Atrophic Plaques Research Articles

Congenital Dermatofibrosarcoma Presenting as an Atrophic Plaque

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma that most commonly occurs on the trunk of adult patients. However, DFSP can present congenitally, and this rarity may lead to misdiagnosis or late diagnosis. The authors present a case of a congenital DFSP that was not diagnosed until the patient was 17 years old. Her tumour was successfully treated with Mohs micrographic surgery. It is important to have DFSP on the differential for atrophic plaques or violaceous to red-brown nodules, even at a young age.

Deep Fungal Infections of Skin and Role of Histopathology in Diagnosis

Abstract Introduction: Deep mycoses acquired by penetrating trauma to the skin can have varied and sometimes atypical morphological presentations resulting in diagnostic dilemmas and delay in treatment onset. Histopathology can be a useful tool in not only diagnosing but also differentiating various deep mycoses. Aims and Objectives: To observe various morphological presentations and histopathological features of deep fungal infections. Materials and Methods: A retrospective multi-centric study was conducted from 2010 to 2020 at 16 centres. The cases with diagnoses of various deep mycoses were included in the study. The patients presenting with cutaneous manifestations were included in the study. Their demographic details, history, presenting signs and symptoms, morphological presentations, histopathological features and treatment details were collected from the case sheets. Results: A total of 124 cases were found from the case records. The most common type was chromoblastomycosis (42) followed by mycetoma (28) and rhinosporidiosis (17). The mean age was 43.76 ± 5.44 years. The average duration of symptoms before presentation was between 2 months to 10 years (average 2.5 ± 1.33 years). Male to female ratio was 1:0.7. Prior history of trauma was recorded in 36% of cases. Chromoblastomycosis cases presented with verrucous to atrophic plaques with black dots on the surface and histopathology findings included pesudoepitheliomatous hyperplasia, epithelioid cell granulomas, copper penny bodies within granulomas and abscesses. Rhinosporidiosis cases had polypoid grape-like lesions in the nose and eyes most commonly with histopathology findings of abundant thick-walled sporangia in dermis packed with thousands of spores. Eumycetoma patients had pigmented, indurated swelling with multiple sinuses discharging black granules and histopathology showed dermal abscesses and foreign body granulomatous reaction with PAS-positive hyphae. Histoplasmosis patients presented with few to multiple nodulo-pustular lesions on skin and palatal ulcers while small basophilic bodies packed in the cytoplasm of histiocytes were noted in histopathology. Phaeohyphomycosis cases presented as deep-seated cystic lesions and biopsy revealed deepithelialized cysts in the dermis or hypodermis with lumen showing necro inflammatory debris and fungal hyphae. Sporotrichosis cases had erythematous, tender nodules and papules either as single lesions or as multiple lesions arranged in a linear fashion and histopathology showed pseudoepitheliomatous hyperplasia of epidermis, loose to well-defined epithelioid cell granulomas and microabscesses. Spores were found in two cases. Cryptococcosis patient had multiple umbilicated lesions resembling giant molluscum contagiosum loose epithelioid cell granulomas and medium-sized spores lying in both intra and extracellularly on histopathology. Penicilliosis patients had nodulo-pustular lesions and histopathology showed an admixture of histiocytes, epithelioid cells, plasma cells, lymphocytes and polymorphs in the dermis with the presence of yeast-like spores in the cytoplasm of histiocytes and epithelioid cells. Entomophthoromycosis cases presented with asymptomatic subcutaneous firm swelling with loss of skin pinchability. Conclusion: Though clinical findings of deep fungal infections are characteristic similar morphology and atypical presentations can be sometimes confusing. Histopathology is useful for confirming the diagnosis.

