Cornoid Lamellation Associated With Lichen Sclerosus To the Editor: Although cornoid lamellation is the histological feature that characterizes porokeratosis, it is not limited to porokeratosis and may be encountered in a wide range of other inﬂammatory and neoplastic skin diseases.1 A cornoid lamella reﬂects disordered progression of epidermal cells to corniﬁcation and should be regarded as one of the “minor tissue reactions” in a variety of other inﬂammatory skin disorders.2 Cornoid lamellation has recently been revisited to deﬁne the large clinicopathological spectrum of porokeratosis and to establish nonporokeratotic conditions in which cornoid lamellas can appear.3 We report for the ﬁrst time cornoid lamella in association with lichen sclerosus (LS), which seems to reﬂect an incidental ﬁnding, and has to be recognized and differentiated from porokeratosis. A 51-year-old man presented to the Dermatology clinics with asymptomatic, large licheniﬁed plaques developing within an erythematous surface affecting his upper arm and shoulder (Figs. 1A, B). The lesions initially appeared as small pink plaques, which then enlarged and evolved with whitish scaling, which did not respond to antifungals. A biopsy taken from the upper arm showed extensive eosinophilic homogenization of the papillary and superﬁcial reticular dermis, immediately beneath the epidermis and focal vacuolization of the dermal–epidermal junction, with scant superﬁcial perivascular lymphocytic inﬁltrate (Fig. 2A). The epidermis was slightly atrophic, and no necrotic keratinocytes were observed. Marginated on the sides of the atrophic epidermis, we could focally note vertical columns of parakeratosis overlying areas of epidermis with loss of the granular layer and dyskeratotic cells (Figs. 2B–D).
The authors declare no conﬂicts of interest.
FIGURE 1. Clinical features of extragenital LS. Coalescing erythematous macules with ivory white surface and well-defined borders (A). Higher magnification of the lesion shows ivory white surface of the lesions, slightly scaly (B).
Cornoid lamella, characterized by a tilted vertical column of parakeratosis overlying an area of hypogranulosis with dyskeratotic cells, can be subtle and easily overlooked clinically and can histologically hinder the diagnosis. Cornoid lamellation is a constant histological marker of porokeratosis. Different subtypes of porokeratosis are well characterized based on the number, size, and distribution of the annular lesions or papules and the prominence and distribution of the cornoid lamellas.3 However, it is also accepted in the literature that cornoid lamellation is not pathognomonic of porokeratosis and can also be found in a variety of inﬂammatory or neoplastic conditions,1 in which it represents a distinctive histologic reaction pattern that reﬂects disordered progression of epidermal cells to corniﬁcation. Inﬂammatory disorders including psoriasis, lichen planus–like keratosis, Wong type-dermatomyositis, keratosis lichenoides chronica, porokeratotic variant of Grover disease and Fox–Fordyce disease, hamartomas such as porokeratotic eccrine ostial dermal duct nevus, and inherited disorders of the keratinization have been described in association with real cornoid lamellation,3 in addition to a wide spectrum of other skin lesions including scars, viral warts, seborrheic keratosis, milia, solar keratosis, squamous cell carcinoma in situ, and basal cell carcinoma. In these particular
Am J Dermatopathol Volume 0, Number 0, Month 2016
settings, it has been related to genetic instability resulting from an unidentiﬁable trigger. LS is a chronic inﬂammatory dermatosis that results in epidermal atrophy of unknown etiology. Both, genital or extragenital presentations have been described, with a clear predilection for the vulva. First described by Darier,4 LS is deﬁned as a vacuolar interface dermatitis with the presence of homogenized eosinophilic acellular zone within the superﬁcial dermis.5 The association of LS with other epithelial alterations is usually interpreted as secondary. Most commonly, vulvar LS, which is predominantly a pruritic condition, frequently results in a superimposed lichen simplex chronicus and excoriations. Similarly, the presence of atrophy in cutaneous lesions of LS is also a common ﬁnding.6 Interestingly, no studies have previously reported the association between LS and cornoid lamellation. This case is the ﬁrst to describe a plaque of extragenital LS associated with the presence of cornoid lamellas in the margins. We interpret it as an incidental ﬁnding secondary to the epithelial alterations occurring in LS; however, genetic instability may play a role in the coincidental manifestation of these lesions. Because the diagnosis of early and evolving lesions of LS without the hallmark sclerosis can be problematic, the purpose of this case is to highlight a new histopathological feature www.amjdermatopathology.com |
Copyright Ó 2016 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Letters to the Editor
Am J Dermatopathol Volume 0, Number 0, Month 2016
FIGURE 2. Microscopic findings of the lesion show a slightly atrophic epidermis with hyperkeratosis (A), and dermal sclerosis, with vacuolar interface changes and a light perivascular lymphocytic infiltrate underlying the sclerosis (B). Two tilted cornoid lamellas are seen overlying the lateral edge of the atrophic epidermis (C). An area of dyskeratotic keratinocytes with hypogranulosis is present under the vertical column of parakeratosis (D).
that can overlap with LS and delay its diagnosis. Maria C. Garrido, MD, PhD* Veronica Monsalvez, MD† Jose L. Rodriguez-Peralto, MD, PhD* *Department of Pathology, Hospital Universitario 12 de Octubre, Instituto de Investigación I+12, Facultad de Medicina, Universidad Complutense, Madrid, Spain
†Department of Dermatology, Hospital Universitario 12 de Octubre, Instituto de Investigación I+12, Madrid, Spain
REFERENCES 1. Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am J Dermatopathol. 1980;2:5–15. 2. Weedon D. An approach to interpretation of skin biopsies. In: Weedon D, ed. Weedon’s Skin Pathology. 3rd ed. London, United Kingdom: Churchill Livingstone Elsevier; 2010:3–18.
3. Biswas A. Cornoid lamellation revisited: apropos of porokeratosis with emphasis on unusual clinicopathological variants. Am J Dermatopathol. 2015;37:145–155. 4. Darier J. Lichen plan sclereux. Ann Dermatol. 1882;3:833–837. 5. Hewitt J. Histologic criteria for lichen sclerosus of the vulva. J Reprod Med. 1986;31: 781–787. 6. Carlson JA, Lamb P, Malfetano J, et al. Clinicopathologic comparison of vulvar and extragenital lichen sclerosus: histologic variants, evolving lesions, and etiology of 141 cases. Mod Pathol. 1998;11:844–854.
Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence
Lichen sclerosus is an inflammatory skin condition characterized by inflammation of the papillary dermis that leads to white scarlike plaques. It occurs classically in the genitals but also has extragenital manifestations with a variety of clinical p
Lichen sclerosus commonly affects the genitalia of post-menopausal women. We describe a woman with painful, disseminated, bullous, extragenital lichen sclerosus that responded to oral acitretin and topical calcitriol and triamcinolone.
Lichen sclerosus is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, labial adhesions, and loss of pigmentation. Treatment
Lichen planus and lichen sclerosus are common, chronic inflammatory vulvar dermatoses with significant morbidity. The course may wax and wane but disease often persists for decades. These autoimmune diseases have varied clinical presentations that ex
Lichen sclerosus is a chronic inflammatory skin disease. It is thought to be underdiagnosed and undertreated. If it is not treated, lichen sclerosus is associated with a greater degree of scarring and an elevated risk of cancer in the genital area.
Lichen sclerosus affecting the male genitalia is a poorly understood but potentially devastating condition. The natural history of the condition is beginning to be understood better with longer follow-up of patients. Recent long-term data suggest tha