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A 19-year-old female and her mother visited our department with a history of unusual reticular brown-black patches on their trunks and limbs since infancy. Besides the obviously fulsome hyperpigmentation, the daughter also showed other unusual clinical manifestations such as generalized hypohidrosis, especially on the hyperpigmented patches, cicatricial alopecia, a cataract on her right eye, abnormal teeth and a right dysplastic breast. The mother had a median diastema between her maxillary central incisors, hypoplasia of the enamel, hypohidrosis and hyperpigmented patches on her left thoracic region. Analysis of the NEMO (NF-κB essential modulator) gene in the patient and her mother revealed a deletion of exons 4–10. Their EDA and EDAR genes were normal.

Background Pigmented fungiform papillae of the tongue (PFPT) is a disorder in which the fungiform papillae of the tongue have abnormal coloration. However, Chinese-specific clinical data for PFPT are lacking. Objective To determine the prevalence and characteristics of PFPT among the Chinese population. Methods A survey was carried out using a clinical examination and a questionnaire on 14,346 first-time outpatients in our dermatology department, and 58 cases of PFPT were subsequently diagnosed. Results The prevalence of PFPT was 0.4% among dermatological outpatients. All patients had pin-sized, brownish fungiform papillae on the tip, lateral or dorsal parts of the tongue. Of the three subtypes, type I was the most common (87.93%). PFPT generally coexisted with Hori’s nevus (48.28%), melasma (20.69%), hysteromyoma (24.14%) and breast cystic hyperplasia (20.69%). Conclusion Our study confirms that PFPT is a relatively common disorder among Chinese outpatients, and it was presumed to be closely coupled with Hori’s nevus, melasma and other disorders.

We report a case of cutaneous silica granuloma with generalized involvement of distal lymph nodes; no similar case has been reported in the published work. The 45-year-old man was a stonemason who had been incidentally sprayed with rock dust from a saw 22 years ago. The subject presented with nodules and erythematous areas on his face and chin. In addition, there was swelling in the cervical and inguinal lymph nodes. An excised lymph node had normal architecture with numerous non-caseating epithelioid cell granuloma and silicotic nodules which contained scattered, multinucleated, giant cells. There were particles in the granuloma exhibiting birefringence under polarized light microscopy. Elemental X-ray spectra of these lesions showed the presence of silica in these granulomas.

We describe a modified method for establishing long-term pure cultures of amelanotic melanocytes (AMMC) derived from human hair follicles. Normal human corpse scalp (just after death, 1 h) was transected 1 mm below the epidermis, and hair follicles in the remaining dermis were isolated by a two-step enzyme treatment. Hair follicle cell suspensions were prepared by 0.50% trypsin treatment for 30 min and cultured in an optimized melanoblast proliferation nature mitogen medium. Cells attached to the substratum were mostly amelanotic melanocytic in character with small, bipolar shapes in the early stage; only a few keratinocytes and rare fibroblasts were observed. Keratinocytes were easily removed by differential trypsinization. After the third passage, the proliferating cells were all amelanotic melanocytes as confirmed by immunostaining with polyclonal antibodies to alphaPEP7h, which recognized the tyrosinase protein located on melanosomes and NKI/beteb, which is a pre-melanosomal antigen against synthetic peptides corresponding to the carboxyl termini of human melanosomal protein GP100. Cultured AMMC were highly positive to L-dopa reactivity after the addition of IBMX to the culture medium for 7 days. Many stage I and II melanosomes and occasional stage III melanosomes without stage IV melanosomes were found in the cytoplasm by transmission electron microscope. This modified technique is potentially more suitable for cultivating amelanotic melanocytes. The availability of pure cultures of hair-follicle amelanotic melanocytes will facilitate investigations of the roles of those cells in migration and differentiation during treatment of vitiligo.

