Central Keratotic Plug Research Articles

Familial Reactive Perforating Collagenosis: Clinical, Dermoscopy, and Histopathological Features in Two Siblings

Transepidermal elimination disorders include reactive perforating collagenosis (RPC), perforating folliculitis, elastosis perforans serpiginosa, and Kyrle’s disease. RPC is a rare transepidermal elimination disorder wherein altered collagen is extruded through the epidermis. It has two types: rare inherited form and relatively common acquired form. Most inherited cases present during infancy or early childhood with papules on extensor surface of hands, elbows, knees preceded by superficial trauma. We report two female siblings of 11 and 10 years with multiple, symmetrically distributed, hyperpigmented papules with central keratotic plug, predominantly over extensor aspects of extremities with no aggravating and relieving factors. Clinical diagnosis of familial RPC was confirmed by consistent dermoscopic and histopathological findings. Both children were started on oral isotretinoin 10 mg/day with topical 12% ammonium lactate.

Acquired reactive perforating collagenosis, a rare entity at uncommon site.

Acquired reactive perforating collagenosis (ARPC), rare disorder characterized by transepidermal elimination (TEE) of collagen fibers, is seen in adult diabetics. Genetic predisposition, familial aggregation, trauma, bites and scratching are implicated. Diabetics develop microvascular diseases leading to intense pruritus causing repeated micro trauma leading to necrosis of connective tissue of dermis, causing TEE. Isolated papules, plaques and nodules with central keratotic plugs, are mostly seen on extensor surfaces of limbs but trunk and face may be involved. Histopathology shows extrusion of abnormal collagen fibers through epidermis. Multiple treatment modalities show variable response. A 52 year old diabetic female had multiple, itchy, well defined, erythematous papules and plaques with central adherent crusting on lower back since 1 month. Histopathology showed cup shaped epidermal depression filled with plug of altered collagen, acanthotic epidermis with hyperkeratosis and parakeratosis. Underlying epidermis was thin with fine slits through which vertically oriented basophilic collagen fibers were extruded.

Clinicopathologic and Dermoscopic Features of Acquired Perforating Dermatosis: A Case Report

Acquired perforating dermatosis represents one of the perforating skin disorders showing transepidermal elimination of dermal contents. Dermoscopy showing concentric zones of a central keratotic plug, white-gray structureless area and peripheral brown pigmentation may aid in diagnosis and in sample selection for biopsy.

Familial reactive perforating collagenosis in two siblings

Reactive perforating collagenosis (RPC) is a rare form of transepithelial elimination disorder, in which genetically altered collagen is extruded through the epidermis. Of the acquired and inherited form, the latter is extremely rare. Here, we present two cases of inherited form of RPC in siblings aged 4 and 7 years presented with a history of itchy raised lesions of 9 months and 3 years duration, respectively. Dermatological examination revealed multiple papules with a central keratotic plug distributed mainly over face and extensors of upper and lower extremities. Koebnerization was present. Skin biopsy revealed perforating collagen bundles in the upper dermis and epidermis.

A rare case of familial reactive perforating collagenosis

Reactive perforating collagenosis (RPC) is a rare disorder of transepidermal elimination in which genetically altered collagen is extruded out through the epidermis. This disease usually starts as asymptomatic or pruritic umbilicated papules on extensor aspects of extremities and face, and the lesions become more conspicuous with age. It has two forms: Acquired and inherited form, of which inherited form is extremely rare. Here, we document a case of inherited form of RPC in a 13-year-old boy having multiple asymptomatic hyperpigmented umbilicated papules with a central keratotic plug distributed chiefly over face and extensor aspect of upper and lower extremities since his infancy, with history of similar lesions in his 18-year-old elder brother.

Folliculocentric Lichen Sclerosus et Atrophicus in a 10-Year-Old Girl

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory scarring disease with a predilection for the anogenital area; however, 15%-20% of LSA cases are extragenital. The folliculocentric variant is rarely reported and less well understood. The authors report a rare case of extragenital, folliculocentric LSA in a 10-year-old girl. The patient presented to the dermatology clinic for evaluation of an asymptomatic eruption of the arms and legs, with no vaginal or vulvar involvement. Physical examination revealed the presence of numerous 2-4 mm, mostly perifollicular, hypopigmented, slightly atrophic papules and plaques. Many of the lesions had a central keratotic plug. Cutaneous histopathological examination showed features of LSA. Based on clinical and histological findings, folliculocentric extragenital LSA was diagnosed.

