis. Orthokeratotic hyperkeratosis was strikingly exquisite. Nonetheless, a variable degree of hypergranulosis and acanthosis was noticeable (Figure 4). Periodic acid-Schiff reaction was unable to identify mycelia/spores in the tissue sections.

Hematoxylineosin stained section of palmoplantar keratoderma tissue depicting variable degree of hypergranulosis and acanthosis.

PPK, an interesting hereditary entity, has been studied from the day its clinical expression was outlined. The condition has since been reported either sporadically or in a family.[10,40,49,50,51,52] The recount of such cases is not only a reminder of the condition but also a way to enrich the knowledge base. In addition to the classic clinical expression, any deviation in its morphologic characteristics should be carefully recorded.

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