Lentigo Related terms: Lentigo is a pigmented lesion containing an increased number of melanocytes Nevus, Myxoma, Hyperpigmentation, Neoplasm, Dysplastic nevus, Lentigo maligna, Carney complex, Melanoma, Lesion, Melanocyte with a hyperpigmentation of adjacent keratinocytes. From: Pathology Secrets (Third Edition), 2009 Pigmented Growths James G. Marks)r MD, Jeffrey J. Miller MD, in Lookingbill and Marks' Principles of Dermatology (Sixth Edition), 2019 Physical Examination Lentigo is a uniform, tan, brown, or dark brown macule. Lentigo simplex is sharply marginated and occurs anywhere on the body and mucosae. These lesions are usually few in number. Lentigo is a brown macule with uniform color. Actinic or solar lentigo is a tan or brown macule, ranging in size from several millimeters to several centimeters, with distinct borders. The lesion occurs in sun-exposed areas of the body: particularly on the dorsum of the hands, neck, head, shoulders, upper trunk, and lower legs. Melanocytic Proliferations Klaus J. Busam, in Dermatopathology, 2010 LENTIGO SIMPLEX Clinical Findings A lentigo simplex (also called a nevoid lentigo) is a well-circurnscribed pigmented macule.Itis usually small (a few millimeters in diameter) and may occur anywhere on the body's surface. Itis likely that some of these lesions represent the earliest stage in the evolution of lentiginous melanocytic nevi. LENTIGINOUS MELANOCYTIC PROLIFERATIONS: Lentiginous __Lentiginous melanoma Solar lentigo Lentigo simplex nevus in situ Size Small(<4mm) Small (¢4 mm) Variable Variable, often large Borders Regular Regular Regular Irregular Density of MC Normalor slightly Slightly increased Increased Significantly increasedincreased Confluence of MC Absent Absent May be present Common Melanocytic nests Absent Absent Present Common Pagetoid MC Absent Absent Absent Common Solitary units of MC along Absent Absent Rare Common adnee Atypia of MC Rare Rare Rare Common Multinucleated Absent Absent. Rare Common melanooytes Skip areas Absent Absent Usually absent Common Epidermal rete ridges Normalor slightly Normaloorslightly Normal or Normal or effaced elongated elongated elongated Mc, melanocyte Pigmented lesions with a lentigo simplex-like histologic picture occurring at mucosal or paramucosal sites have also been termed labial and genital melanotic macules (Fig. 12-2, A). Genital lentigines are often associated with stromal melanophages. A lentigo-like lesion may be the histologic substrate of benign melanonychia striata, Psoralen and UVA treatment-induced hyperpigmentation, or may be seen in the epidermis overlying a dermal scar or dermatofibroma. Various clinical scenarios have been described in which multiple lentigines can occur. In Carney's complex, for example, multiple lentigines and blue nevi are seen in association with melanotie schwannoma, myxomas, and endocrine disorders. Lentigines have also been described in Peutz-Jeghers syndrome, in patients with familial gastrointestinal stromal tumors, and as part of a paraneoplastic phenomenon (Peutz-Jeghers-like pigmented macules, usually in association with esophageal or intestinal adenocarcinomas). Histologic Features Leentigo simplex is histologically characterized by hyperpigmentation of basilar keratinocytes and an increase in the density of solitary units of melanocytes at the dermal-epidermal junction confined to the clinical correlate of a small and circumscribed macule (Fig. 12-2, 8). The melanocytes usually lack cytologie atypia. The nucleus of a melanocyte is usually smaller in diameter than the nucleus of keratinocyte. On occasion the melanocytes and their nuclei may be large and have an epithelioid appearance (Spitzoid lentigo). The presence of enlarged cells per se; that i, without an increase in cell density, should not lead to concern about melanoma or surgical intervention. Epidermal hyperplasia with elongation of rete ridges may be an associated histologic feature of lentigines. Some lesions show marked melanin pigment deposition in keratinocytes, melanocytes, andjor superficial dermal macrophages and may represent a histologic correlate to what is clinically perceived as “ink spot” lentigo SYNDROMES WITH LENTIGINES Name Features Laugier- ‘Melanonychia, mucosal and cutaneous lentigines; sporadic; not associated hamartoma or malignant Hunziker tumor syndromereue-jegn Mucosal (mostly oral labial) and cutaneous lentigines; hamartomatous gastrointestinal polyps syndrome ‘Autosomal dominant; mutation in STK11/LKB1 