Mal de Meleda is a rare autosomal recessive palmoplantar keratoderma (PPK) disease with an estimated prevalence of 1:100,000. Clinically, the onset of the disease is typically soon after birth and features a transgrediens (plantar surface progressing to dorsal surface) and progrediens (worsening with age) pattern of hyperkeratosis of the palms and soles. The disease can feature other potentially disfiguring effects on the hands and feet that can severely impact function. Histologically, the lesions show hyperkeratosis and acanthosis without epidermolysis in the epidermis, accompanied by perivascular lymphocytic infiltrate in the dermis. Secreted LY6/urokinase-type plasminogen activator receptor (uPAR)-related protein-1 (SLURP-1) genetic mutations are implicated in Mal de Meleda. SLURP-1 is involved in mediation of inflammation as well as keratinocyte apoptosis regulation. Because the disease is so rare, there are no set guidelines for management, but the accepted approach tends to include oral acitretin plus topical keratolytic therapy. Genetic counseling should also be offered. This focused review highlights the clinical and histological features, differential diagnoses, genetic background, and the current thoughts on management of Mal de Meleda.
The majority of penile carcinoma is squamous cell carcinoma. Although uncommon in
the United States, it represents a larger proportion of cancers in the
underdeveloped world. Invasive squamous cell carcinoma may arise from precursor
lesions or de novo , and has been associated with lack of
circumcision and HPV infection. Early diagnosis is imperative as lymphatic
spread is associated with a poor prognosis. Radical surgical treatment is no
longer the mainstay, and penile sparing treatments now are often used, including
Mohs micrographic surgery. Therapeutic decisions should be made with regard to
the size and location of the tumor, as well as the functional desires of the
patient. It is critical for the dermatologist to be familiar with the
evaluation, grading/staging, and treatment advances of penile squamous cell
carcinoma. Herein, we present a review of the literature regarding penile
squamous cell carcinoma, as well as a case report of invasive squamous cell
carcinoma treated with Mohs micrographic surgery.
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