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Objective: To establish and validate a new system to define the severity of onychomycosis. The Onychomycosis Severity Index (OSI) score is obtained by multiplying the score for the area of involvement (range, 0-5) by the score for the proximity of disease to the matrix (range, 1-5). Ten points are added for the presence of a longitudinal streak or a patch (dermatophytoma) or for greater than 2 mm of subungual hyperkeratosis. Mild onychomycosis corresponds to a score of 1 through 5; moderate, 6 through 15; and severe, 16 through 35., Design: Consensus conference., Setting: Teleconference., Participants: The consensus group included 5 dermatologists, 1 dermatology resident with an interest in nail disorders, and a statistician. The meetings were held by closed teleconference., Main Outcome Measures: Index reliability., Results: The reliability of the OSI system was assessed in 2 steps. The first assessment included 37 dermatologists who scored 8 photographs of onychomycosis after being taught how to use the OSI. The scoring system showed very high reliability (Cronbach α = 0.99 and intraclass correlation coefficient [ICC] = 0.95). The second assessment entailed evaluation of 49 nails by 3 dermatologists, including an expert in the OSI. This assessment was conducted at the University of Alabama at Birmingham and at the Oregon Dermatology and Research Center, Portland. The scoring system showed very high reliabilities at both sites (Cronbach α = 0.99 and ICC = 0.96 at the University of Alabama at Birmingham, and Cronbach α = 0.98 and ICC = 0.93 at the Oregon Dermatology and Research Center)., Conclusion: The OSI is a new, simple, objective, reproducible numeric system to grade the severity of onychomycosis.

Background: Mycobacterium bolletii and Mycobacterium massiliense are recently described species of nontuberculous mycobacteria. Footbaths preceding pedicures at nail salons have been implicated as reservoirs of infection with nontuberculous mycobacteria. To our knowledge, this case series represents the first documented outbreak of M bolletii/M massiliense furunculosis, identified by heat-shock protein 65 gene, hsp65, sequencing, occurring in immunocompetent patrons of a North Carolina nail salon., Observations: We describe 3 cases of lower extremity furunculosis caused by M bolletii/M massiliense associated with pedicure footbaths from the same North Carolina nail salon. Lesions developed within 1 month of the salon visit and were characterized by erythematous, indurated papules and plaques. Histologic examination revealed suppurative granulomatous dermatitis. Mycobacterium bolletii/M massiliense was identified by sequencing the 16S ribosomal RNA (rRNA) and hsp65 genes. All 3 patients responded to different combinations of clarithromycin, doxycycline hydrochloride, azithromycin, and moxifloxacin hydrochloride for complete lesion resolution., Conclusions: Clinicians should elicit a history of pedicure footbaths and maintain a high level of suspicion when faced with skin lesions of the lower extremities that are culture negative or are refractory to conventional antibiotic therapy. Accurate identification and discrimination of M massiliense and M bolletii is difficult and requires sequencing of multiple gene targets beyond their identical 16S rRNA sequences.

Objective: To further characterize the clinical and pathologic features, disease associations, and laboratory abnormalities of livedoid vasculopathy., Design: Retrospective study of patients identified from our institutional database from January 1, 1990, to December 31, 2000., Setting: Tertiary care institution. Patients Forty-five patients with biopsy-proved livedoid vasculopathy., Main Outcome Measures: Clinical presentation, histopathologic diagnosis, results of testing for coagulation abnormalities, and assessment of vascular status., Results: Thirty-two patients (71.1%) were female (mean age, 45 years; age range, 10-85 years). Bilateral lower extremity disease occurred in 36 patients (80.0%), ulceration in 31 (68.9%), and atrophie blanche in 32 (71.1%). In patients tested, transcutaneous oximetry measurements were decreased in 20 (74.1%) of 27, and factor V Leiden mutation (heterozygous) was noted in 2 (22.2%) of 9, decreased activity for protein C or protein S in 2 (13.3%) of 15, prothrombin G20210A gene mutation in 1 (8.3%) of 12, and lupus anticoagulant in 5 (17.9%) of 28. Anticardiolipin antibodies were present in 8 (28.6%) of 28 patients, and elevated homocysteine levels in 3 (14.3%) of 21. Intraluminal thrombosis was observed in 44 (97.8%) of 45 skin biopsy specimens. Direct immunofluorescence disclosed multiple vascular conjugates in 31 (86.1%) of 36 biopsy specimens., Conclusions: Livedoid vasculopathy was predominantly bilateral, affected the lower extremities, and was associated with ulceration and atrophie blanche. Histologic evidence of intraluminal thrombosis was observed in almost all biopsy specimens reviewed. Laboratory testing revealed numerous heterogeneous coagulation abnormalities, providing further evidence of procoagulant mechanisms.

