Eccrine angiomatous hamartoma eah is a rare, benign. Porokeratotic eccrine ostial and dermal duct nevus jama. A case of porokeratotic eccrine ostial and dermal duct naevus of late onset. Eccrine nevus is a rare skin lesion with protean manifestations. It is usually characterized histologically by the increase in number or size of structurally normal eccrine glands. Eccrine and apocrine poromas typically present as erythematous or flesh. Eccrine poroma developing on nevus sebaceous is extremely rare. It is more often associated with localized hyperhidrosis, while cases not associated have also been reported.
In the supreficial dermis, some melanocytes may produce melanin pigment in the cytoplasm darkbrown, granular. Eccrine nevus is a rare skin lesion with protean manifestations like hyperhidrosis, discolored nodules, papules, and so forth, which has been reported in various anatomic parts of the body including the forearm, leg, thigh, back, and coccyx. The histopathological features comprise both eccrine proliferation and abundant mucin deposition sur rounding the eccrine glands and ducts. A differential diagnosis that should be based on clinicopathologic correlation. Intradermal melanocytic nevus a benign tumor in which the tumor cells form nests in the dermis, are regular, round, with central nucleus and single nucleolus. Normal terminal hair follicles are characteristically absent in the lesion although the same may be seen in rest of the epidermis a feature of diagnostic importance not usually highlighted in literature. A 6yearold girl with congenital porokeratotic eccrine ostial and dermal duct nevus involving the palmoplantar surfaces ie, the back of the hands and feet, the limbs, the neck, and the axillae is described. Spiradenoma, also spiroma or eccrine spiradenoma, is a cutaneous condition that is typically characterized, clinically, as a solitary, deepseated dermal nodule of approximately one centimeter, occurring on the ventral surface of the body. Linear eccrine poroma jama dermatology jama network.
Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish specific. They are pas positive and much of the paspositive material is diastase sensitive. Iphd in dermatopathology and adjunct professor of pathology at the pontificia universidade. Porokeratotic eccrine ostial and dermal duct nevus peoddn is a rare nevoid condition characterized by asymptomatic grouped keratotic papules and plaques with a linear pattern on the extremities, having distinct porokeratotic histopathological features. Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. Porokeratotic eccrine ostial and dermal duct nevus a 3yearold girl presented to us for a rough aspect of the trunk. The lesion was excised to exclude the possibility of melanoma.
Biopsy findings revealed slightly increased numbers of eccrine glands without any other abnormality, consistent with an eccrine nevus. These entities can be differentiated by histopathology. Department of dermatology, ajou university school of medicine, suwon, korea an eccrine nevus is a rare hama rtoma characterized by an increase. Eccrine angiomatous hamartoma eah is a rare, benign cutaneous tumor characterized by proliferation of the eccrine gland elements closely associated with capillary angiomatosis and proliferation of other dermal elements, such as adipose tissue, hair and epidermis. Herein we present a case of eccrine nevus not associated with localized hyperhidrosis on the left wrist of a 6yearold girl. Lateonset solitary porokeratotic eccrine ostial and dermal. One third occur within nevus sebaceus papillary fronds decapitation secretion.
According to argenziano et al, a dermatopathologic distinction between pigmented spitz and reed nevus is difficult, not reproducible and maybe clinically useless. Eccrine hidradenoma is a rare benign cutaneous neoplasm, believed to arise from the distal excretory duct of the eccrine sweat glands 1. Histopathological findings are similar in all cases. What is porokeratotic eccrine ostial and dermal duct naevus porokeratotic eccrine ostial and dermal duct naevus peoddn is a rare congenital disorder of keratinisation. Dermatologic examination showed multiple rough papules in the upper and lower limbs fig. Adultonset eccrine angiomatous hamartoma on the vulva. Widespread involvement along blaschkos lines can also occur. Surgical excision revealed a tissue of fibroelastic consistency.
The deep dermis often shows a proliferation of morphologically normal eccrine glands with. Department of dermatology, ajou university school of medicine, suwon, korea an eccrine nevus is a rare hama rtoma characterized by an increase in the number or size of eccrine glands. They proposed that beckers nevus could be considered a component of the androgen receptor hypersensitivity syndrome spectrum. While the presence of hypertrophy or hyperplasia of eccrine glands is considered to be integral to the designation of a lesion as an eccrine nevus,9. Epidermal nevi are benign, hamartomatous growths of the skin that are present at birth or develop in early childhood.
