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Spontaneous appearance of hyperpigmented macules on chronic vitiligo lesions is a very rare phenomenon, which is described as eruptive lentiginosis. We describe the case of a patient with chronic non-segmental generalized vitiligo who presented with a sudden onset of hyperpigmented macules on depigmented areas of the face. A biopsy showed pigmented basal keratinocytes in the interfollicular epidermis, and immunohisochemistry with anti-SOX10 antibodies showed nuclei of single melanocytes. This case shows that even long-standing depigmented vitiligo lesions may contain functional melanocytes or their precursors.

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Background/purpose: The aim of this study was to measure lentigines' pigmentation over a long period of time and evaluate if summer over-pigmentation can be avoided by the use a SPF30 day skin cream., Methods: Seventeen healthy female volunteers aged 50 and over and presenting lentigines participated in the study from spring to summer. Throughout the study, all subjects applied a SPF30 daily skin cream to only one hand. Color measurements of the target lesions were performed with a chromameter and with a color-calibrated camera. Target lesions were also imaged with in vivo reflectance confocal microscopy (RCM). A specific procedure for re-registering the images was developed to ensure that the same papillae were measured over time., Results: Both color measurement methods, chromametry and color-calibrated camera, showed that lentigines treated over time with the SPF30 day skin cream were significantly lighter than the non-treated lentigines. The RCM images showed a decrease in the papillary contrast for the treated lentigines., Conclusion: This study shows that this over-pigmentation can be avoided using a SPF30 day skin cream. Moreover, we have demonstrated that very fine re-registration of the RCM images is possible and ensures a more robust analysis., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Speckled lentiginous nevus syndrome has been described in individuals with a speckled lentiginous nevus with rare associated neurologic deficits. Because speckled lentiginous nevus syndrome almost always affects adults, it is not typically considered when evaluating children. We present the first reported case of speckled lentiginous nevus syndrome presenting in a young child with muscle atrophy and motor deficits affecting muscles along the same distribution as the speckled lentiginous nevus., (© 2018 Wiley Periodicals, Inc.)

Porokeratosis, a disorder of keratinisation, is clinically characterized by the presence of annular plaques with a surrounding keratotic ridge. Clinical variants include linear, disseminated superficial actinic, verrucous/hypertrophic, disseminated eruptive, palmoplantar and porokeratosis of Mibelli (one or two typical plaques with atrophic centre and guttered keratotic rim). All of these subtypes share the histological feature of a cornoid lamella, characterized by a column of 'stacked' parakeratosis with focal absence of the granular layer, and dysmaturation (prematurely keratinised cells in the upper spinous layer). In recent years, a proposed new subtype, follicular porokeratosis (FP_, has been described, in which the cornoid lamella are exclusively located in the follicular ostia. We present four new cases that showed typical histological features of FP., (© 2017 British Association of Dermatologists.)

Background: Familial lentiginosis syndromes are characterized by a wide array of manifestations resulting from activation of molecular pathways which control growth, proliferation, and differentiation of a broad range of tissues. Familial gastrointestinal stromal tumors (GISTs) are often accompanied by additional features like hyperpigmentation, mastocytosis, and dysphagia. They have been described with mutations in c-kit (most commonly), platelet-derived growth factor receptor A, neurofibromatosis-1, and succinate dehydrogenase genes., Materials and Methods: We report on molecular characterization and tumor histopathology of two siblings in whom lentigines and café-au-lait macules were present along with multifocal GIST. Immuhistochemical analysis of CD34 and CD117 was performed on GIST biopsy samples from both siblings, while c-kit mutational analysis was done by PCR and direct sequencing on DNA from peripheral blood leukocytes of all family members and from paraffin-embedded gastric biopsy specimens of affected siblings., Results: Histopathology revealed positive expression of CD117 and CD34. Mutational analysis showed the germline c.1676T>C mutation in c-kit exon 11, (p.(Val559Ala)), in the peripheral blood of both siblings and a second exon 11 mutation, c.1669T>A (p.(Trp557Arg)) in the tumor biopsy of one of them. Initiation of imatinib treatment resulted in striking resolution of their hyperpigmentation and a stable gastrointestinal disease in one of them., Conclusions: A c-kit mutational test in familial GISTs is indicated before initiation of imatinib therapy, as it can help predict tumor response to treatment., (© 2017 The International Society of Dermatology.)

