Ichthyosiform sarcoidosis revisited.

Cutaneous manifestations of sarcoidosis are present in approximately one-third of the cases. Ichthyosiform lesion is one of the extremely rare cutaneous manifestations of sarcoidosis. It is a uncommon, but specific cutaneous manifestation of sarcoidosis that may precede or appear simultaneously with the diagnosis of systemic sarcoidosis. Approximately 20 cases of ichthyosiform sarcoidosis have been reported in the PubMed database. We report here a case of sarcoidosis with ichthyosiform skin lesions along with central nervous system (CNS) and pulmonary involvement for its rarity and interesting clinical presentation.

Ichthyosiform mycosis fungoides with alopecia and atypical membranous nephropathy.

We describe here a rare case of variant of mycosis fungoides (MF): ichthyosiform MF with alopecia and atypical membranous nephropathy. The diagnosis was made based on the following findings: generalized ichthyosis-like eruption, alopecia, enlarged superficial lymph nodes, proteinuria, and hematuria, the histological features of the skin biopsy from both ichthyotic and alopecic lesions with immunohistochemical staining, and the renal biopsy examination with immunofluorescence. The histological examination of ichthyotic and alopecic lesions displayed a predominant infiltration of atypical lymphocytes in the upper dermis with the characteristics of epidermotropism and folliculotropism. Immunohistochemical studies demonstrated that most infiltrated atypical lymphocytes were CD3, CD4, and CD45RO positive, whereas negative for CD5, CD7, CD20, CD30, and CD56. A renal biopsy examination revealed atypical membranous nephropathy with deposition of immunoglobulin G (IgG), IgM, IgA, C1q, and C3. In this case atypical membranous nephropathy was involved, which is very uncommon and has never been presented in the literature to date. Although ichthyosiform MF usually features a relatively favorable course, diffuse alopecia and the renal involvement in this case might indicate aggressive disease and poor prognosis.

Ichthyosis follicularis with alopecia and photophobia (IFAP) syndrome.

A 12-year-old boy born of a nonconsanguineous marriage presented with dry rough skin and photophobia since birth. His growth and developmental milestones were normal and there was no history of any neurological problem, hearing deficit or scarring around the hair follicles. Cutaneous examination revealed diffuse thinning of scalp hair with loss of eyebrows and eyelashes and a sandpapery texture of the skin all over the body, suggestive of ichthyosis follicularis with alopecia and photophobia syndrome.

Acquired ichthyosis: a marker for internal disease.

When ichthyosis occurs in adulthood, it may signify internal disease, including sarcoidosis and malignancy,--especially Hodgkin's lymphoma. Other systemic diseases associated with acquired ichthyosis are leprosy, hypothyroidism, chronic renal failure and nutritional deficiencies.