An Unusual Skin Finding in a Rare Case of Goltz Syndrome

Abstract Introduction/Objective Goltz Syndrome (Focal Dermal Hypoplasia) is a rare X linked dominant genetic disorder with various dermatologic, musculoskeletal and neurologic manifestations, secondary to a mutation in the PORCN gene. Skin findings include telangiectasias, raspberry-like papillomas (angiofibroma like lesions), nodular fat herniation, xerosis and photosensitivity. Lesions are bilateral, and asymmetrically distribututed along the lines of Blasco. The adipocytic lesions can be diagnosed via a skin biopsy showing adipose tissue in the superficial epidermis and dermis or via molecular techniques showing a mutation in the PORCN gene. Here we report a case of Goltz Syndrome with unique dermal findings. Methods/Case Report A male infant presented at birth with mild diffuse xerosis, large hypopigmented and depigmented atrophic patches and plaques and multiple pink yellow red exophyitic soft papules distributed asymetrically across the body along the lines of Blascho. Additionally the patient had clinodactly, widely spaced nipples, extra pads of fat in the toes and philtrum and fingernails with longitudal ridges and lateral nail plate deviation. A diagnosis of Goltz syndrome was made via molecular analysis that showed mosiacism at C.502G>A (p.Gly168Arg) in the PORCN gene. The patient had a very superficial 2-3 mm erosion at the intersection between the scrotum and perineum. At 18 months, this had grown to finger like projections and eventually grew into multiple tan red exophytic warty papillomatous telangactatic like lesions measuring 3.0 x 0.8 cm that were painful and bled easily. A biopsy of this lesion showed focal mild epidermal hyperplasia and elongation of the rete ridges with wide spread diffuse vascular proliferation with multiple ectactic vessels along with mild dermal and perivascular inflammatory infiltrates. Also noted was infiltration of mature adipose tissue in the superficial epidermis and dermis. We preformed elastin and trichrome stains to identify if this lesion was reactive in nature or de novo. Our results showed retained elastin staining with a negative trichrome eluding to a denovo process. Additionally GLUT1 was negative, ruling out an infantile hemangioma. The above findings in this lesion do not represent any previously described dermatologic entity. Results (if a Case Study enter NA) NA Conclusion This is a rare and unique dermatologic finding of a case of Goltz sydrome, which itself is a rare disorder. In our literature review, this is the only case of Goltz Syndrome thusfar to have presented with this unique skin lesion.

Multifocal linear morphea - a rare presentation with rapid progression and deformities in a young adult: a case report

Morphea or localized scleroderma, is an uncommon skin disorder characterized by skin thickening and hardening. This case report describes a late 20s married woman working at a spinning mill, who presented with a unique and rare manifestation of morphea. Over two years, she developed a linear black color thickening of the skin on her right upper limb, left lower limb, and abdomen, accompanied by deformities and functional impairments. The patient reported a rapid progression of hyperpigmented lesions and weakness, prompting medical consultation. Cutaneous examination revealed distinctive hyperpigmented, atrophic plaques with xerotic changes and flexion deformities. A biopsy from the chest confirmed localized scleroderma. Basic investigations were within normal limits. The patient was managed with weekly pulse therapy involving corticosteroids, resulting in some relaxation of skin tightness. This case highlights the importance of recognizing and promptly treating rare multifocal morphea presentations to prevent severe deformities and enhance the patient's quality of life. Long-term follow-up is essential to monitor treatment outcomes and disease progression.

EXTRAGENITAL LICHEN SCLEROSUS ET ATROPHICUS : A CASE REPORT

Lichen sclerosus et atrophicus (LSA) most commonly presents as atrophic plaques in the genital region but rarely can occur in extra-genital locations (1), reportedly present in 6% to 20% of patients. Its a pathology that can be underdiagnosed and under-treated. The diagnosis should be confirmed with a skin biopsy, and early, thorough treatment should be initiated.(2)We report a case of lichen sclerosus et atrophicus in a woman with purely cutaneous involvement.