A rare case of familial reactive perforating collagenosis

Reactive perforating collagenosis (RPC) is a rare disorder of transepidermal elimination in which genetically altered collagen is extruded out through the epidermis. This disease usually starts as asymptomatic or pruritic umbilicated papules on extensor aspects of extremities and face, and the lesions become more conspicuous with age. It has two forms: Acquired and inherited form, of which inherited form is extremely rare. Here, we document a case of inherited form of RPC in a 13-year-old boy having multiple asymptomatic hyperpigmented umbilicated papules with a central keratotic plug distributed chiefly over face and extensor aspect of upper and lower extremities since his infancy, with history of similar lesions in his 18-year-old elder brother.

Clinical and dermoscopic correlation of reactive perforating collagenosis

The acquired perforating dermatosis is constituted by a group of diseases whose main characteristic is the transepithelial elimination of altered dermal material. It has the clinical appearance of crateriform papules or nodules, with central keratotic plug, which may suggest some kind of transepithelial purging; nevertheless the final diagnosis is histologic. The dermoscopic examination is a useful tool that helps to exclude similar dermatoses, such as nodular prurigo and hypertrophic lichen planus. The present article discusses the clinical and dermoscopic correlation of this dermatosis.

Elastosis perforans serpiginosa, a transepithelial elimination skin disease diagnosed by histopathology: case report

Elastosis perforans serpiginosa is a disease that belongs to the group of perforating dermatoses, in which dermal elastic tissue extrusion occurs through epidermis. It generally affects young male individuals, and is clinically characterized by keratotic papules of verrucous aspect with centrifugal and serpiginous growth. We report the case of a male patient presenting with papular keratotic lesions since the age of 5. Some of the lesions display a central keratotic plug and converge into circular, arciform and serpiginous lesions.

Kyrle disease: report of a Tunisian case

Kyrle disease (KD) was first described in 1916 by Kyrle under the name of hyperkeratosis follicularis and parafollicularis in cutem penetrans [1]. As he described, this is a disorder characterized by the formation multiple hyperkeratotic follicular and parafollicular papular lesions with a central keratotic plug. It is considered as a variant of primary perforating dermatoses which share the same pathophysiology: a transepidermal elimination of dermal substances [2]. The prognosis depends essentially on the association with some systemic disorders such as diabetes mellitus, renal failure, liver disease, congestive cardiac failure hyperlipidemia, etc [2].

A case of keratoacanthoma associated with Basal cell carcinoma.

Dear Editor: Keratoacanthoma is a relatively common epithelial tumor that originates in the pilosebaceous glands and bears a pathological resemblance to squamous cell carcinoma (SCC). Several cases of keratoacanthoma in association with other lesions such as SCC, nevus comedonicus, verrucous cell carcinoma, and cutaneous horn have been reported. Herein, we report a rare case of keratoacanthoma and basal cell carcinoma (BCC) present in a single tumor nest. A 46-year-old male patient presented with a rapidly growing lesion on his left cheek, which had developed 6 weeks previously. He had no relevant medical or family history. Physical examination revealed a 6-mm-sized, protruding verrucous papule on the cheek (Fig. 1A). The edges were raised and the central portion was crateriform with a keratin plug. A 4-mm punch biopsy was performed with a clinical impression of verruca vulgaris or keratoacanthoma. Biopsy specimen results were consistent with keratoacanthoma (Fig. 1B), without features of solar elastosis or basal layer dysplasia. Complete removal of the keratoacanthoma was performed by surgical excision. The excision specimens were composed of two sections. One section exhibited remnant keratoacanthoma, whereas the other section was consistent with BCC (Fig. 2). The BCC contained palisading basaloid cells and a cleft between the tumor nest and stroma. Fig. 1 (A) A 6-mm-sized, protruding, verrucous papule on the left cheek. (B) Central keratotic plug surrounded by epithelial proliferation (H&E, ×20). Fig. 2 Single tumor nest with abundant sebaceous glands and hair follicles (H&E, ×100). Basal cell carcinoma showing palisading basaloid cells with a cleft between the tumor nest and stroma (inset, ×200). A cutaneous collision tumor is the coexistence of more than one neoplasm in a single cutaneous specimen. In most instances, collision tumors are composed of two different tumor portions. To the best of our knowledge, there have been three previously reported cases of keratoacanthoma associated with BCC. In two reports, the keratoacanthoma and BCC lesions were distinct and separate in a single tumor nest1,2, which may be similar to our case. In another report, a histologic transition between the two lesions was observed3. The lesions reported in these three cases were located on the face, specifically on the eyelid, nose, and cheek. Given the small number of cases reported in the literature, the pathogenesis of collision tumors is controversial. One theory suggests that cumulative damages to the skin may induce the development of different neoplasms adjacent to each other4. Considering the risk factors for keratoacanthoma and BCC, we believe that in our case, the collision developed incidentally, due to chronic ultraviolet light exposure. The collision tumor in our case and those of the three previous reported cases1,2,3 were all located on the face. This also supports the above theory. As in our case, we believe that most cutaneous collision tumors are coincidental. However, there are other theories about collision tumor pathogenesis. One suggests that collision tumors may develop due to an interaction between the different tumor portions4. Another theory suggests that the different neoplasms of collision tumors may arise from multipotent stem cells5. In conclusion, we report a rare case of collision tumor composed of keratoacanthoma and BCC, although direct contact between the two lesions was not observed. Further studies will be needed to elucidate the pathogenesis of collision tumors.

