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BackgroundPort wine birthmarks (PWBs) are congenital capillary malformations. Vessel characteristics, such as diameter and depth, may impact presentation and outcomes. They can be imaged using dynamic optical coherence tomography, a high-resolution, noninvasive imaging method.PurposeWe conducted a cross-sectional observational study to measure invivo vascular characteristics as a function of PWB color.MethodsPatients undergoing treatment for PWB were recruited from 3 sites. PWBs were classified by color. Dynamic optical coherence tomography images with calculations were obtained.ResultsOne hundred eight patients were enrolled. Mean age correlated with PWB color, with birthmarks being lighter in younger patients and darker in older patients (P&nbsp

Background Multiple studies have reported on dermoscopic structures in basal cell carcinoma (BCC) and its subtypes, with varying results. Objective To systematically review the prevalence of dermoscopic structures in BCC and its subtypes. Methods Databases and reference lists were searched for relevant trials according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were assessed for the relative proportion of BCC dermoscopic features. Random-effects models were used to estimate summary effect sizes. Results Included were 31 studies consisting of 5950 BCCs. The most common dermoscopic features seen in BCC were arborizing vessels (59%), shiny white structures (49%), and large blue-grey ovoid nests (34%). Arborizing vessels, ulceration, and blue-grey ovoid nests and globules were most common in nodular BCC; short-fine telangiectasia, multiple small erosions, and leaf-like, spoke wheel and concentric structures in superficial BCC; porcelain white areas and arborizing vessels in morpheaform BCC; and arborizing vessels and ulceration in infiltrative BCC. Limitations Studies had significant heterogeneity. Studies reporting BCC histopathologic subtypes did not provide clinical data on pigmentation of lesions. Conclusion In addition to arborizing vessels, shiny white structures are a common feature of BCC. A constellation of dermoscopic features may aid in differentiating between BCC histopathologic subtypes.

Background Nail matrix histopathologic examination is still the criterion standard to diagnose longitudinal melanonychia (LM). Objective To introduce modified shave surgery combined with the nail window technique for managing LM and evaluate the postoperative outcome of the procedure. Methods We retrospectively reviewed the medical records of 67 patients with LM who underwent shave surgery combined with the longitudinal-strip nail window technique at our institution from March 2015 to June 2018. Results Pathologic diagnosis was accessible in all cases, and 60 cases were assessable for the postoperative outcomes. A total of 45 cases (75.0%) had no postoperative nail dystrophy, and recurrence of nail pigmentation was found in only 8 cases (13.3%). Limitations This was a retrospective study. Conclusion Modified shave surgery combined with the nail window technique is the preferable management for LM cases, with limited postoperative nail dystrophy and recurrence of pigmentation.

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The study protocol was reviewed and approved by National Taiwan University Hospital (201605118RINB); National Taiwan University Hospital, Hsin-Chu branch (103-082-E); Chang Gung Memorial Hospital, Linkou and Taipei branches (104-7463B, 104-7317B), and China Medical University Hospital (CMUH107-REC2-160). This work has not been published previously and is not under consideration for publication elsewhere, its publication has been approved by all authors, and if accepted, it will not be published elsewhere, including electronically in the same form, in English or in any other language, without the written consent of the copyright-holder.

Background Cutaneous eruptions in neonatal lupus erythematosus (NLE) are thought to be self-resolving. Limited literature suggests cutaneous changes may persist. Objective To characterize cutaneous residua in NLE and identify predictors for their development. Methods A retrospective cohort study of patients with cutaneous NLE born between January 1980 and May 2017 was performed. Primary outcome was the proportion of patients with cutaneous residua. Secondary outcomes included associations/predictors of sequelae. Results At the last follow-up, at a mean age of 4 years (range, 0.5-18.7 years), 34% of 106 patients had cutaneous sequelae, 13% had telangiectasia, 17% had dyspigmentation, and 9% had atrophic scarring. Scarring at the last follow-up was significantly associated with the presence of skin lesions at birth (P Limitations This study was limited by the retrospective design, short follow-up duration in a subset of patients, and small sample size. Conclusion Cutaneous NLE can exhibit long-term cutaneous residua. These findings underlie the importance of accurate diagnosis, long-term monitoring, and appropriate counseling.

