Hypertrophic Lupus Erythematosus Research Articles

The Spectrum of Cutaneous Manifestations in Lupus Erythematosus: A Comprehensive Review.

Lupus erythematosus (LE) is an autoimmune inflammatory disease with complex etiology. LE may present as a systemic disorder affecting multiple organs or be limited solely to the skin. Cutaneous LE (CLE) manifests with a wide range of skin lesions divided into acute, subacute and chronic subtypes. Despite classic forms of CLE, such as malar rash or discoid LE, little-known variants may occur, for instance hypertrophic LE, chilblain LE and lupus panniculitis. There are also numerous non-specific manifestations including vascular abnormalities, alopecia, pigmentation and nail abnormalities or rheumatoid nodules. Particular cutaneous manifestations correlate with disease activity and thus have great diagnostic value. However, diversity of the clinical picture and resemblance to certain entities delay making an accurate diagnosis The aim of this review is to discuss the variety of cutaneous manifestations and indicate the clinical features of particular CLE types which facilitate differential diagnosis with other dermatoses. Although in diagnostically difficult cases histopathological examination plays a key role in the differential diagnosis of LE, quick and accurate diagnosis ensures adequate therapy implementation and high quality of life for patients. Cooperation between physicians of various specialties is therefore crucial in the management of patients with uncommon and photosensitive skin lesions.

CPC05 A case of extensive hypertrophic lupus erythematosus responsive to topical steroids

Abstract Hypertrophic lupus erythematosus (HLE) is a rare variant of lupus erythematosus, occurring in 2% of cases of chronic cutaneous lupus erythematosus (CCLE). It is an exaggerated proliferative epithelial response manifesting as verrucous-appearing hypertrophic indurated lesions occurring on sun-exposed sites. We present a case of the development of extensive HLE in a patient with subacute cutaneous lupus erythematosus (SCLE) with underlying pulmonary malignancy. A 74-year-old woman with Sjögren syndrome presented to dermatology with a 2-month history of a rash affecting her face and décolletage. Laboratory studies illustrated elevated antinuclear and anti-Ro antibodies. Biopsy from her chest confirmed SCLE. Malignancy screening revealed T1aN0 pulmonary lepidic adenocarcinoma, which was successfully managed with a lower lobectomy. Hydroxychloroquine was initially trialled but required discontinuation following intolerable side-effects. Mepacrine was then initiated, which allowed clearance of the rash, alongside yellow discoloration of the skin. Six months later, she developed a raised pruritic rash on her arms, legs and buttocks, resistant to current management alongside a trial of mycophenolate mofetil. Examination revealed violaceous hypertrophic plaques affecting 40% of her body-­surface area. The differential included hypertrophic lichen planus, nodular prurigo and HLE. Repeat lesional biopsies highlighted a lichenoid inflammatory infiltrate with overlying scale, favouring HLE. Owing to patient choice, topical mometasone furoate 0.1% was used to manage cutaneous disease, with a reduction in lesion size. HLE is a distinct subset of CCLE, regarded as a separate entity to SCLE. Twenty per cent of patients with SCLE also exhibit other types of cutaneous lupus erythematosus (CLE) lesions. No cases in the literature describe progression from SCLE to CCLE, or HLE, in particular. Hallmarks of HLE include verrucous lesions, chronicity and treatment resistance. Early recognition and follow-up are essential as malignancy may arise in long-standing lesions. Lung adenocarcinomas and hypertrophic lupus are both associated with p53 mutations and decreased granzyme B expression. Mutated p53 results in constitutive cell proliferation and DNA damage can accumulate in cells. Granzyme B is a protease in cytotoxic T cells involved in immune-mediated cell lysis. Both processes result in the potential to produce exaggerated hyperproliferative lesions, such as those observed in HLE and the lepidic adenocarcinomas. HLE is a rare clinical entity that can be easily misdiagnosed clinically and histopathologically. Increased awareness of this condition is essential to highlight the variety of clinical presentations of uncommon variants of CLE, to ensure they are considered while approaching clinical diagnosis.