Acquired Perforating Osteoma Cutis.

Dear Editor: Osteoma cutis is a rare, benign condition characterized by dermal or subcutaneous bone formation1 that presents as stony hard nodules2. Although the mechanism of bone formation remains unclear, it is speculated that resident fibroblasts or nests of pleuripotent mesenchymal cells differentiate into osteoblasts to form bone tissue2. This disease can be divided into two types according to origin. The primary type, which accounts for 15% of cases, occurs in the absence of predisposing factors. The secondary type, also known as the acquired type, occurs in cases involving preexisting inflammatory or neoplastic disease, or trauma2. Herein, we report a case of acquired perforating osteoma cutis probably due to chronic irritation. A 40-year-old woman with diabetes mellitus had a solitary 1×0.5-cm erythematous nodule and central keratotic crater on her left breast for 20 years (Fig. 1). She reported that the lesion thickened and oozed fluid for 2 years. Laboratory test results for thyroid and parathyroid hormone were within normal limits. Mammography revealed solitary calcification at the same site, which was interpreted as a phlebolith. Excisional biopsy was performed for diagnosis and removal. The specimen had a central keratotic plug comprising osseous tissue with surrounding inflammatory cells (Fig. 2A) and bony nodules perforating the overlying epidermis (Fig. 2B). Von Kossa staining showed calcium deposition (Fig. 2C). On the basis of these findings, we diagnosed the patient with acquired perforating osteoma cutis. Fig. 1 A solitary, oozing, 1×0.5-cm erythematous papule and central keratotic crater on left the breast. Fig. 2 (A) A central well-demarcated keratotic plug in the epidermis, granulomatous inflammation and fibrosis in the dermis (H&E, ×40). Inset: the calcified plug was composed of osteoblasts, osteoclasts, and osteocytes (H&E, ×400). ... Perforation commonly occurs in perforating collagenosis, perforating folliculitis, granuloma annulare, and calcinosis cutis1. Perforating osteoma cutis is a very rare condition; few cases have been reported1,3,4. Several potential mechanisms of perforation have been described: (a) transepidermal elimination, which is a dermal-induced epidermal reaction to eliminate foreign bodies, degenerated connective tissue or keratin, calcium deposits, and/or ectopic tissue5; (b) the pressure effect from underlying tissue1; (c) simple upward growth of underlying tissue; and (d) superficial inflammation caused by external trauma, leading to the degeneration and perforation of the epidermis5. We speculate persistent irritation by brassieres might have induced superficial inflammation and stimulated the differentiation of cells into osteoblasts in the present case. Calcification occurred much later, and calcium-containing ectopic bone tissue induced transepidermal elimination. In addition, the pressure effect and simple upward growth of bony nodules might have facilitated epidermal perforation. In the present case, the presence of clustered osseous tissue with surrounding dermal inflammatory cells supports the idea that the lesion was secondary to trauma3. Furthermore, von Kossa staining revealed calcium deposition, which indicates the chronic phase of ossification. Osteoma cutis is rare condition often misdiagnosed as thrombophlebitis on radiography. In the present case, a radiologist interpreted the calcification observed on mammography as a phlebolith. The accurate diagnosis of osteoma cutis requires histopathologic confirmation or bone scan. Surgical removal is commonly performed to treat osteoma cutis2. Our patient was successfully treated with excision, and no recurrence has been noted during 6 months of follow-up. In summary, we report a case of secondary perforating osteoma cutis probably due to chronic irritation. Histopathologic confirmation of bone tissue or bone scan is required to establish a definite diagnosis of osteoma cutis.