Acquired disorders with depigmentation are commonly encountered by dermatologists and present with a wide differential diagnosis. Vitiligo, the most common disorder of acquired depigmentation, is characterized by well-defined depigmented macules and patches. Other conditions, such as chemical leukoderma, can present with similar findings, and are often easily mistaken for vitiligo. Key clinical features can help differentiate between acquired disorders of depigmentation. The first article in this continuing medical education series focuses on conditions with a vitiligo-like phenotype. Early recognition and adequate treatment of these conditions is critical in providing appropriate prognostication and treatment.

Visible vascular and pigmentary conditions have a negative impact on children's and adolescents' quality of life (QoL). We sought to quantitate the effect of visible skin anomalies and their camouflage on QoL.In all, 41 patients, 5 years of age and older, were taught to use cosmetic camouflage. QoL was assessed using the Children's Dermatology Life Quality Index (CDLQI) before and 6 months after the intervention. Satisfaction and use were evaluated after 1 and 6 months.Baseline QoL scores revealed a small impact of vascular anomalies (CDLQI score 4.2) and a small to moderate effect of pigmentary anomalies (CDLQI score 6.1). Six months after the intervention, QoL improved in the study population as a whole (CDLQI score 5.1 vs 2.1, P.001), with significant improvements documented for facial lesions and vascular malformations. Cosmetic camouflage was well tolerated and patients with pigmentary anomalies were more likely to continue using the products.Limitations include small study population, few male patients, cultural influences not addressed, and limited range of conditions.Children and teenagers with visible vascular and pigmentary anomalies experience an impairment of QoL that is abrogated by introduction to use of cosmetic camouflage.

Background Children with multiple cafe-au-lait macules (CALMs) may be followed for years before a second National Institutes of Health clinical criterion of neurofibromatosis type 1 (NF1) develops to confirm the diagnosis. Objective We sought to assess the prevalence of nevus anemicus (NA) in NF1 and its association with neuro-ophthalmologic complications. Methods This was a prospective multicenter case-control study of 210 consecutive patients with multiple CALMs. Patients with NF1 were matched for age, sex, and center with control subjects. We documented the number, location, and morphologic appearance of NA; dermatologic features of NF1; magnetic resonance imaging results; and family history. Results In all, 77 (51%) patients with NF1 had NA compared with 6 (2%) control subjects. NA was not detected in 26 patients with other genodermatoses associated with CALMs. Patients with NF1 and NA were younger than those without NA (median age: 17 years) ( P = .002). NA was mostly localized to the upper anterior aspect of the chest. NA was not significantly linked with other clinical manifestations of NF1, including optic glioma and unidentified bright objects. Limitations A potential referral bias associated with tertiary care centers is a limitation. Conclusions NA appears to have a high prevalence and specificity in NF1 and might serve as a marker for NF1 in children with multiple CALMs.

Although traditionally known as "white forelock," poliosis circumscripta, defined as a localized patch of white hair in a group of hair follicles, can involve any hairy area on the body including the scalp, eyebrows, and eyelashes. Microscopically, poliosis demonstrates either decreased or absent melanin and/or melanocytes in the hair bulbs of the affected hair follicles. Classically, poliosis is known to occur in the setting of several genetic syndromes including piebaldism, Waardenburg, and tuberous sclerosis. In addition, poliosis has been described in association with various acquired conditions. These include inflammatory conditions, benign and malignant neoplastic entities that are mainly melanocytic, medications, and others. In this review, we aim to describe the different conditions where poliosis may be encountered, with the aim of helping the clinician to better evaluate any patient presenting with poliosis.