Partial unilateral lentiginosis (PUL) is a rare pigmentation disorder characterized by numerous lentigines with sharp margins in the midline in one or more dermatomes. Its segmental pattern suggests that this presentation accompanied by café-au-lait spots, Lisch nodule or neurofibromas has a close relationship with mosaic neurofibromatosis type 1 or segmental neurofibromatosis (NF) in particular. In a group of 16 patients with PUL, who presented at the dermatology outpatient clinic between 1998 and 2015, an examination was made of consanguineous marriage in the family history, the presence of a similar lesion or NF in first-degree relatives, neurofibroma in the physical examination, the involvement pattern, axillary/inguinal freckling and the presence and number of café-au-lait spots. The ophthalmological examination investigated Lisch nodule and optic glioma. The skeletal system was examined for NF involvement. Of 16 patients, 13 (81.2%) were female and three (18.8%) were male with a mean age of 31.19 years (range, 15-48). There was no family history of PUL in any case. Consanguineous marriage was absent in 15 patients (93.8%). While there were accompanying café-au-lait spots in three patients (18.8%). Lisch nodule was an accompanying finding in three patients (18.8%). Axillary freckling was detected in four (25%) patients. Neurofibroma was found in only one patient. Although café-au-lait spots, axillary freckling, neurofibroma and Lisch nodule were present in a small number of the patients, the presence of the findings may be considered to be specific to NF suggests that PUL is a variant of mosaic NF-1. Genetic studies will help to further elucidate this subject., (© 2016 Japanese Dermatological Association.)

Multiple lentigines confined to psoriatic plaques is a rare entity, which is more frequently recognized after the use of systemic biologic agents for psoriasis therapy. Although this phenomenon was previously accepted as a postinflammatory reaction, recent observations suggest psoriasis-related cytokines and their efficient supression by biologic agents strongly associate with melanogenesis and melanocytic proliferation. Hereby, we report a patient who developed multiple lentigines arising in resolved psoriatic plaques induced by infliximab and review similar cases reported in the literature induced after biologic treatments of psoriasis.

Background: Lentigines are a common pigmentary disorder in adults and in patients treated by psoralen and ultraviolet A (PUVA) radiation. Their appearance following treatment with narrow-band ultraviolet B (NB-UVB) radiation has been reported in only two patients., Objective: To describe the clinical and histological features of NB-UVB-induced lentigines their relation to dosimetry and the course of the eruption in patients with mycosis fungoides (MF)., Methods: The files of all patients with MF treated in our department in 2003-2010 were searched to identify those in whom lentigines appeared following monotherapy with NB-UVB radiation., Results: Of the 73 patients with early stage MF identified, 10 met the study criteria. Lentigines were detected in skin previously involved by MF in seven patients, and in both involved and uninvolved skin in three patients. They appeared during therapy in three patients, after a mean of 56 exposures (range 50-61), and several months (mean 7.8) following completion of treatment in seven patients, after a mean of 69 exposures (range 32-157). Histopathological study of lesions from five patients revealed basal hyperpigmentation relative to adjacent normal-looking skin. Two lesions had a slight increased number of normal-looking melanocytes on immunohistochemical staining with melanoma cocktail. One lesion had elongated rete ridges. The lesions persisted throughout follow-up (mean 26.7 months) in 8 patients., Conclusions: Patients with MF treated with NB-UVB may acquire lentigines. As opposed to PUVA-induced lentigines which are a known common side-effect of long-term treatment, NB-UVB-induced lentigines are uncommon but appear earlier, even after a few months of treatment., (© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.)