Verrucous disseminated discoid lupus erythematosus with oral and palmoplantar lesions treated with hydroxychloroquine

Lupus erythematosus (LE) is an autoimmune inflammatory disease with a wide spectrum of cutaneous and systemic manifestations. A unifying concept of this disease is difficult to develop as clinical features of patients with LE show a great variation. Our objective was to present a case of verrucous disseminated discoid lupus erythematosus (DLE) with atypical morphology, extensive involvement, and having oral and palmoplantar lesions. For confirmation of diagnosis, histopathology was done, which showed characteristic findings of DLE. Combination therapy of hydroxychloroquine with oral and topical corticosteroids resulted in resolution of skin lesions. Sometimes, DLE lesions may pose a diagnostic and therapeutic challenge, especially in a case of unusual presentation, as in the given case of hypertrophic LE. These cases require adequate therapy with clinical and histopathological follow-up considering that squamous cell carcinoma may arise in long-standing lesions.

A case of refractory hypertrophic lupus erythematosus on the face whose irreversible skin fibrosis was treated by local full‐thickness skin graft under disease control with a combined use of topical and systemic immunosuppressants, and hydroxychloroquine

Hypertrophic lupus erythematosus (HLE) remains pose a treatment challenge, and often displays disfiguring scar with cosmetic and/or functional impairment. We report a male case of refractory hypertrophic lupus erythematosus on the face, whose disease activity was controlled with a combined use of topical and systemic immunosuppressants, and hydroxychloroquine. The persisted, irreversible fibrotic skin mass was finally treated with surgical approach using full-thickness skin graft, achieving a favorable cosmetic result.

Diagnostic Challenges of Hypertrophic Lupus Erythematosus

Hypertrophic lupus erythematosus (HLE) often occurs in the absence of systemic symptoms and can be misdiagnosed as squamous cell carcinoma (SCC) as both appear clinically as erythematous scaling plaques in sun exposed areas and histologically show significant epithelial hyperplasia. No single criterion confidently differentiates HLE from SCC histologically, postulating the need for CD123 immunostaining in evaluating locally recurring, supposed SCCs. We present a woman who presented for evaluation of multiple supposed left lower leg well-differentiated SCCs (wdSCC), that did not resolve following Mohs Micrographic Surgery and other techniques aimed at cancer removal. The lesions improved following intralesional Kenalog injection and showed positive CD123 immonostains, which both led to the diagnosis of HLE.

Eruptive Keratoacanthoma-Like Discoid Lupus Erythematosus

Hypertrophic discoid lupus erythematosus can resemble keratoacanthoma histomorphologically. We describe a unique case of eruptive keratoacanthomatous scaly papules in a 56-year-old woman with discoid lupus erythematosus. We review the literature and conclude by discussing important histologic features and useful immunostains to consider when attempting to discern the underlying identity and pathogenicity of such lesions.

Dermoscopy of hypertrophic lupus erythematosus and differentiation from squamous cell carcinoma

Fig 1. Hypertrophic lupus erythematosus. A, Clinical image of extensive verrucous plaques on the right postauricular area, pinna, and cheek. B, Discoid lesion on the left cheek, demonstrating a scaly peripheral edge and central whitish-pink atrophic area. C, Polarized contact dermoscopic view of the scaly anterior edge of the discoid plaque pictured in (B) showing numerous white-to-yellow hyperkeratotic follicles, confluent scaly areas, whitish perifollicular haloes, blurred reddish vascular areas (arrows), and linear vessels. D, Central atrophic area of the same lesion revealing homogeneous white-to-pinkish areas with dotted (circle), hairpin (rectangle), and linear vessels (arrow). The dermoscopic features depicted in (C) suggest active DLE, whereas the features in (D) are consistent with advanced DLE.

Case Report: A case of hypertrophic lupus erythematosus with negative CD123 staining and absence oftransepidermal elimination of elastin.

We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE). HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

Case Report: A case of hypertrophic lupus erythematosus with negative CD123 staining and absence of transepidermal elimination of elastin

We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE). HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin

We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE). HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

A case of hypertrophic lupus erythematosus with negative CD123 staining and transepidermal elimination of elastin

We report the case of a 49-year-old male with clinical and histological findings consistent with hypertrophic lupus erythematosus (HLE). HLE must be clinically and histologically differentiated from keratoacanthoma, hypertrophic lichen planus, squamous cell carcinoma and plaque type psoriasis. CD123 positivity and transepidermal elimination of elastin have recently been reported as tools to distinguish HLE. Interestingly, in this case, biopsies of two separate lesions failed to reveal these two features. The etiology of this discrepancy is unknown and further studies are needed to clarify the utility of CD123 positivity and transepidermal elimination of elastin in the diagnosis of hypertrophic lupus erythematosus.