Background Solitary pink lesions often manifest nondescript clinical and dermatoscopic primary morphologic features. The differential diagnosis for pink lesions tends, therefore, to be broad, ranging from inflammatory processes to malignancy. In vivo reflectance confocal microscopy (RCM) may help in the evaluation of pink lesions. Objective We sought to demonstrate the use of RCM as an adjunct to the bedside diagnosis of pink lesions. Methods We describe a series of patients with clinically and dermatoscopically equivocal pink lesions for which RCM examination allowed for a rapid and accurate diagnosis. All lesions were excised for histopathologic evaluation. Integrating the findings in the case series with a literature review, we present RCM diagnostic criteria for pink lesions. Results Lesions included basal cell carcinoma, squamous cell carcinoma, amelanotic melanoma, and inflamed seborrheic keratosis. RCM shows distinctive findings for each diagnostic entity when stratified by anatomic level into suprabasal epidermis, dermoepidermal junction, and papillary dermis. In the cases presented RCM allowed for a rapid and accurate noninvasive diagnosis. Limitations The study is descriptive and does not test accuracy of RCM criteria in a prospective series of pink lesions. Conclusion RCM may add useful diagnostic features to the clinical evaluation of solitary pink lesions.

We report mucosal melanoma of the upper lip in a patient affected by the Laugier-Hunziker disease. Using dermatoscopy, two distinct parts were identified in the same mucosal area: nodular (malignant) and macular (benign). A complete surgical excision was performed and the patient has been free of disease for 16 months.

"Xanthoderma" is a term that describes a yellow to orange macular discoloration of the skin. The cause of this finding ranges from benign to potentially life-threatening disease. To date the literature fails to comprehensively review the various causes and workup of this clinical manifestation. This article discusses the etiopathogenesis of xanthoderma and suggests a novel diagnostic algorithm for the clinician to use in the initial evaluation of this condition.

Hyperpigmentation associated with prolonged minocycline use is well documented. The histopathology of cutaneous minocycline pigment is characterized by deposition of brown/black, Fontana-Masson, and Perls' positive granules deposited along elastic fibers in the papillary dermis and occurring within macrophages along vessels and eccrine units in the dermis. The subcutis may also be involved; however, the specific subcutaneous findings associated with minocycline hyperpigmentation have not been well established. We present the histopathologic findings of 4 cases of minocycline hyperpigmentation with subcutaneous involvement. Green-gray, flocculent, nonrefractile globules within macrophages were found in the subcutis of all patients. Two of 4 cases exhibited lipomembraneous changes that were also associated with pigment. These distinctive findings may provide additional clues to enable a diagnosis of drug-induced hyperpigmentation to be offered, even in the absence of a clear clinical history.

Longitudinal melanonychia (LM) may represent nail matrix melanocyte activation (defined as a normal number of melanocytes with increased production of melanin), benign hyperplasia, or melanoma, in addition to multiple nonmelanocyte pathologies, including hemorrhage and infection. This article details an algorithmic approach to LM, including a careful history and physical examination, dermoscopy, and ability to sample the matrix using 3 biopsy techniques, a 3-mm punch excision, a lateral longitudinal excision, and a matrix shave biopsy. Facility with all 3 techniques will allow the physician to procure appropriate nail matrix specimens for diagnosis.

Longitudinal pigmentation of the nail is very common. The differential diagnosis varies from subungual hematoma, to a fungal infection, to a melanocytic lesion (lentigo, nevus melanoma, etc.) to others. Often, dermatologists do not feel at ease with these pathologies and management is often not clear. In many cases, a biopsy is not helpful because an inadequate technique was chosen. The use of noninvasive techniques such as dermoscopy has been described to be useful for the preoperative evaluation and the management decision. Using these technique, one will be able to reduce the number of unnecessary surgeries and to choose the most adequate biopsy technique. In this article, we will review the management, including diagnosis as well as differential diagnosis of nail pigmentations and propose a new algorithm for the non invasive diagnosis of nail pigmentation.

We describe the case of a 13-year-old boy with stage I hypopigmented mycosis fungoides in whom minimal residual disease was detected with T-cell receptor gamma-polymerase chain reaction after the disease was in complete clinical remission. We further cloned and sequenced the T-cell receptor gamma-polymerase chain reaction product of the lesion in remission and found that the original T-cell clone still existed in decreased amounts. The patient was followed up for 3 1/2 years without any new lesions developing. The clinical significance of this residual malignant T-cell clone in mycosis fungoides remains to be elucidated.