Background: Knowledge of the histopathology of melasma is a prerequisite for understanding its pathogenesis. However, the histopathological characteristics of male melasma are not well characterized., Objective: We sought to investigate the histopathological characteristics of melasma in men compared with those of women with melasma and solar lentigo., Methods: Biopsy specimens were obtained from both the lesional skin and the adjacent nonlesional skin in 8 men with melasma, 10 women with melasma, and 5 men and women each with solar lentigo. The samples were stained using Fontana-Masson and Verhoeff-van Gieson. Immunohistochemistry for melanocytes, the estrogen receptor, progesterone receptor, factor VIIIa-related antigen, stem cell factor, and c-kit was performed., Results: Increased vascularity was found in the lesion of male melasma. The lesion to nonlesion ratio of the vessel area was increased in male melasma compared with lentigo groups. In the lesion of male melasma, there was a significant increase of stem cell factor and c-kit expression. In addition, the lesion to nonlesion ratio of stem cell factor was increased in male melasma compared with female melasma and lentigo groups. The lesion to nonlesion ratio of c-kit was also increased in male melasma compared with lentigo groups., Limitations: This study did not include clinical data regarding social habits and was not confirmed by other molecular techniques., Conclusion: The results suggest that chronic ultraviolet radiation associated with signaling of paracrine cytokines plays an important role in the mechanism associated with hyperpigmentation in male melasma., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Mal de Meleda (MDM) is palmoplantar erythrokeratoderma with an autosomal recessive inheritance and is caused by a mutation in the gene encoding SLURP-1 (lymphocyte antigen 6/urokinase-type plasminogen activator receptor related protein-1). SLURP-1 is an allosteric agonist to the nicotinic acetylcholine receptor (nAchR) and it regulates epidermal homeostasis. In addition, murine studies have shown that nAchR signalling is important for the regulation of T-cell function. Among the family members, patients with the homozygous SLURP1 (previously known as ARS component B) mutation are prone to melanoma and viral infection, which might link to defective T-cell function as well as a derangement of epidermal homeostasis., Objectives: To investigate the association of the SLURP1 gene mutation with T-cell activation in a Taiwanese family with MDM. To test that SLURP-1 is essential for T-cell activation., Methods: Human peripheral blood mononuclear cells (PBMCs) were isolated from a Taiwanese MDM family bearing the G to A substitution in nucleotide 256 in the SLURP1 gene, corresponding to a glycine to arginine substitution at amino acid 86 (G86R) in the SLURP-1 protein. PBMCs from homozygotes and wild-type controls were stimulated with anti-CD3/anti-CD28 antibodies and the level of T-cell activation was determined by the stimulation index., Results: PBMCs with the heterozygous and homozygous SLURP-1 G86R mutation had defective T-cell activation. This was restored by the addition of 0·5 μg mL(-1) recombinant human SLURP-1 protein., Conclusions: Patients with MDM with the homozygous SLURP-1 G86R mutation may have an impaired T-cell activation. The presence of wild-type SLURP-1 is essential for normal T-cell activation., (© 2010 The Authors. BJD © 2010 British Association of Dermatologists 2010.)

Gynecomastia involves enlargement of the male breast secondary to the proliferation of mammary ductules. It is seen in a number of conditions such as cirrhosis, chronic renal failure, Klinefelter syndrome, and leprosy. Recognition of the cutaneous manifestations of these conditions can help in identifying the correct etiology of the gynecomastia.

Background: Gastrointestinal stromal tumors (GISTs) harbor gain-of-function mutations of the c-kit tyrosine kinase receptor. Imatinib mesylate is an inhibitor of c-kit and is indicated in the treatment of chronic myeloid leukemia and GISTs. Reported adverse effects of imatinib include hypopigmentation, depigmentation, and hyperpigmentation. Although the exact mechanism by which these occur is unclear, it is likely that inhibition of c-kit leads to downstream inhibition of the tyrosinase gene promoter and thus to inhibition of pigment production., Observations: A 45-year-old woman with a history of multiple dysplastic nevi and lentigines was diagnosed as having familial GIST syndrome. Treatment with imatinib mesylate was started in an attempt to decrease the tumor load. Three months after treatment initiation, the patient noted a decrease in the number of pigmented lesions, lightening of the skin in her genital area, and graying of her terminal hair., Conclusions: The potential association between a specific genetic mutation and pigmentation changes secondary to imatinib therapy may account for the variety in presentation of this potential side effect. Further genetic studies paired with melanocyte-specific or c-kit-specific stains of affected tissue are warranted to better understand the relationship between the genetic mutation and the effect of imatinib on pigmentation.