gene rey 1. Cardiccutaneous type, aso called LAMB syndrome (Lentigines, atrial myxomas, mucocutaneous complex myxomas, blue nevi) 2. Multiple endocrine neoplasia type Pigmented nodular adrenocortical disease; pituitary adenoma; gonadal, thyroid tumors and schwannomas Autosomal dominant; mutation in PKARIA gene Leopard Lentigines syndrome 2 ECG abnormality ‘cular hypertelorism Pulmonie stenosis Abnormality of genitalia Retarded growth, skeletal abnormalities Deafness ‘Autosomal dominant; mutation in PTPN1 gene Differential Diagnosis Lentigines need to be distinguished from solar lentigo, lentiginous nevi, including nevus spilus, but most importantly from in situ melanoma, A solar lentigo shares with a lentigo simplex the feature of hyperpigmentation of basilar keratinocytes. However, in contrast with a simple lentigo, a solar lentigo lacks an associated distinct increase in the cellular density of basilar melanocytes. Small lentiginous junctional melanocytic nevi may have only very few nests. Step sections may be necessary to reveal such nests and allow distinction of such nevi from a simple lentigo. Acquired Rashes in the Older Child Kristen E. Holland, Paula J. Soung, in Nelson Pediatric Symptom-Based Diagnosis, 2018 Lentigines Lentigines are 1- to S-mm macules that are darker than freckles and may occur on any cutaneous site, including the mucous membranes. Lentigines have no seasonal variance, and those that manifest during early childhood often disappear during adulthood. A lentigo may be clinically indistinguishable from a junctional nevus (mole); however, these lesions are histologically distinct Several syndromes are associated with multiple lentigines. Lentiginosis profusa is an entity characterized by multiple deeply pigmented macules that are usually present at birth or in early infancy. These individuals have no associated systemic or developmental abnormalities, unlike children with LEOPARD syndrome (multiple lentigines, electrocardiograph abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, growth retardation, and neural deafness), which also manifests during infancy. Multiple lentigines located on the mucous membranes, especially the vermilion border of the lips and buccal mucosa, should alert the clinician to the possibility of Peutz-Jeghers syndrome, which is characteristically associated with intestinal polyposis and subsequent risk of malignant transformation and intussusception. Solar lentigines occur in sun-exposed areas in older children. Tumors of the SkinSAUL SUSTER, ... MARTIN C. MIHIMJR,, in Modern Surgical Pathology (Second Edition), 200 Lentigines Lentigo simplex is thought to represent an abnormality in melanocyte migration that presents clinically as a small, well- circumscribed hyperpigmented macule. Elongated, cub-shaped rete ridges showing basilar hyperpigmentation with increased frequency of melanocytes characterize the process histologically. The lesions differ from a freckle (ephelis) because of the elongation of the rete ridges and the increased frequency of basilar melanocytes. They must also be distinguished from solar lentigo, which is characterized by a more pronounced elongation of rete ridges and increased basal pigmentation, without an increase in the number of melanocytes. Nevus spilus, a congenital lesion that may reach upto 20 cm in diameter, exhibits the same histology as lentigo simplex. Sun Protection in Man Jin Ho Chung, in Comprehensive Series in Photosciences, 2001 Solar lentigo. Solar lentigo refers to circumscribed, pigmented macules on the sun-exposed skin of the elderly. There is an elongated epidermal rete, and an increased number of melanocytes [61]. Usually solar lentigines are seen on older patients following chronic sun exposure. Solar lentigo is present in 90% of whites older than 60 years, its prevalence directly correlated with increasing age [62]. The face, forearm and the dorsum of the hand are common sites. The lesions are large and have an irregular edge but are usually brown. Solar lentigo is known to be more common in Caucasians and prevalent among people who have darkly pigmented skin [63] Lentigines, nevi, and melanomas David Weedon AO MD FRCPA FCAP(HON), in Weedon's Skin Pathology (Third Edition), 2010 SOLAR (SENILE) LENTIGO. Solar lentigos are dark-brown to black macules, 3-12 mm or more in diameter, which develop on the sun-exposed skin, of middle-aged to elderly patients. They are often multiple. They are considered to be a hallmark of aged skin.”