Tiger-like mycosis fungoides: an unusual clinical presentation of a rare variant of mycosis fungoides.

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. Mycosis fungoides classically presents in the skin as patches, plaques, tumors, or erythroderma, progressing to involve the lymph nodes and peripheral blood. The many clinical variants, with different histologic patterns, and the subtle early clinical and histologic changes may delay early diagnosis and present a diagnostic challenge for clinicians. The greatest challenge in diagnosis is the pre-mycotic stage, which may closely resemble eczematous or psoriasiform dermatitis clinically and histologically. The persistence of lesions and inadequate response to treatment are the first warning signs. Later stages of MF have a poor prognosis with poor therapeutic response and fatal outcome. We describe a 72-year-old man, who presented with a two-year history of an unusual eruption, which started on the abdomen, around the waistline, and gradually spread to involve his back, trunk, and buttocks. Clinically, the skin eruption presented as tiger-like stripes. The diagnosis was confirmed after histopathologic examination. The patient was treated with NB-UVB phototherapy with marked improvement.

Exacerbation of X-linked ichthyosis phenotype in a female by inheritance of filaggrin and steroid sulfatase mutations.

BACKGROUND: X-linked ichthyosis (XLI) is a relatively common, recessive condition caused by mutations in the steroid sulfatase (STS) gene. Common loss-of-function mutations in the filaggrin gene (FLG) cause ichthyosis vulgaris and predispose individuals to atopic eczema. OBJECTIVE: To test the hypothesis that co-inheritance of FLG mutations can act as a genetic modifier in XLI. METHODS: An unusually severe XLI phenotype in addition to eczema and mild childhood asthma was investigated in a female Indian patient by fluorescent in situ hybridization (FISH) for the common STS gene deletion. Direct sequencing of the entire FLG gene was also performed. RESULTS: FISH analysis revealed that the proband was homozygous for the common STS genomic deletion mutation. Further investigation revealed a frame-shift mutation 3672del4 in the gene encoding filaggrin (FLG), leading to premature termination of profilaggrin translation. Interestingly, her father, who had a very typical mild presentation of XLI, did not carry this FLG mutation in addition to his STS deletion. Her mother was a heterozygous carrier of the FLG mutation and consistent with this, had mild symptoms of ichthyosis vulgaris; she was also a heterozygous carrier of the STS deletion. CONCLUSION: This is the second reported case of the modifying effects of FLG null alleles on XLI and strengthens the hypothesis that filaggrin defects can synergize with STS deficiency to exacerbate the ichthyosis phenotype.

[Current possibilities of using antimyocotic drugs in the treatment of various skin disorders]. / Aktualne mozliwosci stosowania leków przeciwgrzybiczych w niektórych chorobach skóry.

Current possibilities of using antimycotic drugs in the treatment of various skin disorders. The purpose of this article is to review the literature data on the therapeutic protocols and the results of using some antimycotics in different skin diseases. In addition to the antimycotic action, particular antifungal drugs such as itraconazole, ketoconazole and terbinafine exhibit anti-inflammatory activity by inhibiting the synthesis of 5-lipooxygenase metabolites. As these metabolites are involved in a number of inflammatory and immunoreactive processes the dual action of the drugs may be suitably exploited in the treatment of some skin diseases which are otherwise difficult to cure. Another rationale for the use of antimycotics in certain skin disorders is their action against Malassezia. It has been recently demonstrated that Malassezia, present as a commensal in the epidermis, may play an important role in inducing certain inflammatory processes by stimulating cytokine production by keratinocytes. The antimycotics proved to be useful in the therapy of the following skin conditions: seborrheic dermatitis, Malassezia folliculitis, perioral dermatitis and papulopustular rosacea, as well as adult atopic dermatitis. The use of antimycotic drugs in amicrobial palmoplantar pustulosis and sebopsoriasis remains controversial. These medications are also an alternative in the treatment of leishmaniosis.