Summary We describe a 22-year-old male with a pigmentary disorder that first appeared when he was 5 years of age. Examination revealed that these lesions were distributed in a segmental pattern on the left side of his trunk. Five shades of colors – white, tan, medium brown, dark brown, and black – were present. The patient told us there had been no preceding inflammation in the affected areas. A biopsy specimen from the inguinal region showed a complete absence of melanocytes, whereas most of the hyperpigmented areas were characterized by increased amounts of epidermal pigment and numbers of melanocytes. Based on the clinical appearance, pentachrome vitiligo in a segmental type was diagnosed.

We report a method to establish amelanotic melanocytes (AMMC) in culture and we investigate the effects of various components in the culture medium. Normal human scalp from cadaver donors was transected 1 mm below the epidermis, and hair follicles in the remaining dermis were isolated by a two-step enzyme treatment. The individual hair follicles were washed exhaustively and suspensions of hair follicle cells were prepared and cultured in Eagle's minimum essential medium supplemented with 12-o-tetradecanoyl-phorbol-13-acetate (TPA), cholera toxin and keratinocyte serum-free medium (K-SFM). Geneticin was used to eliminate contaminating fibroblasts. Proliferation of AMMC was observed after addition of TPA and K-SFM including bovine pituitary extract (BPE) into the culture medium. Cell type was determined by staining with monoclonal antibodies, NKI/beteb and HMB-45, which recognize premelanosomal and melanosomal antigens, respectively. The AMMC were also examined using transmission electron microscopy. Treatment with geneticin eliminates the majority of fibroblasts and does not impair the growth of keratinocytes or AMMC. After contaminating fibroblasts and keratinocytes were removed, two distinct cell morphologies remained: (1) large, dendritic and deeply pigmented cells, which did not proliferate and which disappeared by the third passage, and (2) small bipolar cells, which initially were unpigmented and proliferated very rapidly. We observed that TPA at various concentrations stimulated the proliferation of the cells, and at high concentrations could induce the formation of multiple dendrites. K-SFM including BPE accelerated the proliferation of the cells in a dose-dependent manner. After passage 3, almost all cells expressed premelanosomal and melanosomal antigens, recognized by NKI/beteb and HMB-45, respectively. Active mitochondria, abundant rough endoplasmic reticulum, Golgi complexes, ribosomes and melannosomes (predominantly in stages I, II or III with some at stage IV in some AMMC) were observed ultrastructurally in the cytoplasm of the cultured cells.

The dendritic nature, the strategic location, and other accumulated evidence about the immunologic characteristics of melanocytes suggest that they are not only professional melanin producing cells but are also immunocompetent cells. In this study, we demonstrated that cultured melanocytes express low levels of some immunologically important surface markers such as intercellular adhesion molecule-1 and CD 40. Moreover, we report for the first time CD 40 expression by melanocytes can be up-regulated by interferon-gamma (IFN-gamma) stimulation. Optimal enhancement of CD 40 expression was observed at an IFN-gamma concentration of 300 U/ml after a co-culture period of 72 h. Maximal melanocyte-driven T lymphocyte proliferation and interleukin-12 secretion were also observed following the same treatment and proved to be CD 40-dependent. Our data further suggest that upon CD 40 ligation, melanocytes up-regulate their co-stimulating and adhesion molecules. In addition to previous descriptions about the melanocyte's antigen processing and presenting capacity, we therefore hypothesize a dynamic model in which melanocytes alternatively work as heterogeneous antigen presenting cells. As a result of CD 40 expression on the cell surface, melanocytes might contact and subsequently stimulate CD8+ cytotoxic T lymphocytes directly via CD 40-CD 40 L interaction in some cases.

