Two cases of dystrophic calcinosis cutis in burn scars

Dystrophic calcinosis cutis is defined as the abnormal deposition of insoluble calcium salts in dead or degenerated cutaneous tissues in the absence of abnormal serum calcium or phosphate concentrations. Although dystrophic calcification can occur in various diseases, its occurrence on a burn scar has rarely been reported in the dermatologic literature. Herein we describe two patients who presented with a solitary non-healing ulcer in a postburn scar, with histopathologic evidence of calcium deposition in the dermis.     

A case of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber's disease) with dystrophic calcinosis cutis and retinal lesions

A 65-year-old Japanese female developed a nodule on the distal interphalangealjoint of her right thumb. She also had multiple telangiectasias on her face, oral mucosa, tongue, nasal mucosa and upper extremities. A lip biopsy showed irregularly dilated capillaries and venules lined by flat endothelial cells. The nodule in the right thumb was resected. Histopathology revealed calcium deposits in the dermis. This case was diagnosed as dystrophic calcinosis cutis within hereditary hemorrhagic telangiectasia. Ophthalmologic examination showed bilateral retinal vascular lesions with dilated and tortuous retinal venules.     

Case of melorheostosis associated with ipsilateral verrucous epidermal nevus,linear connective tissue nevus,diffuse hyperpigmentation and hypertrichosis: A fortuitous coincidence?

Melorheostosis (MEL) is a rare benign bone disorder that can be associated with several anomalies, including vascular abnormalities, nevus sebaceus, unilateral nevoid telangiectasia, linear scleroderma and hypertrichosis. We report the case of a 6-year-old patient who showed an unusual co-occurrence of bone hyperostosis and different skin lesions affecting the same side of the body: MEL, verrucous epidermal nevus, connective tissue nevus, linear scleroderma-like disorder, hyperpigmentation and hypertrichosis. The spatial co-occurrence of these conditions made us speculate as to whether they originated from a common genetic mechanism or if their co-occurrence was completely accidental.     

Mycosis fungoides mimicking inflammatory linear verrucous epidermal nevus

We experienced an unusual case of mycosis fungoides with the clinical and histological features mimicking inflammatory linear verrucous epidermal nevus (ILVEN) in an 11-year-old boy. Localized linear multiple pruritic verrucous confluent papules and plaques appeared on the his left elbow, forearm and hand for 7 months. Skin biopsies showed characteristic findings of mycosis fungoides (e.g. Pautrier's microabscesses, follicular epitheliotropism, wiry bundles of collagen, etc.). T-cell receptor gene rearrangement analysis in the lesional skin demonstrated rearrangement of the gamma chain. RePUVA (systemic PUVA with retinoic acid) therapy improved his skin lesions and pruritus, but these progressed after discontinuation of treatment. Thus, lesions mimicking ILVEN can be an unusual and potentially misleading presentation of mycosis fungoides.     

Two cases of gigantic dystrophic calcinosis cutis caused by subcutaneous and/or intramuscular injections.

We describe two female patients with gigantic dystrophic calcinosis cutis caused by a large number of subcutaneous and/or intramuscular injections which they received when they were much younger. Laboratory data and physical examinations were generally within normal limits, and we detected no disease which might induce cutaneous calcification. There are many reports of dystrophic calcinosis cutis caused by injection of several kinds of drugs. However, we found no previous report describing a patient with calcinosis cutis induced by local tissue injury from a large number of injections and with extraordinarily widespread calcification at the injection sites. Because we do not know the exact drugs injected, it is difficult to say if a specific ingredient in the injections was related to this condition. We do know that a large number of subcutaneous or intramuscular injections were frequently administered to patients who had difficulty in maintaining venous infusions in the past, so there may be similar cases of dystrophic calcinosis cutis which have not been reported.     

Carbon dioxide laser: first-line therapy in vulvar inflammatory linear verrucous epidermal nevus

Inflammatory linear verrucous epidermal nevus (ILVEN) is normally associated with the failure of topical and systemic treatments and with recurrences on interruption of therapy. Many physical approaches have been used, but they generally resulted in varying rates of recurrence and unacceptable scarring. We reported a case of ILVEN treated with a single session CO(2) laser treatment. In our experience, CO(2) laser was quick, easy, effective, and safe; we therefore believe that this approach should be considered as a first-line surgical option in the treatment of genital ILVEN, particularly in cases of mucosal involvement.     

Inflammatory linear verrucous epidermal nevus treated successfully with crisaborole ointment in a 5‐year‐old boy

Inflammatory linear verrucous epidermal nevus (ILVEN) is a chronic, a linear, or whorled array of inflammatory, following the lines of Blaschko. Treatment of ILVEN is challenging with numerous therapies of varying degrees of success reported. We present a case of ILVEN in a 5‐year‐old‐boy, treated successfully with crisaborole 2% ointment. This brief report suggests that there may be additional cellular immunologic pathways responsible for the presentation of ILVEN that may be explained by management with crisaborole use.     