P25 Two cases of multicentric dermatofibrosarcoma Protuberans (DFSP) and adenosine deaminase deficient-severe combined immunodeficiency (ADA-SCID) in a tertiary centre

Abstract Dermatofibrosarcoma Protuberans (DFSP) are uncommon soft tissue sarcomas that are rarely metastatic though usually high-risk for local aggression and recurrence. DFSP is associated with t(17;22)(q22;q13) chromosomal translocation resulting in COL1A1-platelet-derived growth factorβ(PDGFB) fusion gene(Kesserwan et al. 2009). We present two patients referred to Dermatology at very different ages (patient A 12 years; patient B 6 months) with atrophic plaques and atypical nodules respectively, later demonstrated to be rare cases of multicentric DFSPs. Both were known adenosine deaminase deficient-severe combined immunodeficiency patients (ADA-SCID), associated with multiple additional pathologies(Ochs et al. 2007). Patient A had been treated aged 6 months by undergoing bone marrow transplant (BMT) from an unrelated donor, but did not present with skin lesions until ∼11 years later, whereas Patient B was referred to our centre from another country for BMT and had lesions incidentally detected whilst temporarily in the UK. Histology for both patients demonstrated a paucicellular highly infiltrative spindle cell proliferation in the deep dermis and subcutis and CD34 positivity, consistent with DFSP. Patient A’s tissue was sent to the Northern Genetics Service and the COL1A1-PDGFB fusion gene was detected. Both patients were referred to plastic surgery for multiple excisions (A-3; B-10 lesions). Patient A has since had two further plastic surgery procedures for further or wider excisions. An association between DFSP and ADA-SCID has become more widely reported, though as is highlighted by our two very different cases little is known about timing of presentations, optimal treatment and prognosis.

Punch grafting for the treatment of ulcerated atrophie blanche.

Case report: A 79-year-old woman presented with a large painful ulcer on the lateral aspect of her left leg over a 6-month period and was diagnosed of ulcerated atrophie blanche. On an outpatient basis punch grafting was performed and 3weeks after, complete epithelization was achieved. Discussion: Ulcerated atrophie blanche is a misdiagnosed disorder with painful lesions and, consequently, a high impact on quality of life. Atrophie blanche describes porcelain-white colored, red-dotted atrophic plaques on legs or feet. It may be due to multiple causes, usually associated with alterations in the microcirculation. All causes of atrophie blanche can be included in the term livedoid vasculopathy, a type of occlusive vasculopathy without vasculitis. Many patients with atrophie blanche and livedoid vasculopathy have also chronic venous insufficiency. Etiological treatment should be prescribed in order to avoid progression of the lesions. In case of chronic venous insufficiency, control of venous hypertension is essential. Without anti-edema measures, superficial, very painful, and resistant ulcers may appear. These ulcers can be considered a wound on scar tissue; therefore, it must be treated as a hard-to-heal wound. As we show in this case, punch grafting is an effective therapeutic alternative for wound closure and pain reduction of ulcerated atrophie blanche.

Acquired Cholesteatomas Behind Intact Tympanic Membranes of Adults: A Series of 13 Cases.

Background and Objective: Acquired cholesteatomas behind intact tympanic membrane (ITMC) are rarely encountered in adults in the otology clinic, and the clinical and imaging features of ITMC in adults remain unclear to date. ITMC patients are commonly overlooked because of the absence of both a typical retraction pocket and a tympanic membrane (TM) perforation. The objective of this study was to describe clinical features, pathogenesis, and surgical outcomes in a series of adult patients with acquired ITMC. Materials and Methods: The clinical records of 13 adult patients diagnosed with ITMC were retrospectively reviewed. ITMC were classified into those associated with otitis media with effusion (OME; Group A) and histories of previous perforations (Group B). Results: A total of 13 cases with unilaterally acquired ITMC were included, of which 10 (76.9%) were in Group A and 3 (23.1%) were in Group B. All Group A patients exhibited white masses behind intact TMs, in the anterosuperior quadrants of 6 and the posterior upper quadrants of 4. In Group B patients, white masses in the middle ear were lacking. The TMs were atrophic and white myringosclerotic plaques were evident. The cholesteatomas were confined to the antrum and aditus ad antrum in 2 Group B patients but extended to the epitympanum in 1. All 10 Group A patients had extensive cholesteatomas but intact (noneroded) malleoli. Conclusions: An acquired ITMC should be suspected if symptoms worsen or new symptoms develop in adults with OME or a history of perforation, although the TM is intact with or without pars flaccida retraction, computed tomography/magnetic resonance imaging should be performed. Canal wall up mastoidectomy combined with ventilation tube insertion effectively treated ITMC associated with OME.