? The term ‘ink-spot’ lentigo is sometimes used for a clinical variant characterized by its black color and a markedly irregular outline, resembling a spot of ink on the skin (Fig. 32.2).”? The ‘ink-spot’ lentigo (reticulated melanotic macule of the trunk) has also been regarded as a melanotic macule along with labial and genital melanotic macules.? Solar lentigos may increase slowly in size over many years. The ABCD rule (Asymmetry, irregular Border, Color variegation, Diameter larger than 6 mm), which are clinical criteria for the diagnosis of melanomas and their distinction from nevi, is not applicable to all solar lentigos and some early lentigo malignas. Dermoscopy is more reliable.” Solar lentigos may evolve into the reticulate form of seborrheic keratosis, such lesions developing a slightly verrucose surface.”® Solar lentigos increase with age. They are related to freckling during adolescence and frequent sunburns during adulthood.” They appear to be a common precursor lesion of malignant melanoma in patients with xeroderma pigmentosum’® Gene expression profiling of solar lentigo shows up-regulation of genes related to inflammation, fatty-acid metabolism, and melanocytes and down-regulation of cornified envelope-related genes, suggesting that solar lentigo is induced by the mutagenic effect of repeated ultraviolet light exposures in the past, leading to enhancement of melanin production, together with decreased proliferation and differentiation of lesional keratinocytes.”? Another paper has concluded that multiple solar (senile) lentigos on the face are an aging pattern resulting from a life excess of intermittent sun exposure in dark-skinned Caucasians.”® Solar lentigos on the upper back are an earlier stage of this phenomenon. ”? Abnormal pigment retention in keratinocytes appears to be the primary defect in solar lentigo, which may partly explain the therapeutic effect of retinoids.®° A case can be made for their removal from this chapter as keratinocytic changespredominate over melanocytic changes.®° The dermoscopie features of solar lentigos and their distinction from melanoma have been well studied.*! Pigmented corneocytes are a feature of the ‘ink-spot’ lentigo on dermoscopy.*? Treatment of solar lentigo The treatment of solar lentigines has been the subject of a consensus commentary by the Pigmentary Disorders ‘Academy.*? They recommended ablative therapy using cryotherapy as first-line therapy. This body also believes that there is evidence to suggest that lasers are an effective treatment. An alternative to ablative therapy is topical therapy, and there is evidence to support the use of a fixed double combination such as hydroquinone and tretinoin.® Tazarotene, and other retinoids can also be used. A recent study has found that cryotherapy shows better results than trichloroacetic acid 33% solution in the treatment of solar lentigo. Histopathology The solar lentigo is characterized by elongation of the rete ridges, which are usually short and bulb-like (Fig. 32.3). As they extend more deeply into the dermis, finger-like projections form and connect with adjacent rete ridges to form 2 reticulate pattern resembling that seen in the reticulate type of sebortheic keratosis.”>* Rete ridge hyperplasia is less conspicuous, and may be absent, in lesions from the face.*® in addition, there is basal hyperpigmentation which is. sometimes quite heavy. There is an increased number of melanocytes, particularly at the bases ofthe clubbed and budding rete ridges;*® sometimes the increase is not appreciated on casual examination. Variable numbers of melanophages are presentin the papillary dermis. These melanophages are factor Xilla* dermal dendrocytes.°7 When solar lentigos undergo regression they develop a heavy lichenoid inflammatory cell infiltrate in the papillary dermis. These features are referred to as a lichen planus-like keratosis (see p. 47). The author has seen a number of cases of lentigo maligna (Hutchinson's melanotic freckle) developing in solar lentigos.. The presence of transitional features (unstable solar lentigo) suggests that this is not a ‘collision phenomenon’. Others have also documented this transformation 7##* Electron microscopy The melanosome complexes are much larger than those found in non-involved skin. Skin Problems Marlene J. Mash, ... Lucy Bonnington, in Primary Care Geriatries (Fifth Edition), 2007 Solar Lentigines Solar lentigines (“brown spots” or “lver spots") ae