Cutaneous papillomavirus infection is common in patients who are immunosuppressed. We describe swollen keratinocytes in the granular layer in lesions from four patients who had human immunodeficiency virus infection. These cells were similar to those described in skin lesions of epidermodysplasia verruciformis. Amplification of DNA from the lesions revealed an amplimer for human papillomavirus using a consensus primer for a highly conserved region of the L1 open reading frame; however, specific binding was not noted when radiolabelled probes for human papillomavirus types 6, 11, 16, 18, and 33 were used. We conclude that the presence of these distinctive swollen cells strongly suggests immunosuppression and quite possibly infection by a less common papillomavirus type.

A 31-year-old male patient complained of having follicular and brownish red maculopapules along the Blaschko's lines on the right chest for 2 days. On examination, follicular brownish maculopapules were present on the chest with a uniform size of about 3 mm in diameter. The lesions were isolated without a tendency to merge, giving several S-shaped, band-like appearances. Direct mycological examination of the skin flakes revealed many pseudomycelial hyphae and yeast cells with typical spaghetti and meatball appearance. Wood's light examination of the lesion revealed a golden yellow fluorescence. A diagnosis of blaschkoid pityriasis versicolor was suggested because of blaschkoid distribution of the lesions in this new variant of PV.

Sections from paraffin-embedded tissues of lesions thought to represent verrucous lesions (benign verrucous acanthomas: BVA) as well as materials from controls including seborrheic keratoses (SK), irritated SK, and verruca vulgaris from areas other than the genitalia were analyzed for the presence of multiple human papillomaviruses (HPV) including types 6, 11, 16, 18, and 33 using DNA amplification methodology. HPV DNA sequences were not found in amplified samples of BVA, SK, and irritated SK samples, but were identified in those obtained from verruca vulgaris. Our results suggest that BVA, SK, and irritated SK are generally not associated with HPV DNA even though certain of these lesions may histologically mimic the architecture of verruca.

Hair casts were seen in 22 girls ranging in age from 4 to 13 years. They were localized to the frontal, vertex, and temporal areas. The number of affected hairs varied from one-tenth to one-third. Twenty of the 22 girls styled their hair in ponytails or pigtails. Compound root sheath casts were present in six of nine patients using 1% 4-dimethylaminocinamaldehyde stain. A mass of large polymorphous scales and solitary or clumped spores was seen on the surface of hair casts using scanning and transmission electron microscopy. Energy-dispersive x-ray microanalysis showed that elements of silicon, aluminum, and molybdenum were uniquely present in hair casts. We assume that tightly drawn braids, by causing local circulatory disturbances and inflammation of the scalp, may be one etiologic factor in the disorder.

Background. A viral etiology has been suspected in papillomatosis of the external auditory canal (peac), but virus particles have not been detected so far, although they are easily demonstrable in skin warts. The purpose of the study was to solve this discrepancy by the use of polymerase chain reaction (pcr). Materials and Methods. Specimens from the external auditory canal of 14 patients with PEAC, but no human papilloma virus infection of the genital areas, were examined histologically by light and electron microscopy, as well as by PCR to detect viral dna. Results. Histologically, papillomatosis was present in all specimens. Vacuolated cells were found in the upper part of the stratum malpighii in five cases. On electron microscopy, the numbers of perichromatin and interchromatin granules were increased, but no viral granules were observed. In all specimens, dna of hpv 6 was detected using PCR, but there was no evidence of DNA of other hpv. Conclusions. Papilloma of the external auditory canal is produced by infection with hpv 6.

Fixed drug eruption (FDE) usually presents as a single oval to round violaceous patch after administration of the causative drug. We describe a case of FDE presenting with multiple annular erythematous lesions over dorsal aspects of both feet following the administration of phenobarbital. To the best of our knowledge, such an atypical demonstration had never been reported. Fixed drug eruption should be considered in the differential diagnosis of annular lesions.

We studied a case of infantile digital fibromatosis (IDF) transmission electron microscopy. We could not detect either human papillomavirus (HPV) DNA types 6,11,16, and 18, or herpes simplex virus (HSV) DNA types 1 and 2 in IDF. Our results suggest that IDF is not asso-ciated with HPV and HSV infection.