Objective: To analyze the epidemiology, responsible agents, clinical features, and outcome of white superficial onychomycosis (WSO)., Design: Retrospective study., Setting: University hospital., Patients: A total of 79 patients with WSO seen at the Department of Dermatology of Bologna University from 1994 to 2002. Responsible agents included Trichophyton interdigitale in 58 cases (73%), Trichophyton rubrum in 4 (5%), Fusarium species in 9 (11%), Aspergillus species in 5 (6%), and Acremonium strictum in 3 (3%)., Results: White superficial onychomycosis may have different clinical and epidemiological features. "Classic" WSO, characterized by superficial nail plate involvement, is usually due to Trichophyton mentagrophytes (var interdigitale), although Acremonium strictum or Onychocola canadiensis can sometimes be responsible. A deep and diffuse WSO, characterized by massive penetration of the nail plate by fungi, can be seen in nail infections by molds such as Fusarium species and Aspergillus species, or in nail infections by Trichophyton rubrum in healthy children and in patients infected with human immunodeficiency virus., Conclusions: Severity and spread of WSO is the result of complex host-parasite relationships. When dealing with a patient with WSO, we should always consider the causative organism and the host characteristics to choose the best therapeutic approach.

Background: The standard treatment of Trichophyton rubrum nail bed onychomycosis (or distal subungual onychomycosis [DSO]) with daily terbinafine for 12 weeks involves treating for a fixed period shorter than the time required for complete replacement of the nail bed and overlying nail plate by normal growth. The same total amount of terbinafine pulse-dosed for approximately 12 months would treat the patient until normal replacement of the mycotic nail bed has occurred., Objectives: To determine the effectiveness of intermittent administration of oral terbinafine (250 mg/d for 7 consecutive days every 2-4 months) to cure DSO and to determine the maximum effective treatment interval., Design: A prospective, nonrandomized, open study of sequential groups of office patients., Setting: A private dermatology practice., Methods: A sequence of 4 groups of office patients with DSO (n = 10-20 each) were treated with pulse-dosed terbinafine for 7 consecutive days at intervals of 2, 3, and 4 months, respectively. In each group, treatment was continued until the distally advancing new nail bed and nail had completely removed the mycotic defect or failure of fungistasis was detected., Main Outcome Measurement: Results were determined by monthly evaluation. Cure was noted as complete replacement of the mycotic nail bed and overlying nail plate (ascertained by monthly metric measurements of the mycosis-free nail bed and overlying nail place distal to the proximal nail fold). Treatment failure was noted when the mycosis-free proximal portion of the nail bed failed to increase in correspondence with the distally directed movement of the nail bed and overlying nail., Results: Thirty-nine (93%) of the 42 patients in the first 3 groups were cured (95% binomial confidence interval, 67%-100%) with no evidence of decrease in efficacy. However, the group of patients who received the 7-day pulse treatment every 4 months experienced significantly more failures (P<.01), and cures dropped to 10 of 17 cases., Conclusion: Terbinafine is an effective treatment for DSO when pulse-dosed for 7 days every 3 months but not every 4 months.

Background: Flagellate hyperpigmentation is a well-documented complication of systemic bleomycin sulfate therapy when using doses of 100 U or more as an antineoplastic agent. Two cases occurred after using systemic doses from 15 to 30 U injected intravenously or intrapleurally; however, it has not been described as a complication following intralesional treatment of verruca plantaris., Observations: We report a case of flagellate hyperpigmentation after intralesional injection of 14 U of bleomycin for verrucae plantaris and review the literature associated with this cutaneous complication., Conclusions: Flagellate hyperpigmentation from extremely low doses of intralesional bleomycin is a previously undescribed complication. Although the mechanisms of reaction are not clearly understood, the clinician should be mindful of this uncommon complication.