Ectopic thymic tissue within the thyroid gland is rare. Patients with a complex of myxomas, spotty skin pigmentation, and endocrine overactivity, collectively known as Carney complex (CNC), have a predisposition towards the development of thyroid abnormalities, but there are no reports of thymic defects in CNC. We present the case of a 12-year-old boy with CNC and a growing thyroid nodule. The patient had the c.682 C > T (Arg228X) pathogenic PRKAR1A mutation. Hemithyroidectomy for a Hürthle cell adenoma led to the confirmation of distinct intrathyroidal ectopic thymic tissue. Thymic abnormalities have not been previously reported in CNC.

Background: Many studies have investigated the Mediterranean diet as a risk factor for cancer, none of which has included cutaneous melanoma. The latter is usually fatal, rendering knowledge about prevention extremely important. We assessed the role of some food components of the Mediterranean diet and cutaneous melanoma., Methods: A hospital-based case-control study was conducted in the inpatient wards of IDI-San Carlo Rome, Italy including 304 incident cases of cutaneous melanoma and 305 controls, frequency matched to cases. Information on socio-demographic characteristics, medical history, smoking, sun exposure, pigmentary characteristics and diet was collected. Logistic regression was the method used to estimated odds ratio and 95% CIs., Results: After careful control for several sun exposure and pigmentary characteristics, we found a protective effect for weekly consumption of fish (OR, 0.65, 95%CI = 0.43-0.97), shellfish (OR, 0.53, 95%CI = 0.31-0.89), fish rich in n-3 fatty acids (OR, 0.52, 95%CI = 0.34-0.78), daily tea drinking (OR, 0.42, 95%CI, 0.18-0.95; P(trend) = 0.025) and high consumption of vegetables (OR, 0.50, 95%CI = 0.31-0.80, P(trend) = 0.005) in particular carrots, cruciferous and leafy vegetables and fruits (OR, 0.54, 95%CI =0.33-0.86, P(trend) = 0.013), in particular citrus fruits. No association was found for alcohol consumption and any other food items., Conclusion: Overall, our findings suggest that some dietary factors present in the Mediterranean diet might protect from cutaneous melanoma.

Agminated lentiginosis is an unusual pigmentary disorder, characterized by numerous lentigines grouped within an area of normal skin. The pigmented macules are often in a segmental distribution within a sharp demarcation at the midline. We encountered a 28-year-old woman with an unusual combination of multiple café-au-lait macules and diffuse numerous lentigines involving the right cheek and ipsilateral upper thorax with sharp demarcation at the midline. The multiple lentigines extended bilaterally over the back in a peppered distribution. There were 21 café-au-lait macules on both arms, and the trunk and buttocks; however, there were no Lisch nodules, neurofibromas, or any other clinical manifestations for neurofibromatosis. Histopathology of a macule revealed the features of lentigo.

Context: Carney complex (CNC), a familial multiple neoplasm syndrome with dominant autosomal transmission, is characterized by tumors of the heart, skin, endocrine and peripheral nervous system, and also cutaneous lentiginosis. This is a rare syndrome and its main endocrine manifestation, primary pigmented nodular adrenal disease (PPNAD), is an uncommon cause of adrenocorticotropic hormone-independent Cushing's syndrome., Case Report: We report the case of a 20-year-old patient with a history of weight gain, hirsutism, acne, secondary amenorrhea and facial lentiginosis. Following the diagnosing of CNC and PPNAD, the patient underwent laparoscopic bilateral adrenalectomy, and she evolved with decreasing hypercortisolism. Screening was also performed for other tumors related to this syndrome. The diagnostic criteria, screening and follow-up for patients and affected family members are discussed.

Cutaneous melanoma may develop de novo on normal skin or in contiguity with a potential melanocytic precursor. We report a 45-year-old man who presented with a recently developed nodule in a previously stable congenital nevus. Physical examination revealed a 10x18-cm lesion with speckled lentiginous pigmentation and terminal hairs on the lower back. A 2x2-cm suspicious nodule in the lesion was noted by the patient 2 months earlier. Histopathological evaluation of the nevus and the suspicious nodule revealed the characteristics of a melanocytic nevus and melanoma, respectively. It was interesting for the authors to observe terminal hairs in a lesion that was clinically 'speckled lentiginous' in appearance. This case report is a reminder that there may be great variation in the clinical appearance of nevus spilus, and thus dermatologists must be aware of these lesions as potential precursors of malignant melanoma.