Visual assessment remains one of the "gold standard" methods of assessing skin color and a number of tools are currently available to reduce the interobserver variability. Ultraviolet light examination remains a mainstay of the assessment of pigmentary disorders, while polarized light photography is useful for the appraisal of dermal changes, in particular those related to vascularity. With the introduction of modern instruments, reflectance spectroscopy using tristimulus colorimeters or narrowband spectrophotometers provides a convenient, objective, and reproducible methodology for the evaluation of pigmentation and skin color. In vivo confocal scanning laser microscopy is a powerful technique for the examination of pigmented lesions, which shows promise in the detection and diagnosis of early melanoma. Dermoscopy is also useful for the differential diagnosis of benign melanocytic lesions and melanoma, and its use has been shown to significantly improve diagnostic accuracy.

Aquagenic syringeal acrokeratoderma is a rare acquired condition characterized by painful symmetric swelling and hypopigmentation of the palms and lateral fingers, which develops after brief exposure to water. Histopathologic examination suggests that an aberration in the eccrine sweat gland apparatus may be the underlying cause of this condition. The "hand-in-the-bucket sign," in which patients arrive in their physician's office with their hand in a bucket of water to more readily demonstrate their lesions, is such a common presentation that it almost can be regarded as pathognomonic. All 12 cases reported to date have been in young females. We report a case of aquagenic syringeal acrokeratoderma in a male with unique histologic findings.

Pigmented actinic keratosis and melanoma may exhibit overlapping clinical features, thus representing a diagnostic challenge for dermatologists. Although the differentiation between these two entities is traditionally done by histopathology, dermoscopy has been utilized as a useful additional aid for improving the clinical diagnostic accuracy of such pigmented skin lesions. We report the clinical and dermoscopic features of two pigmented actinic keratoses to discuss the difficulties in their preoperative differential diagnosis.

We describe three generations of a family expressing progressive mottled hypopigmentation and hyperpigmentation on the non-exposed parts of the body from childhood to adult life. At birth, they all had epidermal blistering of the distal extremities. Although the palmoplantar warty keratoses could be related to the bulla formation, the pigmentary changes could not. Otherwise, there were no systemic disorders. Genetic diseases with spotty epidermal hypopigmentation and hyperpigmentation form a long list and the diagnosis is not always easy. Although different diagnostically, the condition resembled an entity described by Siemens in 1922 and epidermolysis bullosa with mottled pigmentation. Molecular biological investigation would be required to characterize the phenotype of this entity, which apparently was a mutation occurring in one family for three generations.

Winchester syndrome is a rare genetic disorder, one of the inherited osteolysis disorders which are a group of diseases characterized by destruction and resorption of affected bones with consequent skeletal deformities and functional impairment. The syndrome is characterized by dissolution of carpal and tarsal bones with generalized osteoporosis, progressive joint contractures, short stature, peripheral corneal opacities, and coarse facial features, though there is variability within the clinical features. Phenotypic heterogeneity of cutaneous features are also reported to date of diffusely thickened leathery skin, hypertrichosis, patches of hyperpigmented, hypertrichotic leathery skin in annular or linear distribution, widespread acne, subcutaneous nodules, and gingival hypertrophy. We describe widespread progressive multilayered symmetrical restrictive banding of the skin developing in a woman with Winchester syndrome during her mid-twenties.

Laser therapy has been a mainstay of dermatologic therapy for more than a decade. However, until recently, most published literature focused on the Caucasian patient. The face of the aesthetic patient is changing to be more representative of the ethnic diversity of the population of the United States. It is imperative that the dermatologic surgeon not only have an understanding of the concerns of the aesthetic patient but also have an awareness of the unique needs of those with darker skin.

With the changing demographics of the US population, there is a need to understand the variety of dermatologic disorders that manifest in ethnic groups of non-Caucasian skin types. This article provides a review of presentations and current treatments of several common dermatologic diagnoses in black, Hispanic, and Asian racial groups and compares them with the presentations in Caucasian skin. The specific diagnoses discussed in the different racial groups include acne; pigmentary disorders such as postinflammatory hyperpigmentation and hypopigmentation, vitiligo, and melasma; and photoaging. Because the majority of the world's population already consists of people with pigmented skin and the population within the United States is approximately one-third non-Caucasian, physicians who practice in the field of dermatology today need a thorough understanding of non-Caucasian dermatoses.