Background: Necrolytic acral erythema is a recently described necrolytic erythema that is unique in its exclusive acral location and strong association with hepatitis C., Observation: We report the first case of necrolytic acral erythema in the United States. The patient is a 43-year-old black woman who presented with a 4-year history of tender, flaccid blisters localized to the dorsal aspect of her feet. Serum zinc and glucagon levels were normal. Serum antibodies were positive for hepatitis C, and a liver biopsy specimen showed chronic hepatitis. She was successfully treated with interferon alfa-2b and zinc. We review all previously reported cases., Conclusions: Necrolytic acral erythema is a distinct entity. In a review of the literature, most patients were between 35 and 55 years of age, although 1 patient was 12 years old. Five of 8 patients were female. Four of 7 patients described previously were treated with variable success using oral zinc sulfate and amino acids, whereas 2 were successfully treated with interferon alfa. All patients were infected with hepatitis C. Necrolytic acral erythema appears to be a skin disorder linked to infection with hepatitis C virus that responds to treatment with interferon alfa and oral zinc.

Background: Idiopathic recurrent palmoplantar hidradenitis (IRPH) is characterized by tender erythematous plaques and nodules on the soles and, less often, the palms of young patients. To date, 10 cases of IRPH have been documented in the literature., Observations: We describe 22 pediatric patients with characteristic cutaneous and histologic findings of IRPH. Their mean age was 6 years (age range, 1.5-15 years). The onset of the disease clustered in 2 peaks, in autumn and spring. All patients had complete resolution of their lesions within 3 weeks, in 16 cases without any treatment. Ten of the 22 patients experienced more than 1 episode of IRPH., Conclusions: Our study represents the largest number of cases of IRPH collected to date. Based on our data, the clinical picture is so characteristic that a histopathologic examination may not be necessary in every case. We suggest that there is a seasonality in the occurrence of this always benign and limited disease.

Background: Eccrine squamous syringometaplasia (ESS) has been associated with characteristic clinical eruption in patients receiving chemotherapy. It has been suggested as a diagnostic clue in the diagnosis of chemotherapy-induced reactions vs acute graft-vs-host disease, as well as other drug reactions. We identified 10 cases of ESS in patients in whom a distinctive clinical eruption developed during or after a pretransplantation conditioning regimen with high-dose chemotherapy. A complete clinical and histologic evaluation was performed in all patients., Observations: All patients developed erythematous and edematous plaques or confluent erythematous macular areas in the axillae and/or groin, with painful areas of well-defined erythema and edema on palms and/or soles in 5 patients. Some discrete papular lesions on the trunk or extremities could also be observed in most patients. The histologic hallmark of the eruption was ESS, with a variable degree of cornification and apoptosis. A vacuolar interface dermatitis and a variable degree of cellular atypica were also consistent findings., Conclusions: Chemotherapy-induced ESS may be associated with a distinctive clinical eruption and should be considered in the differential diagnosis of erythematous eruptions during or after a pretransplantation conditioning regimen with high-dose chemotherapy.

Background: There are several reports of tender, erythematous plantar nodules occurring in pediatric patients. Despite similar morphological features, the histologic findings in these lesions have been quite diverse. A new entity called idiopathic plantar hidradenitis (also termed neutrophilic eccrine hidradenitis in children), which is characterized by tender, red nodules on the feet and unique histopathologic features, was recently described., Observations: We describe two children with these unusual cutaneous lesions and histopathologic findings and compare them with patients with idiopathic plantar hidradenitis. Our patients, in contrast to those with idiopathic plantar hidradenitis, had involvement of the palms as well as the soles. Both children had self-limited recurrent lesions; in one child, the lesions were associated with low-grade fever. Biopsy specimen findings in both cases demonstrated dense neutrophilic infiltrates localized to the eccrine units., Conclusions: Our patients had clinical and histologic findings similar to those recently reported as idiopathic plantar hidradenitis (neutrophilic eccrine hidradenitis in children). Because palms and soles can be affected and the lesions typically recur, we suggest that this condition be referred to as recurrent palmoplantar hidradenitis.