Reflectance confocal microscopy features of vulvar lichen sclerosus in Chinese juvenile girls.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of unknown pathogenesis, characterized by porcelain-white atrophic plaques around the vulvar and anal areas in girls. With this communication, we performed the study on 16 female girls with clinically and histologically confirmed VLS, described the main identifying characteristics of the lesions in reflectance confocal microscopy (RCM) and elucidated the corresponding relationship between RCM findings and histology. We recommend RCM, a noninvasive technique, as a complementary diagnostic tool for VLS.

Patch-Type Granuloma Annulare with Clinical and Histological Features of Morphea: A True Overlap?

A 67-year-old woman was evaluated for a worsening asymptomatic rash located on her torso, back, and legs. She denied any chills or fevers, and reported feeling otherwise well. Clinically, large brownish, slightly atrophic plaques were seen on the torso suggestive of either morphea or a granulomatous condition. Histopathologic examination revealed an increase in interstitial histiocytes infiltrating between altered collagen fibers, palisaded granulomas with increased mucin, suggestive of granuloma annulare (GA), as well as dermal sclerosis, perineural infiltrates with plasma cells and diminished CD34 counts, that are more typical for localized scleroderma/morphea. Morphea and patch-type GA may be indistinguishable clinically and share some histopathological features. This case demonstrates similarities between these conditions and features of both conditions in the same patient.

BI21 The development of diffuse extragenital lichen sclerosus in a patient on palbociclib and fulvestrant for metastatic breast cancer

Abstract A 59-year-old woman with skin phototype VI was commenced on palbociclib (cyclin-dependent kinase 4/6 inhibitor) and fulvestrant (oestrogen receptor antagonist) as chemotherapy agents for metastatic breast cancer. Two months later, she developed a new widespread rash. Past medical history included atopic dermatitis but no autoimmune conditions. Denosumab was her only other medication. On examination, she had hyperpigmented thickened plaques with purple hue and sclerotic plaques with cigarette wrinkling on her buttocks, arms, neck, abdomen and axillae. She also had widespread hypopigmentation. Biopsies showed hyperkeratosis and a lichenoid inflammatory reaction with pigmentary incontinence. Clinicopathological correlation supported the diagnosis of extragenital lichen sclerosus (EGLS). Palbociclib and fulvestrant were stopped and she noted improvement of her rash within 3 weeks. She was also managed with potent topical steroids, which eased her symptoms and further improved the appearance. Lichen sclerosus (LS) is a chronic inflammatory condition affecting the anogenital area. EGLS occurs in approximately 15–20% of those with LS, and only 6% of patients with EGLS do not have genital involvement at diagnosis. LS and EGLS are characterized by white papules, sclerosus and erosions with atrophic plaques. In the skin of colour, the presentation is varied, with hypo- or hyperpigmentation. Currently, the pathogenesis of EGLS is poorly understood, with studies suggesting an autoimmune aetiology, with possible genetic, infectious, hormonal, trauma and drug associations. In addition to specific autoantibodies, the absence of a suppressive function of regulatory T cells may contribute to the induction of autoimmunity. A recent association with immune checkpoint inhibitors (ICIs) further establishes this link. There are 12 case reports of ICIs and LS/EGLS, but cell cycle inhibitors associated with EGLS have not yet been reported (Silvestri M, Cristaudo A, Morrone A et al. Emerging skin toxicities in patients with breast cancer treated with new cyclin-dependent kinase 4/6 inhibitors: a systematic review. Drug Saf 2021; 44:725–32). Laboratory studies demonstrate increased T-cell activation with abemaciclib, a cell cycle inhibitor, which has been hypothesized to have other autoimmune drug associations (Truong K, Jones-Caballero M, Chou S et al. Lichen sclerosus and immune checkpoint inhibitors: a case and review of the literature. Australas J Dermatol 2023; 64:158–61). Other possible theories contributing to the development of EGLS include the Koebner effect of deep intramuscular injection of fulvestrant or subsequent low-oestrogen state. To the best of our knowledge, this is the first case of EGLS following these chemotherapy agents. This case hypothesizes the role of cell cycle inhibitors contributing to the aetiology in a mechanism similar to that of ICIs. It also demonstrates the difference in presentation of EGLS in skin of colour.