Study of a myo-inositol hexaphosphate-based cream to prevent dystrophic calcinosis cutis

Background Calcinosis cutis is a disorder caused by abnormal deposits of calcium phosphate in the skin and is observed in diverse disorders. Myo‐inositol hexaphosphate (InsP₆) is a diet‐dependent molecule found in all mammalian fluids and tissues, which exhibits an extraordinary capacity as a crystallization inhibitor of calcium salts. Objectives To establish the effects of topically administered InsP₆ cream on artificially provoked dystrophic calcifications in soft tissues. Methods Fourteen male Wistar rats were randomly assigned into two groups: control and treated groups. Rats were fed with an InsP₆‐free or phytate diet. Plaque formation was induced by subcutaneous injection of 0·1% KMnO₄ solution. From 4 days before plaque induction to the end of the experiment, control rats were treated topically with a standard cream, whereas treated rats were treated with the same cream with 2% InsP₆ or phytate (as sodium salt). Calcification of plaques was allowed to proceed for 10 days. InsP₆ in urine was determined. The plaques were excised and weighed. Results It was found that when InsP₆ was administered topically through a moisturizing cream (2% InsP₆‐rich), the plaque size and weight were notably and significantly reduced compared with the control group (1·6 ± 1·1 mg InsP₆‐treated, 26·7 ± 3·0 mg control). The InsP₆ urinary levels for animals treated with the InsP₆‐enriched cream were considerably and significantly higher than those found in animals treated topically with the cream without InsP₆ (16·96 ± 4·32 mg L^?1 InsP₆‐treated, 0·06 ± 0·03 mg L^?1 control). Conclusions This demonstrates the important capacity of InsP₆ as a crystallization inhibitor and also demonstrates that it is possible to propose topical use as a new InsP₆ administration route.     

Perianal verrucous epidermal nevus masquerading as warts

Verrucous epidermal naevus(VEN) is a rare form of epidermal naevus. We present a case of VEN occurring over the perianal region of a 7-year-old boy. The lesion was initially thought to be an area of chronic dermatitis; however it was refractory to treatment. Histopathology confirmed the diagnosis of VEN. VEN in the inguinogenital region may be misdiagnosed as flexural psoriasis, genital warts or sexual abuse. This is what precisely happened to our patient for a duration of more than 1 year.     

A dystrophic calcinosis cutis case treated with CO2 laser

Abstract

Calcinosis cutis is the deposition of insoluble calcium salts within cutaneous tissue. It may be divided into four major subtypes: dystrophic, metastatic, idiopathic, and iatrogenic. The most common subtype is dystrophic calcinosis cutis. It can occur as a result of local tissue injury. We herein present a child with dystrophic calcinosis cutis developed following trauma and successfully treated with CO₂ laser.     

Inflammatory linear verrucous epidermal nevus associated with erosive monoarthritis

Inflammatory linear verrucous epidermal nevus and linear psoriasis are different entities with clinical and histopathologic similarities. Isolated reports of inflammatory linear verrucous epidermal nevus with concomitant psoriasis or a positive family history of psoriasis have been described, and the possibility that inflammatory linear verrucous epidermal nevus may be a mosaic form of cutaneous psoriasis has been postulated. We report a 17‐year‐old boy with a congenital, linear, erythematous, keratotic plaque on the dorsum of the fifth finger of the left hand with ipsilateral nail dystrophy. Histopathologic examination showed epidermal hyperplasia with alternating orthokeratosis and parakeratosis. During follow‐up, he developed erosive monoarthritis of the distal interphalangeal joint. This case seems to confirm the association between inflammatory linear verrucous epidermal nevus and arthritis and supports a possible relationship between inflammatory linear verrucous epidermal nevus and psoriasis.     

Epidermolytic hyperkeratosis in inflammatory linear verrucous epidermal nevus

Epidermolytic hyperkeratosis presents with perinuclear vacuolization of the keratinocytes in spinous and granular layers, keratinocytes with ill-defined limits, which leads to a reticulate appearance of the epidermis, an increased number of variously shaped and sized basophilic keratohyalin granules and the same sized eosinophilic trichohyalin granules, at any level of epidermis, mainly in the stratum granulosum, and compact hyperkeratosis. This minor reactive pathologic reaction pattern of skin is found in large variety of diseases. This paper is the first case report of such pattern in inflammatory linear verrucous epidermal nevus. Our case is of a 23-year-old man with pruritic verrucous lesions of trunk and extremities initiated since 13 years ago. Physical examination revealed white linear hyperkeratotic lesions, some of them on erythematous background and also classic epidermal nevus. No skeletal, ophthalmic, and nervous system involvement was detected. Microscopic study of pruritic verrucous lesions showed psoriasiform acanthosis, mild papillomatous, hyperkeratosis, and epidermolytic hyperkeratotic changes in hair follicles and acrosyrinx accompanied with moderate perivascular inflammation.     