Background: We describe a new variant of inherited epidermolysis bullosa and elucidate the clinical, histologic, and ultrastructural features of this condition., Observations: This form of epidermolysis bullosa displays an autosomal dominant inheritance pattern, is characterized by acral bullae, and histologically demonstrates suprabasal clefting with acantholysis. Ultrastructural findings are nonspecific but reminiscent of those observed in benign familial pemphigus., Conclusion: Acantholytic epidermolysis bullosa is a rare but distinct clinicopathologic entity that warrants inclusion in the nosologic classification of epidermolysis bullosa.

Background and Design: In vivo epiluminescence microscopy is now used as a useful noninvasive method for determining clinical diagnosis of pigmented skin lesions. Until now, however, pigmented lesions on the volar skin have been hardly studied with this method. In the present epiluminescent study, various kinds of pigmented lesions on the volar skin were extensively investigated by means of video macroscope, a newly developed electronic device with a higher magnification power, and correlation between the magnified features and histopathologic findings was evaluated., Results: Magnified features of most lesions of acquired or congenital melanocytic nevus on the volar skin were classified into the following three typical patterns: (1) a parallel pattern formed by pigmented parallel lines corresponding to the furrows of the skin markings, (2) a latticelike pattern composed of pigmented lines along and across the furrows of the skin markings, and (3) a fibrillar pattern formed by densely packed, fibrillar pigmented lines arranged in the direction crossing the furrows. In contrast, macular or plaque portions of acral lentiginous melanoma exhibited disorderly arranged, irregular pigment patterns, mainly affecting the ridges of the skin markings. In addition, brown globules of various shades and many black dots of variable sizes were often observed and, on the margin of the lesions, pseudopods and/or the "serrated" pattern were detected. Cutaneous hemorrhagic macule and so-called black heel showed highly specific features and thus could be easily diagnosed with video macroscopy., Conclusion: Video macroscope proved to be a very useful instrument for the diagnosis of pigmented lesions on the volar skin.

Background: Various kinds of viral warts have their own typical clinical appearances and histologic properties. From each of them, a corresponding unique type of human papillomavirus (HPV) has been detected. More than 60 types of HPV have been identified from the warts and other squamous cell epithelial tumors. Human papillomavirus type 60 has been identified in an epidermal cyst on the sole. We present a case of a peculiar plantar wart infected with HPV type 60., Observations: A 21-year-old male student complained of yellowish hyperkeratotic slightly elevated nodules on the right plantar arch. The surface of the nodules was not papillary, but a normal ridge pattern of the sole could be traced. Histologic findings included moderate acanthosis, papillomatosis, and eosinophilic intracytoplasmic large granules with or without surrounded vacuoles. These were noted in the middle and upper epidermis, including the hypertrophic stratum corneum. Virologic findings included HPV antigen detected by anti-HPV polyclonal antibody. The DNA of HPV type 60 was detected by in situ hybridization., Conclusion: A new type of plantar wart infected with HPV type 60 is reported. It shows unique clinical and histologic findings that can be distinguished from those of ordinary verruca or myrmecia on the sole.

Background: We had recently cloned a new cutaneous human papillomavirus type 60 (HPV-60) from a plantar cyst with characteristic pathologic changes and identified it in additional cysts. However, it is not known whether or not HPV-60 infection causes the other cutaneous lesions., Observations: Six plantar warts were found in five patients. These warts were found either with concomitant plantar cysts (four patients) or without an accompanying cyst (one patient). All warts showed a similar clinical appearance, including a smooth and slightly elevated surface and the retained appearance of the dermal ridges. Their histopathologic features were identical to those found in the wall of the cysts reported previously, namely hyperkeratosis with vacuolated structure with or without nuclei in the horny layer and amorphous eosinophilic cytoplasmic inclusions in the cells with peripherally located nuclei in the granular and prickle cell layers. By Southern blot analysis, all warts were found to harbor HPV-60 DNA. In addition, immunohistochemistry and in situ hybridization analysis revealed the presence of viral capsid antigen and viral genome in these lesions, respectively., Conclusion: We conclude that HPV-60 infection on the plantar skin causes the warty lesion, designated as ridged wart, distinct from those infected with other cutaneous human papillomaviruses.