DP13 A case of poikilodermatous plaque-like haemangioma: a newly described entity

Abstract A 56-year-old man was referred with a 1.5 cm × 1.5 cm asymptomatic erythematous and scaly plaque on his right upper calf of 6 months’ duration. He had no similar lesions or prior skin issues. He had no significant comorbidities and was on no regular medications. There was no known family history of relevance. The initial clinical findings were felt to be in keeping with Bowen disease. He was treated once with cryotherapy, followed by two cycles of 5-fluorouracil cream, applied once daily over 4 weeks. Despite this, the plaque persisted and increased in size, measuring 3 cm × 2.5 cm. A 4-mm incisional biopsy was performed. Histopathology findings showed an acanthotic epidermis with compact hyperkeratosis, overlying a superficial dermal vascular lesion that consisted of a distinctive band-like proliferation of small endothelial cell-lined vessels. Background fibrosis and oedema were noted, as well as loss of elastic fibres within the lesional area, demonstrated by elastic van Gieson stain. Positive immunohistochemistry staining included CD31 and erythroblast transformation-specific-related gene stains, helping to confirm this lesion’s vascular origin, with a negative result for D2-40 (podoplanin), suggesting no lymphatic involvement. The histological appearances were thought to fit with a diagnosis of poikilodermatous plaque-like haemangioma (PPLH), a newly described entity of acquired vascular proliferation. Poikilodermatous plaque-like haemangioma, first described in 2019, usually presents as a solitary atrophic erythematous-to-violaceous plaque typically found in the lower limbs (Semkova K, Carr R, Grainger M et al. Poikilodermatous plaque-like hemangioma: case series of a newly defined entity. J Am Acad Dermatol 2019; 81:1257–70). Reassuringly, it has a benign course, but it can be persistent (Semkova et al.). Our patient was keen for treatment as the lesion was continuing to grow, causing him concern. However, there is sparse information available in the literature regarding effective treatments for PPLH. The case was discussed at the departmental clinicopathological meeting. The plaque was felt to be too superficial to merit any radiological investigation of the soft tissues. Surgical excision was discussed but given the size and location would most likely require a large skin graft. Sclerotherapy was considered but not felt to be appropriate in this setting. A trial of laser therapy was regarded as the best option given the superficial nature of this vascular lesion. He has since started a course of pulsed dye laser (PDL) at a wavelength of 585 nm, and we wait with interest to determine if it is effective. We therefore present this case to raise awareness of PPLH as a newly described entity and to consider it in the differential of a solitary atrophic erythematous plaque on a sun-protected site.

Post-traumatic ulcerated and chronic necrobiosis lipoidica of the elbow: A new entity?

BackgroundNecrobiosis lipoidica (NL) is a chronic granulomatous dermatosis usually affecting the lower limbs, although less common sites have been described. Herein we report a series of cases of NL located on the elbow, with an unusual presentation and occurring after trauma or surgery. ObservationsOur series includes three men and one woman, with a mean age of 64 years. Three had undergone surgery for elbow bursitis and one had had trauma after a fall from a horse, with exposure of subcutaneous tissue prior to healing. Within 5 years, they had all developed an atrophic erythematous annular plaque with papular and telangiectatic edges, with recurrent episodes of ulceration and scarring. Repeated tests for infectious agents were negative. Histological examinations showed granulomas and necrobiosis with palisading or early-stage palisading. Partial healing was achieved in two patients after 6 months of doxycycline. Treatment with adalimumab resulted in disappearance of the ulcers at 6 months in one patient. DiscussionUnusual sites of NL impose consideration of other types of palisading granuloma or mycobacterial infections, which we were able to rule out. Two other cases of NL of the elbow similar to ours are reported in the literature. These cases, involving multiple ulcerations over a very long period of time, probably constitute a distinct entity because of the very distinct character of these 6 cases. Tetracyclines are partially active and tumour necrosis factor alpha (TNF)-alpha inhibitors may offer an option.

