Tinea folliculorum complicating tinea of the glabrous skin: an important yet neglected entity.

Dermatophytes are capable of infecting the skin and its appendages such as nails and hairs producing a variety of clinical conditions. Hair invasion by dermatophytes is a key feature of tinea capitis and tinea barbae but not of tinea of glabrous skin. In this project, we studied the clinico-mycological aspects of follicular involvement in patients with dermatophytosis of the glabrous skin. In total, 16 patients, eight males and eight females, were included in the study. All were adults except for one girl. The disease durations ranged from one month to more than ten years. Fourteen (78.5%) had multiple lesions, and most of them had undergone treatment with antifungals, antibiotics, or steroids. Dermoscopic examination showed infected hairs in the form of broken stubs, coily, curly, or as black dots on the surface of the lesions. Pathogens were either anthropophilic (seven cases of Trichophyton rubrum) or zoophilic (six cases Microsporum canis, three cases of the T. mentagrophytes). Patients responded well to oral griseofulvin or terbinafine, and topical antifungals. No antifungal resistance developed during the treatment course. Follicular involvement of glabrous skin is not as rare as previously thought and should be considered for systemic antifungal treatments.

Recurrent digital glomus tumor: analysis of 75 cases.

BACKGROUND: Glomus tumors are rare, benign, cutaneous neoplasms that must be excised completely to prevent recurrence. OBJECTIVE: To investigate factors associated with recurrence of glomus tumors after surgery. METHODS AND MATERIALS: Fifty-eight women and 17 men with digital glomus tumors underwent surgery between 1990 and 2008 at our hospital. These cases were retrospectively analyzed. RESULTS Mean age at diagnosis was 41.8, with an average diagnostic delay of 3.9 years. The tumor was located on a finger in 70 cases (right, 29; left, 41) and a toe in five (right, 3; left, 2). The tumor recurred in 13 (17%) patients. Recurrence was more likely if the tumor was skin-colored (odds ratio (OR)=31.67, 95% confidence interval (CI)=2.68-373.74, p=.006) or located within the nail matrix (OR=5.79, 95% CI=1.03-32.49, p=.046). No recurrence occurred in patients who had had preoperative magnetic resonance imaging or ultrasound studies. CONCLUSION: Skin-colored tumors or those in the nail matrix are at higher risk of recurrence. The authors have indicated no significant interest with commercial supporters.

NEMO gene mutations in Chinese patients with incontinentia pigmenti.

BACKGROUND/PURPOSE: Incontinentia pigmenti is a rare, X-linked, dominant genodermatosis affecting skin, teeth, eyes, and central nervous system. Symptoms are associated with mutations in the nuclear factor-kappa B essential modulator (NEMO) gene on chromosome Xq28. Here, a subpopulation of Chinese patients with incontinentia pigmenti were examined to investigate the frequency and pattern of NEMO mutations, and to analyze their clinical features. METHODS: From January 1996 to August 2006, 52 participants (21 probands and 31 family members) were screened for symptoms of incontinentia pigmenti and NEMO gene mutations. We designed a NEMO-specific PCR primer, referred to as In2S, to detect a deletion of exon 4-10 of the NEMO gene, which represents the mutation most frequently associated with incontinentia pigmenti. For participants without this deletion, all exons were sequenced to screen for other NEMO mutations. In addition, the clinical manifestations and family histories of the participants were analyzed. RESULTS: Exon 4-10 was deleted in 13 probands, and one proband had a novel point mutation (G549C) in exon 5 that converted a glutamine to a histidine. Seven probands (33%) had no mutation in any of the exons of the NEMO gene. One of four participants who presented with hyperpigmentation also had the exon 4-10 deletion. One patient had a positive family history before the study took place, but no NEMO mutation was identified in any of the family members. Remarkably, the mothers of three of the probands exhibited the exon 4-10 deletion; however, their clinical manifestations were subtle and unrecognizable. CONCLUSION: Mutational analysis of the NEMO gene was helpful in diagnosing incontinentia pigmenti among participants with a nearly normal phenotype or an incomplete form of the disease that only caused hyperpigmentation symptoms.

Necrolytic acral erythema without hepatitis C infection.

Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. More than 30 patients have been reported since 1996, all of whom had anti-hepatitis C virus antibody. A 32-year-old Taiwanese woman had been diagnosed with and treated for systemic lupus erythematosus with lupus nephritis about 10 years earlier. Soon thereafter, she noted several well-demarcated keratotic plaques with erythematous borders on her feet, with sparing of the soles. Histopathology showed diffuse parakeratosis with a neutrophil infiltrate, hypogranulosis, pale upper keratinocytes, scattered and grouped dyskeratotic cells, psoriasiform hyperplasia and a mild lymphocytic infiltrate in the upper dermis. The diagnosis was made after three biopsies. The lesions regularly worsened just before and during menstruation, but patch and intradermal tests for progesterone and estrogen were negative. There was no evidence of either hepatitis B or hepatitis C infection. The lesions did not respond to treatment with zinc. The rash regressed spontaneously when corticosteroids were stopped and recurred when they were restarted, finally resolving completely after she was treated with high-dose pulse steroids for her lupus.

Nail changes and association of osteoarthritis in digital myxoid cyst.

BACKGROUND: Digital myxoid cyst is relatively common on the digits. Nail changes and osteoarthritis are usually associated with this pathogenesis. OBJECTIVE: The objective was to clarify this relationship and its significance by analyzing the clinical, radiologic, and pathologic data. METHODS AND MATERIALS: Fifty-one patients with digital myxoid cysts were diagnosed and analyzed during a 5-year period. RESULTS: Thirty-eight (74.5%) of 51 patients showed radiologic evidence of primary interphalangeal joint osteoarthritis in the affected digits. Among the 39 cysts removed surgically and examined pathologically, 28 were myxomatous and 11 were ganglion. Different clinical manifestations of nail change, including longitudinal grooves (20 cases), concave canaliform dystrophy (9 cases), "washboard" transverse lines (5 cases), and longitudinal grooves with beaded ridges (1 case) were found. CONCLUSION: Osteoarthritis of the adjacent terminal joint is an important factor involved in forming digital myxoid cysts. Of the two cyst types, ganglion cysts are related to osteoarthritis more often than myxomatous cysts. Nail changes, secondary to matrix damage, are reliable clinical signs of underlying cyst formation. Understanding the pathogenesis of the digital myxoid cyst in relation to osteoarthritis and nail deformity helps inform the care of these patients.

African tick bite fever.

Physicians may encounter unfamiliar diseases as a result of international travel. Fever with rash is an important sign that usually represents initial manifestation of infectious disorders. A 62-year-old Taiwanese woman presented with multiple eschars, a papulovesicular rash, and fever 5 days after returning from South Africa. A biopsy specimen of an eschar had wedge-shaped tissue necrosis, hemorrhage, necrotizing vasculitis of the small venules and arterioles, and a dense perivascular lymphocytic infiltrate in the dermis. Serologically, there was cross reaction with both Rickettsia conorii and R. rickettsii. DNA sequencing demonstrated R. africae, confirming the diagnosis of African tick bite fever. The patient responded well to minocycline. Recognition of the symptoms and signs, and diagnostic tools for different types of rickettsiosis are essential for correct diagnosis and treatment.

Generalized Dowling-Degos disease.

BACKGROUND: Dowling-Degos disease (DDD) is a rare inherited disease characterized by reticular hyperpigmentation on flexor surfaces. OBJECTIVE: We sought to describe several cases of generalized DDD, a presentation that resemble dyschromatosis universalis hereditaria. METHODS: The clinical manifestations, histopathologic, and genetic studies of a family with autosomal dominant inheritance were analyzed. RESULTS: The father and his sister had reticular hyperpigmentation on flexor surfaces, whereas the daughter and son had generalized hyperpigmentation with numerous hypopigmented or erythematous macules and papules on the trunk and limbs. Skin biopsy specimens from both types of lesions all had typical features of DDD. Biopsy specimens from axillary skin had features of Galli-Galli disease, an acantholytic form. There were no mutations of the double-stranded RNA-specific adenosine deaminase or keratin 5 genes. LIMITATION: Generalizations cannot be drawn from genetic study of only one family. CONCLUSION: DDD can present with generalized hyperpigmentation and hypopigmented papules.

