Robert Auerbach

Methotrexate in psoriasis: Revised guidelines

The decision to administer methotrexate for the t reatment of psoriasis should be individualized. Each patient should be evaluated with reference to disease severity, amount of discomfort, degree of incapacity, and general medical and psychologic condition. Methotrexate is indicated in the symptomatic control of recalcitrant psoriasis not responsive to topical therapy. The diagnosis of psoriasis and the need for methotrexate therapy should be established by dermatologic consultation. In general, these patients should have severe psoriasis that m a y be life-ruining physically, emotionally, or economically. The psoriasis should be so severe that it cannot be adequately controlled by standard topical antipsoriasis therapy. Some examples of candidates for methotrexate therapy are patients with the following:

Telomere phenotypes in females with heterozygous mutations in the dyskeratosis congenita 1 (DKC1) gene.

Dyskeratosis congenita (DC) is a telomere‐mediated syndrome defined by mucocutaneous features. The X‐linked mode of inheritance accounts for half the cases, and is thought to predominantly manifest in childhood as bone marrow failure. We identified two male probands who presented in the fifth decade with idiopathic pulmonary fibrosis and cancer. Their pedigrees displayed consecutively affected generations. Five of six females (83%) manifested mucocutaneous features of DC, and two had wound‐healing complications. No mutations in autosomal dominant telomere genes were present, but exome sequencing revealed novel variants in the X‐chromosome DKC1 gene that predicted missense mutations in conserved residues, p.Thr49Ser and p.Pro409Arg. Variants segregated with the telomere phenotype, and affected females were heterozygotes, showing skewed X‐inactivation. Telomerase RNA levels were compromised in cells from DKC1 mutation carriers, consistent with their pathogenic role. These findings indicate that females with heterozygous DKC1 mutations may be at increased risk for developing penetrant telomere phenotypes that, at times, may be associated with clinical morbidity.

Malignant melanoma in situ in two patients treated with psoralens and ultraviolet A.

Two patients are reported who were treated with 8-methoxypsoralen and ultraviolet A (PUVA) for psoriasis and developed cutaneous lesions of malignant melanoma in situ (atypical melanocytic hyperplasia). One patient received 324.5 joules/cm2 of UVA. Seven months after discontinuing therapy, he developed a superficial spreading melanoma in situ in association with an intradermal nevus on the left posterior thoracic area. The second patient received 2,802 joules/cm of UVA. While on PUVA therapy she developed an in situ lentigo melanoma on her lower lip. To our knowledge only one other psoriatic patient and one patient with vitiligo have developed malignant melanomas after PUVA therapy, so that an increased incidence of malignant melanomas after PUVA therapy, so that an increased incidence of malignant melanoma following PUVA is not documented.

Basal Cell Epithelioma of the Sole

In reporting the sixth case of basal cell epithelioma of the sole, it is suggested that basal cell epitheliomas derive from `pluripotential epithelial cells which may differentiate into any of the epithelial structures of the skin. The current consensus that basal cell epitheliomas derive from cells analagous only to primary epithelial germ cells is considered to be too restrictive.

Acquired reactive perforating collagenosis

Background: Reactive perforating collagenosis (RPC) is characterized by transepithelial elimination of altered collagen. Two types have been recognized: the childhood form and the adult form. Objective: Our purpose was to review the associated disorders, evaluate the possible causes, and set criteria for the diagnosis of the disease. Methods: The clinical and pathologic findings of six patients with the adult form of RPC are reviewed. The literature on this subject is compared with our findings. Results: Pruritus was reported in all cases. Treatment of pruritus cleared the lesions in many patients. This is the first report of an association between RPC and hyperparathyroidism, hypothyroidism, liver disorders, and neurodermatitis. Conclusion: Various disorders can be associated with the adult form of RPC. Pruritus is the common factor among all types. Control of itching might be helpful for clearing the lesions. We propose the following diagnostic criteria for acquired RPC: (1) histopathologic findings of elimination of necrotic basophilic collagen tissue into a cup-shaped epidermal depression, (2) clinical presentation of umbilicated papules or nodules with a central adherent keratotic plug, and (3) onset of skin lesions after the age of 18 years.

Dermatitis-eczema disorders.

The term “eczema” is commonly used to describe such a variety of skin eruptions that it is often misused and misinterpreted. In the United States, the terms “dermatitis” and “eczema” are used synonymously. Here, the dermatitis-eczema disorders are defined according to clinical and histologic features and categorized on a basis of definite etiology and clinical reaction patterns. General rules and specific guidelines for management are given.