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IgG4-related disease is a recently defined emerging entity. Many different organs may be affected by this disease: pancreas, salivary and lacrimal glands, liver, peritoneum and lung. Also the skin may be affected, as secondary localization, while as primary cutaneous localization it has been rarely described. A male patient presented at our Institute with a two-year history of sclerosing erythematous nodules of the scalp. Histological examination showed a T-lymphocyte (CD3+) infiltrate with interspersed plasmacytoid cells and the interposition of a fibrosclerotic tissue. We found numerous IgG4+ cells at the periphery of the nodular structures, while the serum levels of IgG4 and the remaining blood chemistry analysis were normal. Only a few cases of primitive cutaneous pseudo-lymphomatous IgG4-related disease have been described in the literature. Our case showed the same clinical and histologic features of those previously described; the etiology of IgG4-related diseases remains to be elucidated.

The Birt Hogg Dubé syndrome (BHD) is a rare autosomal dominant genodermatosis predisposing patients to developing fibrofolliculoma, trichodiscoma and acrochordon. The syndrome is caused by germline mutations in the folliculin (FLCN) gene, encoding the folliculin tumor-suppressor protein. Numerous mutations have been described in the FLCN gene, the most frequent occurring within a C8 tract of exon 11. This hypermutability is probably due to a slippage in DNA polymerase during replication, resulting in gains/losses of repeat units, causing cancer predisposition. The main phenotypic manifestations related to this disease are lung cysts, leading to pneumothorax, and a 7-fold increased risk for renal neoplasia, although other neoplastic manifestations have been described in BHD-affected individuals. Of particular interest is the often reported genotype/phenotype correlation between FLCN mutations and risk of colon or breast cancer. This paper describes our current knowledge on the association between BHD and cancer predisposition and briefly summarizes our experience in this field.

We present a case series of 63 patients diagnosed with juxta-clavicular beaded lines. This condition is more frequent in dark-skinned people and corresponds to an anatomical variant of simple sebaceous hyperplasia. In view of the strong reactivity of the melanocytes in dark-skinned people, and of the possible hypochromic results, no treatment is advised.

Milia en plaque (MEP) is an unusual and extremely rare clinical variant of milia, characterized by multiple milia-like lesions overlying an erythematous edematous plaque with histologic findings consistent with milia. MEP tends to affect the middle-aged patients and shows a predilection for women. Among children, this entity is rarely described and, to our knowledge, only four cases have been reported to date in the dermatologic literature. We add three new cases of children, one of whom had an unusual site of presentation. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented commenting on the etiology of Kyrle's disease.

We report an Italian prepubescent girl with the typical clinical and histologic features of prurigo pigmentosa associated with an atopic diathesis. The dermatitis disappeared after treatment with minocycline, leaving a brown, reticulated hyperpigmentation, with no recurrence. The association with an atopic diathesis could justify, in this instance, the major susceptibility to environmental factors claimed by some authors as triggering factors for prurigo pigmentosa. To our knowledge, this is only the second occurrence of this disease in a prepubescent patient reported in the literature. [ABSTRACT FROM AUTHOR]