Congenital curved nail of the fourth toe--three different clinical presentations.

A congenital curved nail of the fourth toe (NIM 219070) is a rare nail deformity with no other associated abnormalities. Three patients with this congenital anomaly are reported here. Radiologic examination in all three revealed distal symphalangism of the fourth toes bilaterally. The clinical manifestations in these patients included white discoloration of the proximal nails and hyperkeratotic tylosis.

Histopathologic-molecular correlation in early mycosis fungoides using T-cell receptor gamma gene rearrangement by polymerase chain reaction with laser capture microdissection.

BACKGROUND/PURPOSE: Early mycosis fungoides (MF) is difficult to distinguish from other benign inflammatory dermatoses. We evaluated clonal T-cell receptor (TCR) gamma gene rearrangement by polymerase chain reaction (PCR) as a surrogate to histologic diagnosis in early MF. METHODS: Twenty paraffin-embedded skin biopsies from nine patients diagnosed with MF were included. Two multiplex PCR encompassing various Vgamma and Jgamma regions were used to detect TCRgamma gene rearrangements. Histologic diagnoses were categorized as "diagnostic", "consistent", "suggestive", or "nondiagnostic". We compared TCRgamma PCR results with histologic parameters to determine the differences between PCR-positive and PCR-negative groups. RESULTS: TCRgamma PCR was positive in 53% (8/15) of the patch stage, in 100% (2/2) of the plaque stage, and in 100% (3/3) of the tumor stage. TCRgamma PCR was positive in 50% (4/8) of the specimens in both the diagnostic and consistent of MF groups, 71% (5/7) in the suggestive of MF group. We found that inflammation was more severe in PCR-negative specimens. Papillary dermal fibrosis was common, and differed significantly between PCR-positive and PCR-negative groups (p = 0.01). T-cell monoclonality was detected in one nondiagnostic lesion in a patient with psoriasis and MF. CONCLUSION: TCRgamma PCR allows the diagnosis of MF in patients with lymphocyte-poor lesions, suggestive of MF pathologically. TCRgamma PCR is more likely to be negative with moderate to severe inflammation, particularly with papillary dermal fibrosis. We suggest that the ratio of malignant clonal to reactive T-cells is critical for MF diagnosis.

Cytoid bodies in cutaneous direct immunofluorescence examination.

BACKGROUND: The clinical significance of scattered or grouped cytoid bodies seen on direct immunofluorescence (DIF) examination has not been systematically analyzed in a variety of disorders. METHODS: We prospectively studied skin biopsy specimens obtained from July 2002 to December 2005 for which DIF examination using immunoglobulin (Ig) A, IgG, IgM, C3 and fibrinogen had been performed. Slides with cytoid bodies were further reviewed to assess the number, intensity and location of the cytoid bodies and for other specific findings. These data were compared with both clinical records in the medical charts and pathologic findings from concurrent skin biopsies. RESULTS: Of 1080 DIF examinations performed during the study period, 117 (10.8%) had cytoid bodies. These specimens were from patients with 28 different cutaneous disorders, the most common of which were various forms of lupus erythematosus (29/117, 24.8%) and erythema multiforme (22/117, 18.8%). In 81 (69.2%) of these specimens, interface dermatitis was found histopathologically. CONCLUSION: Cytoid bodies are not infrequent on DIF examination and, in the absence of a clear diagnosis, might point toward several common disorders, particularly those involving interface dermatitis.

Isotopic response of fungal granuloma following facial herpes zoster infections - report of three cases.

BACKGROUND: An isotopic response is the occurrence of a new skin disease at the site of another unrelated, healed skin disorder. METHODS: We report three cases of unilateral granulomatous fungal infection in immunocompetent patients at sites of resolved herpes zoster on the face. Diagnoses were made by potassium hydroxide preparation, histopathologic findings, and fungal culture. Two patients had Candida albicans folliculitis, and the other was infected with both Epidermophyton floccosum and Trichophyton mentagrophytes. RESULTS: The patients responded well to antifungal therapy. CONCLUSIONS: Localized isotopic fungal infections, although rare, can occur in immunocompetent patients.

Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child.