Background The discovery of signaling networks that drive oncogenic processes has led to the development of targeted anticancer agents. The burden of pigmentary adverse events from these drugs is unknown. Objective To conduct a systematic review and meta-analysis of published clinical trials and determine the incidence and risk of development of targeted therapy–induced pigmentary changes. Methods A comprehensive search was conducted to identify studies reporting targeted therapy–induced pigmentary changes. The incidence and relative risk were calculated. Case reports and series were reviewed to understand clinical characteristics. Results A total of 8052 patients from 36 clinical trials were included. The calculated overall incidences of targeted cancer therapy–induced all-grade pigmentary changes in the skin and hair were 17.7% (95% confidence interval [CI], 11.9-25.4) and 21.5% (95% CI, 14.9-30.1), respectively. The relative risk of all-grade pigmentary changes of skin and hair were 93.7 (95% CI, 5.86-1497.164) and 20.1 (95% CI, 8.35-48.248). Across 53 case reports/series (N = 75 patients), epidermal growth factor receptor and breakpoint cluster region–abelson inhibitors were the most common offending agents. Limitations Potential under-reporting and variability in oncologists reporting these events. Conclusion There is a significant risk of development of pigmentary changes during treatment with targeted anticancer therapies. Appropriate counseling and management are critical to minimize psychosocial impairment and deterioration in quality of life.

Background The current literature suggests that approximately 5% to 10% of melanonychia striata cases in adults are the result of subungual melanoma. Objective We sought to evaluate the clinical and histopathologic features and to determine the outcomes and causes of melanonychia striata in a cohort of children. Methods We assessed 30 childhood cases of melanonychia striata for features typically associated with melanoma such as Hutchinson sign, width of the pigmented band, evolution, color, and nail dystrophy. We assessed the histopathology of lesional biopsy specimens, including melanocyte counts and suprabasal movement of melanocytes. Clinical follow-up information was reviewed when available. Results Histopathologic diagnoses included subungual lentigo in 20 cases, subungual nevus in 5 cases, and atypical melanocytic hyperplasia in 5 cases. Although a number of cases exhibited worrisome clinical or histopathologic features, none showed evidence of aggressive behavior or warranted a diagnosis of melanoma. Limitations The sample size and follow-up times are limited. Conclusions Melanonychia striata is typically associated with benign stable melanocytic proliferations in childhood. The overwhelming majority of cases can be managed conservatively. Biopsy is required in select cases.

Background Many researchers have been concerned about the association of hair graying with systemic diseases. However, the common factors associated with hair graying and systemic diseases have not been elucidated. Objective This study aimed to identify risk factors for premature hair graying (PHG) in young men. Methods We conducted a cross-sectional study using questionnaires in young men. After a pilot study that included 1069 men, we surveyed 6390 men younger than 30 years about their gray hair status and various socioclinical characteristics. Results The age of participants in the main survey was 20.2 ± 1.3 years (mean ± SD). Of the 6390 participants, 1618 (25.3%) presented with PHG. Family history of PHG (odds ratio [OR], 12.82), obesity (OR, 2.61), and >5 pack-years history of smoking (OR, 1.61) were significantly associated with PHG. In the multivariate analysis, family history of PHG (OR, 2.63) and obesity (OR, 2.22) correlated with the severity of PHG. Limitations Owing to the use of questionnaires, the possibility of recall bias exists. Women were not evaluated in this study. Conclusion Smoking, family history of PHG, and obesity are important factors associated with PHG.

Alcohol consumption and abuse can have a variety of cutaneous manifestations. In addition to the well-recognized stigmata of the chronic alcoholic patient, even early abuse can result in distinctive skin changes or exacerbate existing cutaneous disorders. An accurate history of alcohol intake will facilitate recognition of these alcohol-induced cutaneous disorders and treatment resistance of dermatoses such as psoriasis as well as help decrease morbidity in surgical procedures. Familiarization with the spectrum of cutaneous manifestations of alcohol abuse and alcoholic liver disease can also allow for early detection and treatment in an attempt to minimize the medical consequences. We review the medical literature and discuss the spectrum of dermatologic disease associated with the use and abuse of alcohol. (J Am Acad Dermatol 2000;43:1-16.) Learning objective: At the conclusion of this learning activity, participants should be familiar with the cutaneous manifestations of alcohol abuse and alcoholic liver disease.

