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A case of hyperpigmentation distributed in streaks and whorls was recently delineated and separated from a confusing number of pigmentary disorders. A 13-year-old boy had hyperpigmented, reticulate spots on his trunk and neck in a linear and whorled distribution following the lines of Blaschko. Onset was at age 1 year. Histologically, the spots showed increased melanin in the basal layer of the epidermis, but no increase in melanocytes, incontinence of pigment, or melanophages were seen in the dermis. The karyotype was 46,XY and there was no evidence of chromosomal mosaicism or chimerism. This entity represents a disorder separated recently from other pigmentary alterations that follow Blaschko lines, such as Bloch-Sulzberger incontinentia pigmenti, hypomelanosis of Ito, early systematized epidermal nevus, and chimerism.

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A 42-year-old woman presented with a well-defined pruritic erythematous scaly plaque on the sole of each foot. The lesions, first noted about 15 years previously, were located in the medial plantar region and extended laterally. Mycological study revealed infection by Scopulariopsis brevicaulis. Treatment with oral itraconazole led to temporary improvement, but the symptoms returned after treatment had been stopped (presumably due to re-infection from ungual foci). Similar results were subsequently obtained with oral terbinafine. S. brevicaulis is an aetiologic agent of onychomycosis, panophthalmia following a penetrating eye injury and generalized infections in immunocompromised patients, but it is not considered as habitual fungal pathogen of the skin. Cutaneous lesions caused by S. brevicaulis are very rare. Our case was resistant to terbinafine and itracomazole.

Generalized (diffuse) plane xanthoma (GPX) is a normolipaemic xanthomatosis that is very frequently associated with a haematological disorder, and especially with monoclonal gammapathies, which it may precede by several months or years. Here, we report a case of GPX associated with idiopathic Bence-Jones proteinuria, a very rare type of monoclonal gammopathy of undetermined significance. To our knowledge, this association has not been previously described.

A typical clinical and histologic case of so-called ‘pagetoid reticulosis’ or Woringer-Kolopp disease is reported. This is a chronic cutaneous condition usually with a single focus or at most with a few patches confined to a circumscribed region. A disseminated form has been described as Dupont-Vandaele type, but its relationship to the classic Woringer-Kolopp disease is uncertain. The nosological location of pagetoid reticulosis has not been established, but there are reasons to regard it as a particular and well-defined entity. In relation to the nature of this condition and to the origin of the ‘pagetoid cells’, it is tempting to consider the possibility that Woringer-Kolopp disease might be a lymphoproliferative epidermotropic disorder, classifiable as a benign form of ‘cutaneous T-cell lymphoma’, but the early histologic changes are rather indicative of an epidermal origin.

We report the case of a 47-year-old male with Kartagener’s syndrome (KS; situs inversus, bronchitis and sinusitis) who showed three types of cutaneous lesions: recurrent outbreaks of nummular eczema, recurrent deep folliculitis and two episodes of pyoderma gangraenosum. The patient had also IgA gammopathy of undetermined significance. This is the second case of KS associated with cutaneous lesions published so far and suggests that primary ciliary dyskinesia syndromes may have skin symptoms.