Perforating elastic fibers (‘elastic fiber trapping’) in the differentiation of keratoacanthoma, conventional squamous cell carcinoma and pseudocarcinomatous epithelial hyperplasia

Keratoacanthoma (KA), an epithelial neoplasm occurring in sun-exposed skin of the elderly, is considered a well-differentiated form of conventional squamous cell carcinoma (SCC) that often follows a course of spontaneous regression. Distinguishing KA from conventional SCC or pseudocarcinomatous epithelial hyperplasia ensures proper diagnosis, treatment and management. For some time, perforating elastic fibers have been utilized in differentiating KA from SCC. This phenomenon may also occur in association with scars and hypertrophic lupus erythematosus (LE). To assess the diagnostic utility of perforating elastic fibers, we compared their incidence in KA, SCC, scars with overlying pseudocarcinomatous hyperplasia, hypertrophic LE, hypertrophic lichen planus (LP) and lichen simplex chronicus (LSC). A retrospective case search identified 359 lesions and the presence of perforating elastic fibers was evaluated using routinely stained sections. This phenomenon was documented in all studied groups except hypertrophic LP. The incidence was found to be 71% in KA, 37% in SCC, and was lowest in inflammatory conditions with associated pseudocarcinomatous hyperplasia (hypertrophic LP 0%, hypertrophic LE 5.9% and LSC 28.2%). The observed frequency in pseudocarcinomatous hyperplasia overlying scars (57.8%) vs. KA (71%) was not statistically different. Although elastic fiber trapping has potential value as a diagnostic criterion for KA, dermatopathologists should consider its limitations. Its diagnostic utility was greatest in distinguishing KA from hypertrophic LE and hypertrophic LP. Conversely, elastic trapping is not helpful differentiating pseudocarcinomatous hyperplasia from recurrent/persistent KA following surgery.

Bechet vs Behçet: The Name Is Not the Same

Recently, during a superb lecture on lupus erythematosus (LE), the followingphraseslipped in: “hypertrophicLEwasdescribedbyBehcet.”This is a commonmistakewith a simple explanation. Every dermatologist is proud to be able to spell Behcet, and when confronted with the name Bechetwiththemiddle2 letters transposed,one’s “intrinsicspell checker” converts Bechet to Behcet. When one searches for Bechet on www .jamadermatology.com, Behcet turns up quite often. The describer of hypertrophic LE was Paul Esnard Bechet (Be-shay) (1881-1962) (Figure,A), a dermatologist of French descent fromNewOrleans, Louisiana, who practiced in New York City after training at Tulane University. Bechet frequentlypresentedcasesat theNewYorkDermatological Society, and the proceedings of thesemeetingswere published in theArchivesofDermatologyandSyphilology. In 1940hereportedthecase ofa49-year-oldmanwithdestructivehyperkeratoticnodulesonhis lipthat histologicallywere interpretedasLE. Inhis lateryears,Bechetbecamefascinatedwith dermatological history, writing an array of articles, including oneson thedevelopmentofAmericandermatology,NewYorkdermatology, the Skin and Cancer Hospital, and the American Dermatological Association, aswell asmany biographical sketches and obituaries. Bechet’smost famousnamesake is the legendary jazzmusician Sidney Bechet (1897-1959), a Creole whowas also born in NewOrleans. A childprodigyandmulti-instrumentalist, Bechet’smusical giftswere first recognized at the age of 6 years. After playing clarinet for years with a variety of local and nationally touring bands, in 1919 Bechet traveled to London,England,wherehetookupthesopranosaxophone,which,along withhispopular compositions suchas “PetiteFleur,”would catapult him to life-long international fame. Our othermain protagonist, Hulusi Behcet (Beh-chet) (1889-1948) (Figure, B), was the first professor of dermatology in Turkey, being appointed in 1933. In 1937he reported the caseof apatientwith recurrent oral aphthous ulcers, genital ulcers, and eye lesions to the Istanbul Dermatological Society. He later published a description of this patient and several others. AfterWorldWar II, both GuidoMiescher in Zurich, Switzerland, and Helen Ollendorff Curth in New York proposed the name Behcet disease for this constellation ofmucosal and ocular findings, often associated with severe ischemic vasculitis. Adamantiades-Behcet disease isprobablyamoreappropriatename.BenediktosAdamantiades (1875-1962), aGreekophthalmologistpracticing in theancientByzantine townofPrussa(whichtoday isBursa,Turkey),describedayoungmanwith recurrent iritis,oralandgenitalulcers,andarthritis inAthens in1930.However, the rivalriesbetweenGreeceandTurkeyduring thisperiodprobably contributed to Adamantiades not getting proper recognition. We hope this note will clear the confusion surrounding the similar surnames of 2 of the 20th century’s great dermatologists so that each is recognized appropriately—Behcet for describing the mucocutaneous ocular syndrome and Bechet for identifying hypertrophic LE.

