Multifaceted array-based keloidal gene expression profiling reveals specific MDFI upregulation in keloid lesions.

BACKGROUND: Keloid lesions are characterized by mesenchymal cell proliferation and excessive extracellular matrix deposition. Previous microarray analyses have been performed to investigate the mechanism of keloid development. However, the molecular pathology that contributes to keloid development remains obscure. AIM: To explore the underlying essential molecules of keloids using microarrays. METHODS: We performed microarray analyses of keloid and nonlesional skin tissues both in vivo and in vitro. Gene expression levels were compared between tissues and cells. Quantitative reverse transcription (qRT)-PCR and immunohistochemical staining were used to determine the expression levels of molecules of interest in keloid tissues. RESULTS: Several common molecules were upregulated in both keloid tissues and keloid-lesional fibroblasts. PTPRD and NTM were upregulated both in vivo and in vitro. The genes MDFI and ITGA4 were located at the centre of the gene coexpression network analysis using keloid tissues. qRT-PCR revealed significant expression levels of PTPRD and MDFI in keloid tissues. Immunopathological staining revealed that MDFI-positive cells, which have fibroblast characteristics, were located in the keloid-associated lymphoid tissue (KALT) portion of the keloid tissue. CONCLUSION: Our gene expression profiles of keloids could distinguish the difference between lesional tissue and cultured lesional fibroblasts, and MDFI was found to be commonly expressed in both tissues and cells. Thus, MDFI-positive cells, which were located in the KALT, may play an important role in keloid pathogenesis and thus might be useful for in vitro keloid studies.

Granulocyte and monocyte apheresis can control juvenile generalized pustular psoriasis with mutation of IL36RN.

Patients with deficiency of interleukin-36 receptor antagonist (DITRA), due to mutation of IL36RN, exhibit psoriatic phenotypes, typically generalized pustular psoriasis (GPP). We report a paediatric patient with DITRA, whose cutaneous lesions varied from psoriasis vulgaris in infancy to annular pustular psoriasis with acute exacerbation to GPP at 13 years of age. Conventional systemic treatments for GPP, which include oral retinoids, ciclosporin and methotrexate, are controversial in paediatric cases, because of their adverse effects and uncertain long-term consequences. Granulocyte monocyte apheresis, a process associated with few adverse events, promptly controlled the GPP of our paediatric patient, and has potential as a suitable alternative treatment for paediatric patients with DITRA.

Glycosaminoglycan and versican deposits in taxane-induced sclerosis.

Docetaxel and paclitaxel are widely used in the treatment of various malignant neoplasms. Taxane-induced sclerosis is dose-dependent and usually not generalized. Little information on the pathogenesis of scleroderma is currently available. Here, we report a case of generalized scleroderma and a case of early-stage oedematous sclerosis, both of which presented with intense versican deposits after administration of taxane for angiosarcoma.

Malignant melanoma derived from cerebriform intradermal naevus.

We report a case of malignant melanoma (MM) derived from cerebriform intradermal naevus (CIN) in a 66-year-old Japanese man. The patient had cutis verticis gyrata (CVG) on the posterior area of the scalp at birth. He noticed a dome-shaped nodule at the centre of the CVG at 66 years of age. Histopathological examination found a nodule of MM arising within an extensive area of intradermal naevus. There was no metastasis to lymph nodes or other organs. To our knowledge, only two cases of CIN in which MM had later developed have been reported. We estimated that the incidence of melanoma from CIN including our case is 4.5% (3 of 67 reported cases), which seems to be comparable to the frequency of malignant alteration of giant pigmented naevi. This suggests that pathological examination is recommended for CVG, and once pathological diagnosis of CIN is confirmed, long clinical follow-ups are necessary for detecting development of MM.

Occupation-related pigmented macules on the sole with parallel-ridge pattern on dermatoscopy.

27-year-old man presented with pigmented macules on the right sole, which showed a parallel-ridge pattern on dermatoscopy. His work for a chemical company involved handling para-phenylenediamine. Histological examination of a biopsy from a lesion did not find any proliferation of atypical melanocytes. Shaving the cornified layer of the lesions with a surgical knife resulted in the disappearance of macules. We speculate that para-phenylenediamine on the sole of the patient's work boot might have become blotted to the cornified layer of the cutis. This report adds a new occupation-related differential diagnosis for skin diseases showing a parallel-ridge pattern on dermatoscopy.

Two cases of dyshidrosiform pemphigoid with different presentations.

We report two cases of dyshidrosiform pemphigoid (DP) with different presentations. One patient was a 65-year-old Japanese man, who had been diagnosed with dyshidrosis and had been treated before visiting our hospital. When we stopped all treatments, the vesicles increased and spread to the trunk and limbs. We made a diagnosis of vesicular pemphigoid (VP) that was concomitant with or transformed from DP. Using Western blotting, the sera reacted with antigens with molecular weights of 60 and 180 kDa. The 60-kDa antigen has not been found previously in the sera of patients with VP. The other patient was a 94-year-old Japanese woman, who presented with redness and swelling with bullae on the palmoplantar areas. Five days later, areas of oedematous erythema, as seen in prototypical bullous pemphigoid (BP), developed on the limbs. Study of the distribution of the BP antigen may elucidate the mechanisms involved in localized forms of BP such as DP.

Extensive skin necrosis of the arm in a patient with complex regional pain syndrome.

We report a 36-year-old woman with complex regional pain syndrome (CRPS) type 1 presenting with extensive skin necrosis of the left arm. The patient cooled her arm with ice packs to ease severe pain due to CRPS, in spite of repeated cautions against frostbite injury. The regions of skin necrosis corresponded with the sites where she had applied ice packs. We considered that the severe skin necrosis in our case was due to a self-induced frostbite injury.