A slowly enlarging mass on the finger

An otherwise healthy 71-year-old woman presented with an asymptomatic mass on her right 4th finger that had been slowly enlarging over the past 2 years. On physical examination, there was a tan-pink, freely mobile, rubbery 9-mm papule with a central keratotic plug located over the extensor aspect of the proximal interphalangeal joint of the right 4th finger. A 3-mm punch biopsy of the lesion was performed. Histopathologic findings were consistent with a diagnosis of aggressive digital papillary adenocarcinoma (ADPAca), an adnexal tumor with high metastatic potential that is unfortunately commonly misdiagnosed owing to its rarity and nondescript appearance. Aggressive surgical treatment including radical excision or digital amputation with or without sentinel lymph node biopsy is advocated in the treatment of ADPAca.

Generalized eruptive keratoacanthoma: proposed diagnostic criteria and therapeutic evaluation.

Generalized eruptive keratoacanthoma (GEKA) of Grzybowski is a sporadically occurring, extremely rare variant of keratoacanthoma characterized clinically by severely pruritic, generalized eruption of numerous small follicular papules, often with a central keratotic plug, and histologically by typical features of solitary keratoacanthoma. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is not yet determined. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course and prognosis. Different therapeutic approaches and their impact on the course and prognosis of the disease are also evaluated and presented. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid overlapping and confusion with other types of multiple keratoacanthoma. The first set comprises constant clinical and histopathological features that almost always present in every case and the second set includes variable features that were reported in some patients, and to which any emerging finding could be added to avoid missing cases. Although different therapeutic options have been used, either as single agents or in combinations, there is no standard therapy for GEKA and the disease still represents a therapeutic challenge.

Generalized eruptive keratoacanthoma of Grzybowski: strict diagnostic criteria are still lacking

Conflict of interest: none declared. We read with interest the article by Davies and Davies on multiple eruptive keratoacanthoma (KA) entitled ‘A widespread, itchy papular eruption’,1 recently published in Clinical and Experimental Dermatology. In this article, Davies and Davies have described a case of multiple eruptive KA, and classified it as KA of Witten and Zak. However, we do not agree with this concept, and believe that this case is more consistent with the diagnosis of generalized eruptive keratoacanthoma (GEKA) of Grzybowski. As described, the patient was elderly (81 years), presented with hundreds of erythematous small follicular papules (< 5 mm in size) with a central keratotic plug, and had only two nonulcerating larger nodules. The distribution of the lesions was generalized, itching was a prominent feature, and the course was progressive. Based on the available literature,2,3,4,5 these features were always present in the reported cases of GEKA, and seem to be consistent features necessary for the diagnosis of the disease (Fig. 1). Other reported features, such as mucosal involvement, ectropion and masked facies, may be considered as variable features. Moreover, the absence of either a positive family history or of multiple large, noduloulcerative and destructive tumours argues against the diagnosis of multiple KA of Witten and Zak in this case, and helps to differentiate between these two types of KA (Table 1).

A rare case of familial reactive perforating collagenosis

A 4 year old boy presented with history of itchy raised lesions on body of 2 years duration. Though parental consanguinity was not present, his elder brother had similar complaints. Dermatological examination revealed multiple hyperpigmented papules with a central keratotic plug distributed mainly over face and extensors of upper and lower extremities. Koebnerisation was present. Skin biopsy revealed perforating collagen bundles in the upper dermis and epidermis which was confirmed by Van Gieson staining. Patient was being treated with topical retinoids and intralesional corticosteroids with minimal relief.

Um caso de dermatose perfurante adquirida

We present a case of acquired perforating dermatosis (also known as acquired perforating collagenosis) which is frequently associated with diabetes mellitus and chronic renal failure. A 61 year old diabetic woman had a history of umbilicated, pruritic papules and nodules, with a central keratotic plug, located on the extensor surface of the lower limbs. The diagnosis of acquired perforating dermatosis was made six years after the initial clinical presentation. This article seeks to alert physicians to the existence of this under-diagnosed condition with considerable psychosocial impact.