Cutaneous Hypertrophic Lupus Erythematosus: A Challenging Histopathologic Diagnosis in the Absence of Clinical Information

Hypertrophic lupus erythematosus (HLE) is rare variant of chronic cutaneous lupus characterized histologically by irregular epidermal hyperplasia associated with features of classic chronic cutaneous lupus, including interface changes. Lesions frequently demonstrate reactive squamous atypia of the basal layer and may show histopathologic overlap with other more common cutaneous atypical squamoproliferative lesions. Typical histologic features of cutaneous lupus, such as follicular plugging, angiocentric lymphocytic inflammation, and dermal mucin, are very helpful clues to the diagnosis of hypertrophic lupus erythematosus. Recently, immunohistochemistry for CD123 used to detect increased plasmacytoid dendrocytes in hypertrophic lupus erythematosus has proven to be diagnostically useful. A high index of suspicion for hypertrophic lupus erythematosus is essential to avoid overdiagnosis of squamous neoplasia, particularly in limited cutaneous biopsies in the absence of adequate clinical information.

Hypertrophic (Verrucous) Cutaneous Lupus Erythematosus of the Lip and Oral Cavity: A Series of 4 Cases

So-called “hypertrophic cutaneous lupus erythematosus (LE)” (also termed “verrucous cutaneous LE”) is a dis-tinct and rare clinical variant of chronic cutaneous LE that is characterized by intense tissue hyperplasia and hyperkeratosis. Hypertrophic LE causes marked disfigu-rement and is usually resistant to therapy (1, 2). Lesions occur more commonly on the face and upper limbs, with no specific mention in the literature of lesions occurring on the lips or oral mucosa. We describe here a series of 4 cases with a diagnosis of LE who presented with hypertrophic lesions on the skin and oral mucosa. Clinical and laboratory data are presented, together with detailed characterization of the mucosal involvement.CaSE REpoRtSClinical data for the 4 patients are shown in table SI (available from http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1433). all patients presented hypertrophic cutaneous LE of longstanding duration. only patient 1 had multiple active cutaneous LE lesions at the time of examination (Fig. 1A, B). Skin lesions in patients 2 and 4 were mainly residual, with intense scarring. The lesion in patent 3 was confined to the lip area (Fig. 1C–F). Histopathology of biopsied oral lesions revealed marked hyperkeratosis, presence of a granular layer, focal hydropic degeneration of the basal layer, necro-tic basal keratinocytes, and melanophages of variable intensity (Fig. 1G, H). There were no signs of kerati-nocytic atypia. Dermal components included dilated papillary vessels, mucin, and scarce inflammatory in-filtrate, except for case 2, in which inflammatory cells were abundant in a superficial and deep pattern. Direct immunofluorescence findings were non-diagnostic.In patients 1, 2 and 3, LE activity was more pronoun -ced on the tegument. In addition to skin lesions, patient 4 had a several year history of lupus arthritis.DISCuSSIonthere are few published case series of oral compromise in LE. of these, some uniformity can be observed in the clinical picture, despite the great variety of descriptive terms employed (“oral discoid lesion”, “chronic pla-que”, “lupus cheilitis”, “acute ulcer”, “erythematous ulcer”, “ulcerated plaque”, “pebbly red areas”, “ho-neycomb lesions”, “keratotic white lesion”, “purpuric lesion”, and “diffuse palatal petechial erythema”, among others); a clinical characterization based on established dermatological criteria would be useful. Our group has proposed a method that simply compares mucosal LE lesions with their cutaneous counterparts (acute, subacute and chronic mucosal LE in analogy to acute, subacute and chronic cutaneous LE) in order to better understand those lesions (3).We describe here 4 patients with what is known as hypertrophic/verrucous LE (a subtype of chronic cuta -