A clinical and histopathological study of nevus sebaceous. Verrucous eccrine angiomatous hamartoma verma p, kaur m. It involves the intraepidermal ducts of the eccrine sweat glands with a characteristic histopathology. The lesions have histopathology consistent with dariers and are found to have somatic mutations in atp2a2,the causative g ene for darier disease. Eccrine nevi are very rare cutaneous lesions which may or may not be accompanied by localized hyperhidrosis. Acquired melanoyctic naevus or mole is a common benign tumour, usually appearing during childhood and adolescence. Porokeratotic eccrine ostial and dermal duct nevus. Path department of cellular pathology, pathlinks pathology services, lincoln county hospital, lincoln, united kingdom abstract. They may be composed of a variety of epidermal cells and structures, including keratinocytes, sebaceous glands, hair follicles, apocrine and eccrine glands, and smooth muscle cells, and are thought to represent a form of cutaneous mosaicism. The lesions usually present at birth or in childhood. Porokeratotic eccrine nevus is another keratotic epidermal nevus.
Melanocytic nevus associated with syringofibroadenomatous. Casereport porokeratotic eccrine ostial and dermal duct nevus. Apr 19, 2020 eccrine hidradenoma is a rare benign cutaneous neoplasm, believed to arise from the distal excretory duct of the eccrine sweat glands 1. The clinical and histopathologic findings are presented. Eccrine nevus is a rare disease, which may be present at birth or at an early age. Coccygeal polypoid eccrine nevus archives of pathology. The term porokeratotic eccrine ostial and dermal duct. Our patient was a 26yearold male, who presented with increasing colorless and odorless episodic umbilical discharge. Apocrine nevus consists of numerous tubular structures lined by a layer of apocrine secretory with evidence of secretion by decapitation at the luminal edge. The term eccrine angiomatous hamartoma was coined by hyman et al.
The histopathological hallmark of porokeratotic eccrine ostial and dermal duct nevus is the presence of a cornoid lamella with subjacent acrosyringium. Francisco bravo puccio and cesar chian 2011 acral junctional nevus versus acral lentiginous melanoma in situ. Rare cases have been reported in childhood, 19 including one congenital case. Porokeratotic eccrine ostial and dermal ductal nevus peoddn is a rare benign nevoid disorder characterized by cornoid lamella, which are a thin column of parakeratotic cells with a decreased or absent granular layer and vacuolated or dyskeratotic cells in the spinous layer that involve the acrosyringia 1. Pdf porokeratotic eccrine nevus may be caused by somatic. Blue nevus bn is a common specimen in routine pathology practice. Poroma is a benign adnexal neoplasm of the terminal sweat gland duct. They had the histologic appearance of eccrine poroma. Two cases of coccygeal polypoid eccrine nevi presenting as skin tags min young park, m. The condition was first named by abell and read in 1980 2 and first. Introduction e term porokeratotic eccrine ostial and dermal duct nevus peoddn was rst described in by abell and. Keratinisation is the process by which the skin cells form in the epidermis. Histologic correlation of dermoscopy findings in a. We report a case of a 30yearold female who presented with a congenital, hairless, verrucous, yellowish lesion on the scalp and an erythematous nodule arising within the yellowish lesion for 8 months.
The cells are smaller than, and well delineated from, the epidermal cells with which they are in contact. Eccrine angiomatous hamartoma is a lymphatic proliferation. Jan 23, 2017 eccrine nevus is a rare skin lesion with protean manifestations like hyperhidrosis, discolored nodules, papules, and so forth, which has been reported in various anatomic parts of the body including the forearm, leg, thigh, back, and coccyx. Eccrine angiomatous hamartoma eah is a benign nodular or plaquelike tumor of hamartomatous nature characterized by the proliferation of eccrine and vascular structures. Although poromas have traditionally been thought to originate from the eccrine sweat gland, there have been cases of apocrine etiology as well.
Mar 21, 2017 typical histopathology of eccrine nevus includes an increase in the number or size of eccrine coils. Eccrine and apocrine sweat gland tumors are believed to constitute as few as 1% of primary cutaneous lesions. There are few case reports of eccrine poroma developing within nevus sebaceous. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Pdf eccrine nevi are very rare cutaneous lesions which may or may not be accompanied by localized hyperhidrosis. Histological findings usually reveal a slightly increased number of eccrine glands without any other abnormalities.
The patient was a 50yearold african american woman who noticed a new mole on her foot that began as a dark speck but quickly grew larger. A relatively rare tumor, poroma comprises approximately 10% of these lesions. Porokeratotic eccrine nevus may be caused by somatic connexin26 mutations jennifer a. Skin nonmelanocytic tumor nevus sebaceus of jadassohn. The lesions were limited to the right lower extremity and were arranged in a linear fashion.