Acquired reactive perforating collagenosis

A 58-year-old woman with diabetes mellitus and chronic kidney disease who was receiving hemodialysis presented with a five-month history of pruritic skin lesions on her upper back. Examination showed numerous erythematous papules and plaques with central keratotic plugs ([Figure 1][1]).

Rare presentation of Kyrle's disease in siblings.

Background:Kyrle's disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings.Materials and Methods:Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction.Results and Conclusions:A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle's disease. This helps to differentiate the rare primary Kyrle's disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.

Multiple keratoacanthomas in a young woman: report of a case emphasizing medical management and a review of the spectrum of multiple keratoacanthomas

A 27-year-old white woman was referred for consultation with regard to the presence of extensive multiple keratotic lesions. She began to develop these lesions at the age of 9 years, with healing of the lesions resulting in scar formation. A biopsy was performed at the age of 16 years, but the patient was unsure of the results. Since then, she had not had any treatment or biopsies, and stated that she had not suffered from any health problems during the intervening period. She was most concerned about the tumors on her heels and soles, which caused difficulty with ambulation. The family history was negative for skin diseases, including melanoma, nonmelanoma skin cancer, psoriasis, and eczema, and positive for Type II diabetes mellitus. A relative reported that the patient's grandfather had similar lesions, but the patient's parents and siblings were healthy. She was married and had one child, a 9-year-old daughter. Her child had no skin lesions. The patient's only medication was Ortho-Tricyclene birth control pills. She had no known drug allergies. Physical examination revealed the presence of multiple lesions on her body (Fig. 1). Her left superior helix contained a well-demarcated, dome-shaped nodule with a rolled, mildly erythematous border with a central hyperkeratotic plug. A similar lesion was present in the scaphoid fossa of the left ear and smaller lesions were scattered on her face. Numerous lesions were present on the arms and legs bilaterally, with the majority of lesions being located on the anterior lower legs. There were also lesions present on the palms and soles. The lesions ranged in size from 5 mm to 3 cm, the largest being a verrucous exophytic nodule on the anterior aspect of her left leg. Overall, there appeared to be two distinct types of lesion. One type appeared round, oval, and symmetric with a central keratotic plug, similar to that on the ear. The other type was larger, more exophytic, and verrucous, including the lesions on the volar surfaces. Also present were numerous, irregularly shaped atrophic scars where previous lesions had healed spontaneously. There were no oral lesions or lesions on her fingernails or toenails, and her teeth and hair were normal. A biopsy was obtained from an early lesion on the right dorsal forearm. Histology revealed an exo-/endophytic growth having a central crater containing keratinous material (Fig. 2). The crater was surrounded by markedly hyperplastic squamous epithelium with large squamous epithelial cells having abundant glassy cytoplasm. Some cells were dyskeratotic. Within the dermis was a dense, chiefly mononuclear inflammatory infiltrate. A buttress of epidermis surrounded the crater. The clinical and pathologic data were consistent with keratoacanthomas. Initial laboratory screenings revealed elevated triglycerides and total cholesterol, 537 mg/dL (normal, < 150 mg/dL) and 225 mg/dL (normal, < 200 mg/dL), respectively, with all other laboratory results within normal limits. In anticipation of starting oral retinoid therapy for her multiple keratoacanthomas, she was referred to her primary care physician for control of hyperlipidemia. After her lipids had been controlled, she was placed on isotretinoin (Accutane) 40 mg/day. There was some interval improvement with regression of some lesions leaving atrophic scars. She was also started on topical application of tazarotene (Tazorac) for all nonresolving lesions. Possible side-effects from the isotretinoin occurred, including dry mouth and eyes. After 8 months of isotretinoin, the patient was switched to acitretin (Soriatane) 25 mg to determine whether it might have a more beneficial effect on the resistant lesions. Many of the larger lesions regressed leaving atrophic scars. The dose of acitretin was subsequently increased to 35 mg because the lesions on her heel and the ball of her foot persisted. Almost all of the lesions resolved, except those on her feet, which are slowly regressing. Currently, the patient is on a regimen of acitretin 25 mg once a day with tazarotene 0.1% gel applied directly to the few residual keratoacanthomas on her feet, which are slowly improving.