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Steroid-responsive facial eruption with cornoid lamellae - a possible new entity

  • Author(s): Tran, Hien
  • Bossenbroek, Nicole M
  • Rosenman, Karla
  • Meehan, Shane A
  • Sanchez, Miguel
  • Prystowsky, Stephen
  • et al.
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Steroid-responsive facial eruption with cornoid lamellae - a possible new entity
Hien Tran MD PhD, Nicole M Bossenbroek MD, Karla Rosenman MD, Shane A Meehan MD, Miguel Sanchez MD, Stephen Prystowsky MD
Dermatology Online Journal 14 (10): 9

Department of Dermatology, New York University

Abstract

The histopathologic presence of a cornoid lamella is often associated with a diagnosis of porokeratosis. However, this feature is not pathognomonic for porokeratosis and can be found in a number of other dermatologic conditions, which include seborrheic keratosis, verruca vulgaris, actinic keratosis, squamous-cell carcinoma in situ, basal-cell carcinoma, milia, and scar. Notably, the etiology of none of these entities is inflammatory. Wade and Ackerman consider cornoid lamellation to be a distinctive histopathologic reaction pattern that reflects the disordered progression of epidermal cells during cornification [1]. As such, this pattern is not specific for any given disease process. We report a case in which the lesions appeared inflammatory clinically as well as histopathologically, did not resemble porokeratosis despite the presence of cornoid lamellae, and responded to topical glucocorticoids.



Figure 1Figure 2

History

A 48-year-old man presented to the Dermatology Clinic of the Bellevue Hospital Center with a pruritic eruption on his face that initially appeared on his scalp during a 24-week course of pegylated interferon alpha-2b for a hepatitis C virus infection. Approximately four months after completing the treatment, the lesions spread to his face. Application of ethyl alcohol and non-prescription acne medications produced no beneficial effect. He denied allergies, previous skin disease, or sensitivity to sunlight. At the time of presentation and during six months of follow-up evaluation, no lesions developed on his scalp, mucous membranes, legs, palms, soles, or any other part of his body except for two papules on his upper back.

The patient was initially treated with calcipotriene ointment and imiquimod cream without detectable change in the appearance of the lesions. However, after three weeks of fluocinonide 0.025 percent ointment, the size of the lesions was diminished. Treatment was changed to acitretin 25 mg daily and triamcinolone 0.1 percent ointment to the left side of the face only. After two weeks, improvement was confined to the left side of the face. Due to elevated lipids and lack of efficacy, acitretin was discontinued after 20 weeks. Topical therapy was changed to clobetasol 0.05 percent ointment, and within four weeks most of the lesions had resolved. The remaining papules healed without scars after a single intralesional injection of triamcinolone acetonide diluted to a concentration of 5 mg per mL. The patient has developed only a few lesions that respond to topical glucocorticoids.


Physical Examination

Discrete, well-demarcated, round, erythematous, 2-to-8-mm papules were present on the forehead, nose, cheeks, lateral aspects of the face, and chin. The early lesions were pink, concave papules with fine scale. These lesions grew and became slightly annular and hyperkeratotic, with yellow-gray crusts that covered excoriated areas. While the borders of the larger papules appeared slightly raised, neither surrounding thin ridges nor furrows were apparent.


Lab

A complete blood count, metabolic panel, fasting lipids, rapid plasma reagin test, and human immunodeficiency virus test were normal or negative.


Histopathology

All four biopsy specimens show similar findings. There are multiple cornoid lamellae composed of tiers of parakeratotic stratum corneum, an absent granular layer, and dyskeratotic keratinocytes. Many are found within adnexal ostia. There is associated epidermal hyperplasia with mild spongiosis and a dense, superficial, perivascular and interstitial infiltrate of lymphocytes, eosinophils, and some plasma cells.