Syringocystadenoma papilliferum raised warty plaque. Lesions evolve with age, the initial lesion being macular with nests of proliferating. Lateonset solitary porokeratotic eccrine ostial and. A 44yearold woman had multiple papular lesions of 20 years duration. Nevus or nevi if multiple is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. Porokeratotic eccrine ostial and dermal duct naevus. It is also supposed that porokeratotic eccrine nevus may be caused by somatic connexin 26 mutations. It is usually associated with the dilation of eccrine duct. Two distinct variants of eccrine nevus have been described in the literature. It can also present with discolored nodules, papules, and plaques. Acral junctional nevus versus acral lentiginous melanoma in. Eccrine angiomatous hamartoma is a rare, benign cutaneous proliferation of eccrine glands and thinwalled vascular channels.
A point mutation in the braf gene most often v600e is usually the initiating genetic mutation. Porokeratotic eccrine nevus may be caused by somatic. After puberty, histologic features characteristically include papillomatous hyperplasia of the epidermis, a large number of mature or nearly mature sebaceous glands, and a lack of terminally differentiated hair follicles. Case report porokeratotic eccrine ostial and dermal duct nevus. It is a disorder of keratinization involving the intraepidermal eccrine duct acrosyringium and is characterized by eccrine hamartoma and cornoid lamellation in pathology. Peoddn is a special type of epidermal naevus porokeratotic eccrine and hair follicle naevus is a very similar entity. Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. Typical histopathology of eccrine nevus includes an increase in the number or size of eccrine coils 3. Twelveofthese cases were female, 3 of them were congenital, and the. Twenty percent of the cases showed dilated, prominent apocrine glands and fifteen percent showed prominent, dilated eccrine glands.
Histology showing slightly increased numbers of eccrine sweat duct units. Sometimes, ductal hyperplasia and dilation of lumina are notable as well 4. Sebaceous nevus is a relatively common hamartoma that presents most often as a single congenital hairless plaque on the scalp. It generally arises at birth or later in childhood, with a few reports of puberty or adultonset lesions, as solitary or multiple lesions affecting mainly the distal. Histopathology revealed multiple wellcircumscribed. Porokeratotic eccrine nevus may be caused by somatic connexin26 mutations article pdf available in journal of investigative dermatology 29. Hidradenoma refers to a benign adnexal tumor of the apical sweat gland. At the periphery of these tubules, there is a discontinuous layer of spindleshaped myoepithelial cells. An eccrine nevus is an extremely rare cutaneous condition that, histologically, is characterized by an increase in size or number of eccrine secretory coils 774.
A rare multifocal variant with features suggesting trauma veena naik md 1, nebojsa arsenovic md 2, malcolm reed md 2 dermatology online journal 15 9. Lateonset and rare clinical presentation as pruritic lesion are the characteristic features that make this patient an extraordinary presentation of peoddn. Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome. Request pdf porokeratotic eccrine ostial and dermal duct nevus porokeratotic eccrine ostial and dermal duct nevus is a rare disorder characterized by distinctive histopathology. A 7yearold boy without any relevant medical history and with no family history of consanguinity had skin lesions located. Association of nevus of jadassohn, sebaceoma and trichoblastoma in a scalp lesion. We report three men aged 40, 20 and 16 years with porokeratotic eccrine ostial and dermal duct nevus. Peoddn is a rare benign cutaneous disorder that clinically resembles comedo nevus but favors the palms and soles, where pilosebaceous follicles are absent. Sun exposure is a causative factor, particularly in childhood. Differentiation of pigmented spitz nevi and reed nevi by.
Histologic correlation of dermoscopy findings in a sebaceous. Trichoblastoma is the most common neoplasm developed in nevus sebaceus of jadassohn. However, to date, many textbooks or atlases of dermatology or histopathology assert that reed nevus is a variant of spitz nevus 4,6,2125. Kawaoka jc, gray j, schappell d, robinson bostom l. Friedmanbirnbaum, porokeratoticeccrineostialanddermalductnevus. Porokeratotic eccrine ostial and dermal duct nevus, or porokeratotic eccrine nevus pen, is a hyperkeratotic epidermal nevus. Eccrine poroma is a circumscribed tumor composed of cords and broad columns of uniform basaloid cells extending into the dermis from the undersurface of the epidermis fig. It may present in childhood or adolescence with signs and symptoms such as localized unilateral hyperhidrosis with tendency to involve extremities but has been reported in other anatomic locations as well. Two cases of coccygeal polypoid eccrine nevi presenting as. An eccrine angiomatous hamartoma with histopathological features of a papillary haemangioma in a neonate.
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