Atrophic violaceous plaques as the first manifestation of a disorder of GNAS inactivation.

We report the case of a 10-month-old girl who presented with failure to thrive and multiple small atrophic violaceous plaques, with no other findings on her physical examination. The laboratory examinations, abdominal ultrasound and bilateral hand radiography performed were unremarkable. The skin biopsy revealed fusiform cells and focal ossification in the deep dermis. The genetic study showed a pathogenic variant of GNAS.

Congenital Atrophic Dermatofibrosarcoma Protuberans: A Case Report and Review of the Literature

Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchymal tumor of intermediate malignant potential. The neoplasm is locally aggressive with a high rate of recurrence. It typically presents in adults. Atrophic congenital DFSP is extremely rare. The few reported cases have presented as a morphea-like plaque that persists for years, before progressing into a nodular form. To our knowledge, congenital atrophic DFSP has been only reported fourteen times, and of those, only nine were confirmed by molecular studies. Herein we report a congenital case of atrophic DFSP, which initially presented as a bruise-like atrophic plaque on the dorsal forearm, initially mistaken for child abuse. The clinical appearance, histopathology, and molecular features of this rare form of DFSP are reviewed. Our case highlights the importance of early detection and adequate sampling of congenital DFSP; early treatment allows for treating small lesions without large, disfiguring, and potentially disabling excisions.

ACTINIC CHEILITIS – A CASE REPORT

Actinic cheilitis (AC) is a chronic inammatory disorder that occurs mainly occurring in the lower lip in susceptible individuals. The susceptible individuals include people with fair complexion, people engaged in more of outdoor activities and those with eversion of lips. It is usually caused by chronic and excessive exposure of the lips to solar ultraviolet (UV) radiation. The lesion is potentially malignant and may transform into squamous cell carcinoma (SCC). The Clinical features involve diffuse and poorly demarcated atrophic, erosive or keratotic plaques which may affect a little or the entire vermilion border of the lower lip. This is a case report of actinic cheilitis, its clinical features and treatment options

Cutaneous Sarcoidosis Masquerading as Papular Amyloidosis Revealed Post--Hair Removal.

Cutaneous Sarcoidosis Masquerading as Papular Amyloidosis Revealed Post--Hair Removal.

A linear atrophic and hyperpigmented plaque on the face

A linear atrophic and hyperpigmented plaque on the face

Hamartome angio-eccrine : A propos de deux observations a Dakar, Sénégal

Angio eccrine hamartoma (EAH) is a rare, benign tumoral lesion composed of eccrine glands and vascular structures in the mid dermis. We report on two observations in Dakar. Observation 1: A four-month-old infant was seen for an erythematous plaque, rounded, with a smooth surface, asymptomatic, localized to the right thigh, evolving for three months. The clinical appearance suggested a spitz tumour. Cutaneous histology confirmed the diagnosis of angioeccrine hamartoma. Observation 2: The second case was concerned with a ten-month-old infant received for an erythematous, atrophic plaque, localized at the level of the inter-gluteal fold, evolving since birth. This clinical aspect was suggestive of a sclero-atropic lichen. Cutaneous histology confirmed the diagnosis of angioeccrine hamartoma. Discussion: We reported two observations of HAE original by the rarity and the clinical presentations leading to confusion with a nevus of Spitz and the sclero-atrophic lichen. Cutaneous histology was of considerable contribution.

Atrophic plaques on the trunk of an 8-year-old.

Atrophic plaques on the trunk of an 8-year-old.