Hydroa vacciniforme (HV) is a chronic photosensitivity disorder induced by ultraviolet radiation. Hydroa vacciniforme-like lymphoma is a rare cutaneous T-cell lymphoma occurring mainly in childhood. Recent studies have demonstrated an association between chronic latent Epstein-Barr virus (EBV) infection and both the benign skin disorder and the lymphoma. The authors report a 6-year-old boy with chronic EBV infection, HV-like skin eruptions, and chronic hepatitis. Histopathologic examination of a skin biopsy specimen demonstrated epidermal ballooning degeneration and dense superficial and deep perivascular and periappendageal lymphoid cell infiltrates extending to the fat lobules. Some blood vessels in the deep plexus were infiltrated by predominantly CD4+ and TIA-1+ cytotoxic T cells. The EBV genomes were found within tissue from three skin biopsies and peripheral blood cells. Monoclonal T-cell receptor gene rearrangement was present in skin biopsy specimens. Although no lymphoma has been found during 2 years of follow-up treatment, the possibility of lymphoma developing out of the current smoldering stage is of concern. The clinical manifestations of lymphoproliferative disorder and chronic active EBV infection are discussed.

Verruciform xanthoma-like phenomenon in seborrheic keratosis.

Verruciform xanthoma is xanthomatous dermal infiltrate in a proliferating epidermal lesion and is an uncommon phenomenon. It has been reported in various neoplastic or inflammatory conditions. We report a 72-year-old man who had an asymptomatic 1-cm black nodule on his abdomen. Histopathology showed a typical acanthotic type of seborrheic keratosis characterized by basaloid keratinocyte proliferation and pseudohorn cysts. Many aggregated xanthomatized cells were seen in dermal papillae within the acanthotic epithelium. Papillomatosis, parakeratosis, and neutrophil infiltrates, the histologic features of typical verruciform xanthoma, were not seen. The foamy cells were positive for CD-68 and vimentin and negative for cytokeratin and S-100. No human papillomavirus DNA was found by nested polymerase chain reaction. The blood lipid profile was normal. The presence of verruciform xanthomatous change in seborrheic keratosis provides further evidence that verruciform xanthoma may be a reactive phenomenon occurring in common skin disorders.

A surgical approach to ingrown nail: partial matricectomy using CO2 laser.

BACKGROUND: Ingrown nail is a deformity characterized by a transverse overcurvature of the lateral nail plate that causes nail fold inflammation. Currently recommended surgical procedures vary considerably. OBJECTIVE: We report our experience with a modified partial matricectomy and nail edge excision with CO2 laser. METHODS: A total of 116 ingrown nail edges in 77 patients were treated with the procedure. RESULTS: The incidence of recurrence was 2 of 76 cases (2.6%). CONCLUSION: This new surgical approach was found to be effective and is presented as an alternative treatment technique.

Subungual pleomorphic fibroma.

BACKGROUND: Pleomorphic fibroma is a benign fibroblastic tumor characterized by pleomorphic, hyperchromatic cells or giant multinucleated cells embedded in a collagenous stroma. These cytologic features may lead to an incorrect diagnosis of malignancy. Most cases reported in the literature are located on trunk or extremities; the presentation as a subungual mass is rare. METHODS: We report an unusual case of a subungual pleomorphic fibroma in 66-year-old woman. Clinical information was obtained. Histologic examination and immunohistochemical studies were performed. RESULTS: A 66-year-old woman presented with a longstanding (40 years), subungual mass that deformed the nail of the left middle finger. Microscopic examination revealed a paucicellular tumor composed of hyperchromatic spindled, pleomorphic, floret-like giant cells embedded in haphazardly arranged collagen bundles in the dermis. No mitotic figures were seen. The tumor cells were vimentin-positive but did not stain with antibodies to S-100, cytokeratin, smooth muscle actin, factor XIIIa or CD34 negative. The diagnosis of a pleomorphic fibroma was made. Follow-up shows no evidence of tumor, 36 months after excision. CONCLUSION: Pleomorphic fibroma of the subungual region is an unusual cutaneous tumor with histologic features that may cause confusion with true sarcomas. This is only the second case reported of a subungual pleomorphic fibroma. Pleomorphic fibroma should be considered in the differential diagnosis of pleomorphic subungual tumors.