Background: Cutaneous neonatal lupus erythematosus (NLE) is an uncommon disease described mainly through isolated case reports. Objective: Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE. Methods: This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. Results: Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital "owl-eye" or "eye mask" facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 neonatal lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring. Conclusion: Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.(J Am Acad Dermatol 1999;40:675-81.)

Epiluminescence microscopy (ELM) significantly increases the early diagnosis of pigmented skin lesions (PSL) using established criteria and pattern analysis. The ABCD rule for dermatoscopy (ie, ELM) provides a simplified approach to the interpretation of ELM images on the basis of asymmetry (A), border (B), color (C), and dermatoscopic structure (D).We set out to determine whether the diagnostic accuracy of the ABCD scoring algorithm can be significantly improved by incorporating information about morphologic changes of the lesion observed and provided by the patient.We prospectively collected 356 small pigmented skin lesions (1 cm) including 73 (20.5%) melanomas. Before excision all patients were asked whether the lesion had changed in size, color, or shape within the last year or whether they experienced any sign of ulceration or spontaneous bleeding. ELM images of the lesions were evaluated according to the ABCD rule for dermatoscopy to yield a semiquantitative score. Accuracy of diagnosis was evaluated in terms of sensitivity, specificity, and area under receiver operating characteristic curves (AUC).The frequency of reported changes was significantly higher for melanomas than benign PSL (65.8% vs 29.7%, P.001). In a multivariate model morphologic change was a significant independent predictor of malignancy (odds ratio = 3.17, 95% confidence interval [CI]: 1.96 to 5.14, P.001). The mean final score achieved when using the enhanced ABCD-E criteria including morphologic change (E) was significantly higher for melanomas (5.7, 95% CI: 5.3 to 6.0) than benign PSL (2.9, 95% CI: 2.8 to 3.1, P.001). Diagnostic accuracy was significantly higher when the lesions were evaluated by the enhanced ABCD-E criteria as compared with the standard ABCD score (AUC(ABCD) = 0.87 vs AUC(ABCD-E) = 0.90; P = .006).Information about morphologic changes of PSL as reported by the patient is a useful extension of the ABCD rule for dermatoscopy.

Two patients had been taking long-term tricyclic antidepressant therapy. Each developed a blue to slate-gray hyperpigmentation in sun-exposed areas. On histologic examination there were refractile golden brown granules free in the dermis along collagen bundles. Similar pigment was present in macrophages and along the basement membrane zone. The granules stained for melanin, but not for iron, and were bleached by the permanganate method. Electron microscopy showed varying size and shaped electron-dense granules within lysosomes and free in the dermis, which, in unstained sections, showed a less dense peripheral halo. This peripheral halo was also evident on light microscopy. Energy dispersive spectroscopy showed these granules to be rich in copper and sulfur (elements present in tyrosinase and pheomelanin, respectively). We believe that this represents a drug-melanosome complex, which is most likely caused by chronic photoactivation. (J Am Acad Dermatol 1999;40:290-3.)

Mitochondrial DNA syndromes are an emerging class of diseases that can present at any age. Clinical findings are legion and may include renal tubulopathy, growth retardation, myopathy, seizures, and ophthalmoplegia. Mitochondrial DNA syndromes have presented with symmetric cervical lipomas, poikiloderma, and anhidrosis. We describe a child with a novel mitochondrial DNA syndrome who had poikiloderma on sun-exposed areas. We also reviewed 274 patients with mitochondrial DNA disorders for skin findings. Symmetric cervical lipomas were consistently associated with myoclonic epilepsy as part of 1 syndrome. With the exception of lipomas, skin findings were reported in 16 patients. (J Am Acad Dermatol 1998;39:819-23.)