Background: Acral lentiginous melanoma is the most common type of malignant melanoma in the Japanese population. In most instances, classic acral lentiginous melanoma or acral lentiginous melanoma in situ can be readily distinguished from other benign pigmented lesions by virtue of its atypical clinical appearance., Observation: We present three cases of seemingly malignant pigmented lesions on the foot, all arising in Japanese females. Clinically, the lesions are characterized by irregular borders and variegated pigmentation closely mimicking those of acral lentiginous melanoma in situ. However, the histologic findings revealed only focal slight melanocytic hyperplasia with minimal cytologic atypia along the basal layer. Despite the malignant clinical features, thorough histologic examination failed to disclose any area with significant melanocytic atypia or evidence of malignancy., Conclusions: To the best of our knowledge, similar lesions with the clinical appearance of melanoma in situ and completely lacking histologic evidence of malignancy have not been reported. We, therefore, prefer to designate these lesions as atypical melanosis of the foot to highlight the clinically apparent atypical findings and to distinguish them from malignant melanoma in situ of the foot.

Background and Design: Epidermolytic hyperkeratosis (EHK) is a rare autosomal dominant disorder of cornification. While different clinical presentations of EHK have been described, the distinctions have not been clear. We have examined 52 patients with EHK from 21 families in an effort to define and characterize the specific clinical features of this disorder., Results: We found that several features were useful for separating patients with EHK into clinical groups. The most distinctive characteristic was presence vs absence of severe palmoplantar hyperkeratosis. Twenty-nine patients in six families had this finding and were grouped into "PS types" (those with severe palm/sole hyperkeratosis). The remaining 23 patients (from 15 families) were classified as "NPS types" (those without severe palm/sole hyperkeratosis). We identified three distinct PS types and three distinct NPS types. The classification was always found to be consistent in all affected family members. In those families in which mutations were defined, keratin 1 mutations were identified in the PS types and keratin 10 mutations in the NPS types., Conclusions: We were able to classify our cohort of 52 patients with EHK from 21 families into distinct types. There was a correlation between presence or absence of severe palm/sole hyperkeratosis and the specific keratin involved.

Background: Reiter's syndrome is a disease characterized by crusted, scaling, acral and genital plaques; urethritis or cervicitis; and arthritis, which occur in genetically susceptible patients in response to any of many infections. This disease rarely occurs in women, and specific characterizations of vulvar and cervical lesions are rare., Observations: We describe a 39-year-old woman with a history of mucocutaneous candidiasis that was refractory to oral ketoconazole therapy. She presented with well-demarcated, erythematous, crusted plaques over the vulva, hands, and feet, as well as with cervical lesions and a history of conjunctivitis and iritis. Following the biopsy of characteristic skin lesions, recognition of systemic signs, and cultures that were negative for yeast, her condition was diagnosed as Reiter's syndrome., Conclusions: Reiter's syndrome of the vulva, vagina, and cervix may not be recognized because of its uncommon occurrence in women and the physician's consequent unfamiliarity with its clinical appearance in the genital area. This disease and pustular psoriasis share many common features and exist on a spectrum. A high index of suspicion and correlation of the many facets of the disease will better enable the clinician to make this diagnosis.

Fourteen cases of plantar epidermoid cyst were studied immunohistochemically and five of them were also studied electron microscopically for the presence of human papillomavirus. In eleven cases, the test results were positive for papillomavirus antigens, and in five of them, papillomaviruslike particles were observed by electron microscopy. Histological examination showed most of the cysts to contain intracytoplasmic eosinophilic bodies in cells of the cyst wall as well as the vacuolar structures in the keratinous mass within the cavity. These findings suggest an etiologic association between the papillomavirus and plantar epidermoid cysts. The "cystic papilloma" produced in rabbits by the Shope papillomavirus has been clearly demonstrated. We propose that human papillomaviruses play an important role in the genesis of plantar epidermoid cysts. This is the first report on the possible existence of a cystic papilloma in humans.