Milialike idiopathic calcinosis cutis and syringoma in Down's syndrome

A 6-year-old girl with Down's syndrome presented milialike whitish small papules on her hands and feet and periorbital syringoma. Histopathological examination of the hand lesion revealed small localized calcium deposits and syringoma in the adjacent upper dermis. This is a very rare but typical case of calcinosis cutis with syringoma in a patient with Down's syndrome.     

Disseminated linear calcinosis cutis associated with the Koebner phenomenon in an infant with congenital acute monocytic leukaemia

We present a unique case of an infant with acute monocytic leukaemia who presented at birth with multiple rubbery, erythematous to violaceous subcutaneous nodules secondary to leukaemia cutis. As these infiltrates regressed with chemotherapy, numerous white to yellow linear confluent papules appeared in a scratch-like pattern. These lesions were widely disseminated but were concentrated across her face, trunk and extremities with relative sparing of the napkin area and back. We propose that these lesions represent a form of dystrophic calcinosis cutis that occurred secondary to koebnerization in an infant with congenital leukaemia cutis.     

Inflammatory linear verrucous epidermal nevus: why a combined laser therapy

Abstract

Inflammatory linear verrucous epidermal nevus (ILVEN) is a benign cutaneous hamartoma, and more precisely an uncommon variant of the verrucous epidermal nevus. In our case report we describe an ILVEN female patient, resistant to previous treatments but responsive to a combined laser therapy, 10,600-nm CO₂ laser and Fractional CO₂ laser, with good and longstanding results. A complete resolution of the lesion was observed at the 9-month follow-up. The application of lasers has been reported in literature over recent years for resolving ILVEN lesions. Based on our clinical experience, 10,600-nm CO₂ pulsed laser therapy seems to be the best treatment and Fractional CO₂ laser treatment can be regarded as a very promising technique to combine with CO₂ laser for reducing pigment modifications and endowing a more youthful appearance to the treated areas.     

Adult‐onset inflammatory linear verrucous epidermal nevus: Immunohistochemical studies and review of the literature

Adult‐onset inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon cutaneous disease compared to childhood‐onset ILVEN. The typical histopathologic features are alternating parakeratosis and orthokeratosis with an absent granular layer underneath parakeratosis, in contrast to a thickened granular layer below the foci of orthokeratosis in psoriasiform epidermal hyperplasia. Herein, we present a 49‐year‐old woman with typical clinical and histopathologic characteristics of adult‐onset ILVEN, including linear arrangement of thick scaly papules and plaques localized on the medial side of her right leg, ankle, and foot. Immunohistochemical studies included involucrin, Ki‐67, and keratin‐10. Compared to the staining pattern in psoriasis, the expression of involucrin in this case was of lower intensity and localized to upper epidermal layers with relatively less extensive staining beneath regions of parakeratosis as compared to orthokeratosis; Ki‐67 showed lower basal layer proliferative activity; and keratin‐10 showed a greater intensity of staining within suprabasal epidermis.     

Successful treatment of verrucous epidermal nevus with fractional micro-plasma radio-frequency technology and photodynamic therapy

Verrucous epidermal nevus (VEN) is a skin disorder that commonly presents at birth; it is characterized by skin-colored to brown verrucous papules in a linear distribution following Blaschko’s lines. Even though it is extremely rare, VEN has been associated with malignant transformation. VEN has been treated by different treatment modalities with varying and frustrating results. We introduce a new type of treatment. The fractional micro-plasma radio-frequency (RF) technology, which uses unipolar RF technology to provoke plasma spars, creating multiple controlled micro-perforations on the skin. Photodynamic therapy (PDT) is a type of technology for disease diagnosis and treatment, in which a photosensitizer gathers within the nidus and kills the diseased cells. In this report, we present a case of VEN that was successfully treated with fractional micro-plasma RF technology and PDT without side effects or complications; a follow-up was conducted after 24 months and no signs of recurrence were observed.     

Giant proliferating trichilemmal cyst arising from a nevus sebaceus growing for 30 years

Nevus sebaceus of Jadassohn, a congenital cutaneous hamartoma, has the potential to develop into various epidermal adnexal‐origin neoplasms. While the most common neoplasms are trichoblastoma or syringocystadenoma, proliferating trichilemmal cysts are exceptionally rare. We report a case of a 63‐year‐old Cuban male with a giant proliferating trichilemmal cyst arising from a nevus sebaceus on the right shoulder which had been growing for 30 years. Proliferating trichilemmal cysts arising from nevus sebaceus cases are difficult to diagnose clinically and histologically as they are very rare and have not been defined by exact diagnostic criteria. Our case creates awareness of this particular tumor in nevus sebaceus and shares clinical and histological diagnostic information that can be used to make a proper diagnosis.