Comment

The porokeratoses are a group of hyperkeratotic skin disorders that are characterized clinically by an elevated, thread-like border that expands centrifugally and histopathologically by the presence of cornoid lamellae. There are five well-recognized variants, all of which are associated with autosomal dominant inheritance although ulcerative, labial, and drug-induced cases also have been described [1, 2, 3]. The Mibelli type usually occurs in children and presents as a single a or few papules or plaques. In time, the center of the lesion may become depressed and hypo- or hyperpigmented. The linear form also develops in the pediatric population, and the papules form a linear array. In the disseminated superficial type, brown, annular, keratotic papules arise predominantly on the extensor surfaces of the legs and the arms. The actinic form (DSAP) usually affects middle-aged women with photodamaged skin. The non-actinic type is characterized by small, barely-raised papules that are surrounded by a narrow, hyperkeratotic ridge and tend to involve the trunk, genitalia, palms, and soles. Disseminated superficial actinic porokeratosis has been associated with electron beam skin irradiation, hepatitis C-related hepatocellular carcinoma, renal failure, organ transplantation, human immunodeficiency virus infection, and other immunosuppressive disorders [4, 5, 6, 7, 8, 9]. In the punctate variety, small, barely-raised, hyperkeratotic papules with raised rims are observed on the palms and soles while in the palmoplantar type the lesions spread in a generalized distribution that may involve mucous membranes.

Although its precise pathogenesis remains unclear, porokeratosis is considered a disorder of keratinization. Lesions typically appear on the trunk and extremities; however, in a number of rare cases, porokeratosis has been described on the face [10, 11, 12, 13, 14, 15]. In DSAP, facial lesions are seen in approximately 15 percent of patients and, in rare cases, may be the only involved area. The lesions are described as having an elevated, ridge-like border that histopathologically corresponds to the cornoid lamella [10, 11, 12, 13, 15]. In contrast, the lesional morphology in this case is different from other reported facial cases - the papules were concave with raised borders, the color was bright red rather than skin-colored, the scale was diffuse throughout the surface, and there was no rim or elevated margin. The lesions of porokeratosis are asymptomatic or barely pruritic; however, in our patient the lesions were intensely pruritic and resulted in excoriations.

Histopathologically, the mounds of porokeratosis are consistent with cornoid lamellae. However, in contrast to most cases of porokeratosis, these structures were present throughout the lesion and not only at the periphery [16, 17]. They correlate with the hyperkeratosis uniformly present throughout each lesion. Although cornoid lamellae may be found in more central areas in lesions of DSAP or Mibelli types, the number of these structures and their uniform distribution throughout the lesion is remarkable in our particular case. Acanthosis, which was present in our case, tends to be a feature of porokeratosis of Mibelli but not DSAP.

Despite the absence of corroborative studies, improvement and remission of lesions have been reported with topical and systemic retinoids, vitamin D-3 analogues, imiquimod cream, and topical 5-fluorouracil preparations. However, in most cases, the results are disappointing even after prolonged therapy, and destructive methods, such as cryotherapy, electrodesiccation, and laser may be needed [10, 11]. Oral acitretin invariably produces decreases in size and thickness of the lesions, but no such changes were noted with retinoid therapy after 20 weeks. Response to anti-inflammatory agents is unusual, but in our patient, the lesions resolved rapidly with topical glucocorticoids. In cases of drug-induced disseminated superficial actinic porokeratosis, almost complete resolution of the eruption occurred after discontinuation of all suspected drugs and administration of systemic and topical glucocorticoids [15].

Owing to all of these discrepancies in morphology, distribution, symptomatology, histopathologic features, and treatment response between our patientÕs eruption and those of porokeratosis, we propose that this represents a rare new entity that is distinct from porokeratosis. Indeed, one previously reported patient with hyperkeratotic papules and plaques that were limited to the face is clinically similar to ours [18]. Histopathologically, the parakeratosis of that case was described as a column, but the available figure showed a more mound-like structure rather than the typical column observed in porokeratosis. Based on the response to topical glucocorticoids, clinical appearance, and histopathologic changes, we propose that the etiology is inflammatory in nature. A drug reaction was considered, especially because the eruption developed during a course of interferon. However, new lesions formed and old ones persisted after the medication had been discontinued. It is important to distinguish this case from reported forms of porokeratosis in order to avoid ineffective treatments and to allow for the timely administration of anti-inflammatory agents.

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