Partial unilateral lentiginosis (PUL) is a rare pigmentary disorder characterized by multiple lentigines on otherwise normal skin affecting one side of the body. We report on a girl with an extensive form of bilateral lentiginosis with systematized segmental distribution and a unilateral congenital cataract. The arrangement of the lentigines is reminiscent of a checkerboard pattern. The benign condition is discussed in the context of systemic diseases with lentigines.

Nevus depigmentosus (ND) is classically defined as a congenital nonprogressive hypopigmented macule, stable in size and distribution. There have been many reports of colocalization of ND and lentigines. We describe development of multiple lentigines over ND in a 9-year-old girl along with hypoplasia of the underlying breast. The case is being reported to highlight the phenotypic manifestation of reverse mutation and the coincidental breast hypoplasia that has not been reported before.

Background: Benign pigmented lesions of the genitalia, such as genital lentigines and melanocytic nevi, often show clinical and histopathologic features highly suggestive of malignant melanoma (MM). Superimposed changes of lichen sclerosus (LS) may cause real concern and lead to an erroneous diagnosis of MM., Objective: This study was performed to assess clinicopathologic characteristics of genital lentigines and melanocytic nevi with associated LS., Methods: We performed a retrospective review of 5 cases., Results: Histopathologic sections of the 2 cases of genital lentigines with concurrent changes of LS showed a lichenoid lymphocytic infiltrate and pigment incontinence with melanophages in a fibrosed papillary dermis, features reminiscent of completely regressed MM. The 3 cases of genital melanocytic nevi and superimposed LS were sharply circumscribed, relatively symmetric, but revealed confluent nests varying in size and shape and pagetoid upward spread of melanocytic nests and single melanocytes. Changes of LS extended beyond the melanocytic proliferation., Conclusion: Genital lentigines and melanocytic nevi with associated LS may show features that mimic MM.

A 9-year-old Taiwanese girl had an unusual combination of multiple lentigines clustered on her left face, 3 cafe-au-lait spots on her left arm and ipsilateral axillary freckling. Two Lisch nodules of the iris were recognized in the left eye but none in the right eye, which is why we established a diagnosis of segmental neurofibromatosis 1. No family member had any sign of neurofibromatosis 1. Moreover, the patient had a large speckled lentiginous nevus involving the left side of her trunk, and her father showed a similar skin disorder on his left buttock. These lesions were taken to be unrelated to the patient's segmental neurofibromatosis 1. This case provides further evidence in favor of the concept that partial unilateral lentiginosis represents a mosaic manifestation of type 1 neurofibromatosis., (Copyright (c) 2004 S. Karger AG, Basel.)

Familial progressive hyperpigmentation is rarely described in the literature. We report on five patients from three different families presenting with a peculiar progressive pigmentary disorder. The patients show a progressive diffuse, partly blotchy, hyperpigmentation, intermixed with scattered small hypopigmented macules, a few large hypopigmented areas, occasional café-au-lait spots and, most remarkably, a generalized lentiginosis. Histology revealed different degrees of basal layer hyperpigmentation and pigment incontinence, also in the spots appearing hypopigmented. Ultrastructural analysis showed a normal mode of Caucasian-like melanogenesis with varying content of regular melanosome complexes within the keratinocytes. All families are clustered in a small area around the town of Teublitz in south-east Germany with about 20,000 inhabitants, suggesting a genetic founder effect. Pedigree analysis is compatible with an autosomal dominant mode of inheritance with variable penetrance. Only a few similar, but not identical, cases have been reported in the past. This cluster of cases may therefore represent a rare and perhaps novel variant of a familial progressive disorder of hyperpigmentation.

A 15-year-old boy had an unusual combination of giant congenital melanocytic nevus on his back and a large speckled lentiginous nevus arranged in a checkerboard pattern on the dorsal and lateral aspects of his trunk. The two pigmentary nevi showed distinguishing features both clinically and histopathologically. The speckled lentiginous nevus was not noted at birth but became visible during childhood. We hypothesize that this uncommon co-occurrence may represent a further example of twin spotting and may be categorized as a new, distinct type of phacomatosis., (Copyright John Libbey Eurotext 2003)