Solitary hypertrophic lupus erythematosus of the nose without other cutaneous or systemic involvement

Solitary hypertrophic lupus erythematosus of the nose without other cutaneous or systemic involvement

Hypertrophic and Ulcerated Discoid Lupus Erythematosus

Hypertrophic lupus is an uncommon clinical variant of chronic cutaneous lupus that remains a challenge to treat. A 45-year-old female day-care worker with long-standing lupus presented with hypertrophic cutaneous involvement on the dorsal hand, elbow, and toe of 6 years' duration. Treatments included monotherapy with either hydroxychloroquine or chloroquine and potent topical and intralesional steroid injection. Systemic chemotherapy with R-CHOP chemotherapy for stage IIA diffuse large B-cell lymphoma did not clear the skin findings. To review the clinical presentation and treatment of hypertrophic lupus. This report documents clinical improvement in refractory hypertrophic lupus with a regimen of acitretin and combination antimalarial therapy. The available published literature on the treatment of hypertrophic lupus was reviewed. There is limited published experience combining retinoids and antimalarials for the treatment of refractory lupus. Combination therapy with two antimalarials (ie, quinacrine with either hydroxychloroquine or chloroquine) provides therapeutic benefit for resistant cutaneous disease. In this case, triple therapy with two antimalarials and an oral retinoid achieved clinical clearance relatively rapidly, and this effect was maintained over a year. This article reports successful treatment of refractory hypertrophic discoid lupus with combination therapy that included chloroquine 250 mg/d, quinacrine 100 mg/d, and actitretin 25 mg/d. This report suggests that combination therapy using two antimalarials and an oral retinoid is a consideration for refractory hypertrophic lupus, but further study is warranted.

Chronic Cutaneous Graft-Versus-Host Disease Simulating Hypertrophic Lupus Erythematosus—A Case Report of a New Morphologic Variant of Graft-Versus-Host Disease

The spectrum of clinical and histopathologic features associated with chronic graft-versus-host disease (GVHD) is broad, with recognized variants simulating scleroderma, lichen sclerosus, eosinophilic fasciitis, and de novo diffuse melanoderma. We report a case of a patient with multiple myeloma who presented approximately 1 year after his allogeneic hematopoietic stem cell transplantation with lesions of chronic lichenoid GVHD that harbored features of hypertrophic lupus erythematosus (LE) and that was initially mistaken for a superficial well-differentiated squamous cell carcinoma (SCC). However, in 4 years of follow-up, the patient failed to develop any evidence of cutaneous or systemic LE, actinic damage, or SCC, and the lesions cleared with topical and systemic treatments appropriate for chronic GVHD. For proper interpretation of the histologic findings of GVHD, it is important for the dermatopathologist to be aware of unusual manifestations. Knowledge of the occurrence of hypertrophic LE and familiarity with its histologic features is also important to avoid an erroneous diagnosis of SCC in immunosuppressed patients.

Extensive hypertrophic lupus erythematosus: atypical presentation.

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Clinical features of patients with LE show a great variation, and for this reason it is difficult to develop a unifying concept of this disease. Our objective is to present a case of hypertrophic LE with atypical morphology and extensive involvement, who responded favorably to isotretinoin. Diagnosis of hypertrophic lupus erythematosus (HLE) was confirmed by characteristic histopathological findings. Combination therapy with isotretinoin and hydroxychloroquine resulted in flattening and repression of previously refractory skin lesions. Sometimes, HLE lesions may present a diagnostic and therapeutic dilemma. In long standing lesions, squamous cell carcinoma may arise. Therefore, HLE requires adequate therapy with clinical and histopathological follow up.

Hypertrophic lupus erythematosus: the diagnostic utility of CD123 staining

CD123-positive plasmacytoid dendrocytes are prominent in the infiltrate of discoid lupus erythematosus (LE). We hypothesized that these cells would also be present in hypertrophic LE and would aid in the histopathologic distinction from squamous cell carcinoma (SCC) and hypertrophic actinic keratosis (AK). Five cases of hypertrophic LE and 10 cases each of SCC and hypertrophic AK were stained with CD123. A heavy band of CD123-positive cells was present at the epidermal-dermal junction in all cases of hypertrophic LE, and only single or rare scattered clusters of CD123-positive cells were seen in SCC and actinic keratoses. The pattern of CD123 staining can be a useful feature to distinguish hypertrophic LE from SCC and hypertrophic AK.