circumscribed, pigmented, nonmalignant macules (plate 3). They are ‘approximately 0.5 cm in diameter and induced by both natural and artificial sources of UV radiation. In rare cases and ‘over a period of many years, lentigines develop into a melanoma (lentigo- maligna melanoma). These malignant lesions are usually larger than lentigines (ranging from 3 to 6 cm), and they are irregularly pigmented and irregularly shaped with irregular borders, whereas lentigines are usually circumscribed. If adequate treatment of a lentigo-maligna melanoma (i.e., complete surgical excision with adequate margins) is not provided, there is a 50% chance that the lesion will become invasive malignant melanoma and a 10% chance that it will metastasize (Plate 4).? Cutaneous Congenital Defects* Katherine B, Piittgen, Bernard A. Cohen, in Avery's Diseases of the Newborn (Ninth Edition), 2012 Lentigines Lentigines are small tan-to-dark brown macules that most commonly appear sporadically in adulthood. They are distinguished from other pigmented lesions by histologic examination that reveals elongated rete ridges, an increased number af sinoly disnersed melanacytes along the hasal laver_ and increased melanization of the hacal keratinocytesMultiple or congenital lentigines are features of several syndromes Carney Complex, Type 1 (Including NAME and LAMB) [OMIM 160980] Carney complex is an autosomal dominant multiple neoplasia syndrome due to a mutation in the protein kinase A regulatory subunit-1-alpha gene (PRKARIA) on 179, Cardiac, endocrine, cutaneous, and myxomatous neural tumors ‘occur. The related phenotypes designated NAME and LAMB share the features of atrial myxoma and pigmented skin lesions. NAME includes nevi and ephelides, whereas LAMB includes lentigines and blue nevi. Pigmented lesions are present at birth. LEOPARD Syndrome [OMIM 151100] Leopard syndrome is an autosomal dominant disorder due to mutations in the PTPNILI gene that includes multiple lentigines, dectrocardiographic defects, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, growth retardation/restriction, and sensorineural deafness. Skin lesions do not appear until after the Ist year of life. Peutz-Jeghers Syndrome [OMIM 175200] Peutz-Jeghers syndrome, a disorder of autosomal dominant inheritance, is due to mutations in the serine/threonine kinase STK11 gene and includes mucocutaneous pigmented macules and intestinal polyposis. The pigmented lesions ‘may be congenital and usually appear on the lips, buccal and gingival mucosae, and dorsa of the fingers and toes. Cutaneous Tumors and Pseudotumors of the Head and Neck Mark R. Wick, in Diagnostic Surgical Pathology of the Head and Neck (Second Edition), 2009 Lentiginous Melanoma in Situ and Invasive Lentiginous Melanoma Melanomas with lentiginous features, that is, an essentially confluent proliferation of atypical melanocytes in the basilar portion of the epidermis, are most common in sun-exposed skin areas. However, there are several additional histologic criteria that must be met before a diagnosis of lentiginous melanoma in situ (lentigo maligna) can be made. These include epidermal atrophy, extensive dermal actinic elastosis, and the presence of atypical melanocytes within the superficial aspects of skin appendages (e.g.,pilosebaceous units) that are present in the biopsy specimens (Figs. 14-92 and 14-93)522555-55 |f jn addition to this image, there is also an invasive tumor component in the corium, the term invasive lentiginous melanoma (lentigo maligna melanoma) may be applied.°55°6 Particular cytologic features of invasive lentiginous melanomas are similar to those of invasive melanomas in general, although spindle cell differentiation is overrepresented in this specific context. Disorders of Pigmentation John C. Mavropoulos, Bernard A. Cohen, in Pediatric Dermatology (Fourth Edition), 2013 Solar lentigines Solar lentigines usually do not become apparent until the 4th and 5th decades of life, although they may appear in later childhood or adolescence, particularly in lightly pigmented individuals who spend extensive time outdoors. These irregularly shaped, darkly pigmented macules range from a few millimeters toa few centimeters in diameter and appear ‘on sun-exposed skin. Although the risk of malignant degeneration is minimal, they provide a marker of significant sun ‘exposure. Children and parents should be counseled regarding the cumulative risk of actinic damage and use of protective dothing and sunscreens. View full topic index