Histopathologic findings in ointment pseudo-cheilitis: An alert to dermatopathologists.

Ointment pseudo-cheilitis is a recently recognized distinctive type of self-induced cheilitis. Lesions consist of a variable amount of crusts adhered to the vermilion. These crusts consist of dried saliva and dead cells mixed with applied medications attached to the lip surface. Patients are typically severely anxious or depressed; the condition impacts quality of life. Ointment pseudo-cheilitis is frequently misdiagnosed as exfoliative cheilitis or cheilitis glandularis. Biopsy reports are often non-revealing because there are no established histopathological criteria for this disease, and clinicians usually do not formulate the correct diagnostic hypothesis. Here, we present the histopathological findings of four cases of ointment pseudo-cheilitis. The most consistent finding was the presence of laminated parakeratotic material detached from the epithelium in biopsies that are devoid of other significant diagnostic changes. This material at the lip surface possibly represents physiologic labial desquamation mixed with dried saliva and applied medication. With this report, we intend to alert dermatopathologists to the diagnosis of ointment pseudo-cheilitis if they receive biopsies from patients who present clinically exuberant labial lesions that show only minimal histopathological changes.

Oral lichen planus: case series and experience in a tertiary dermatology service in Brazil

Abstract Background: Lichen planus is an inflammatory disease that can affect both the skin and mucous membranes, including the oral mucosa. There is very little original Brazilian dermatology literature about oral lichen planus. Objective: To describe the clinical, pathological, and treatment data of 201 patients diagnosed with oral lichen planus followed at the Stomatology Outpatient Clinic of Hospital das Clínicas, Universidade de São Paulo, from 2003 to 2021. Method: The patients demographic profile, the morpho-topographic features of the lesions, the treatment employed, and the possible presence of squamous cell carcinoma were analyzed. Results: The disease was more common in women over 50 years of age, tending to be chronic, with a large number of cases showing cicatricial sequelae in the mucosa. Topical treatment with potent corticosteroids was shown to be effective in the vast majority of cases. Squamous cell carcinoma in oral lichen planus cicatricial sequelae was observed in eight cases. Study limitations: Retrospective study of medical records, with gaps regarding the filling out of data; unequal observation time among the studied cases. Conclusions: This is the largest Brazilian dermatology series on oral lichen planus. The response to topical corticoid therapy was excellent in the vast majority of cases. The high prevalence of atrophic lesions, demonstrating the chronicity and tissue destruction potential of this disease, may explain the large number of cases of squamous cell carcinoma.

Squamous Cell Carcinoma Arising in Discoid Lupus Erythematosus of the Nail Unit.

Introduction: Discoid lupus erythematosus can affect periungual tissues leading to onychodystrophy. Squamous cell carcinoma can occur in persistent scars of discoid lupus; this rare occurrence has not yet been reported on the nail unit. Case presentation: we report a case of squamous cell carcinoma occurring on the distal phalanx of the thumb in a patient with longstanding periungual discoid lupus on several fingernails. Discussion: Periungual discoid lupus erythematosus is rare. The scars caused by this disease can very rarely develop into squamous cell carcinoma. This is the first report of this occurrence at the periungual tissues.

Oral lichen planus: case series and experience in a tertiary dermatology service in Brazil.

BACKGROUND: Lichen planus is an inflammatory disease that can affect both the skin and mucous membranes, including the oral mucosa. There is very little original Brazilian dermatology literature about oral lichen planus. OBJECTIVE: To describe the clinical, pathological, and treatment data of 201 patients diagnosed with oral lichen planus followed at the Stomatology Outpatient Clinic of Hospital das Clínicas, Universidade de São Paulo, from 2003 to 2021. METHOD: The patients demographic profile, the morpho-topographic features of the lesions, the treatment employed, and the possible presence of squamous cell carcinoma were analyzed. RESULTS: The disease was more common in women over 50 years of age, tending to be chronic, with a large number of cases showing cicatricial sequelae in the mucosa. Topical treatment with potent corticosteroids was shown to be effective in the vast majority of cases. Squamous cell carcinoma in oral lichen planus cicatricial sequelae was observed in eight cases. STUDY LIMITATIONS: Retrospective study of medical records, with gaps regarding the filling out of data; unequal observation time among the studied cases. CONCLUSIONS: This is the largest Brazilian dermatology series on oral lichen planus. The response to topical corticoid therapy was excellent in the vast majority of cases. The high prevalence of atrophic lesions, demonstrating the chronicity and tissue destruction potential of this disease, may explain the large number of cases of squamous cell carcinoma.

Three cases of oral mucosal tuberculosis in patients on tumour-necrosis-factor-α blockers.

We present three cases of oral mucosal lesions caused by Mycobacterium tuberculosis in patients treated with anti-tumour necrosis factor-α for psoriasis or rheumatoid arthritis. Diagnosis of oral mucosal tuberculosis was not easily established in any of the cases. A comparison between these cases and other previously described forms of oral mucosal tuberculosis is presented.

Bullous lesions following phototherapy in a newborn

ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood's lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.

The Role of Reflectance Confocal Microscopy in the Evaluation of Pigmented Oral Lesions and Their Relationship With Histopathological Aspects.

ABSTRACT: Oral pigmentations are a heterogeneous group and can be the result of physiological activity of oral mucosal melanocytes, secondary to exogenous causes, associated with systemic or local diseases, or due to proliferative activity of melanocytes. Their diagnosis is critical because these lesions can be markers of internal diseases or, in the case of melanocytic proliferative processes, they may represent a malignant neoplasm. In the past decade, the use of reflectance confocal microscopy, a noninvasive imaging tool, has aided the analysis of such lesions, but the establishment of firm criteria in their evaluation is still lacking. This study evaluated a series of 19 cases of pigmented oral lesions and correlated the reflectance confocal microscopy findings with histopathological classical criteria. We found 13 cases of melanotic macule, 1 of them associated with Peutz-Jeghers syndrome and 2 with Laugier-Hunzinker syndrome; 1 melanocytic nevus; 2 lentigo maligna; 2 pigmented actinic cheilitis; and 1 case of postinflammatory pigmentation secondary to a lupus erythematosus oral discoid lesion. The main difference between benign and malignant lesions was the presence of atypical proliferation in lentigo maligna. Langerhans cells with thick dendritic processes, which may be present in other benign and inflammatory pigmentations is one of the main reasons for diagnostic pitfalls.

Reflectance confocal microscopy (RCM)-based criteria for progression of lower-lip squamous cell carcinoma: A prospective study.

BACKGROUND: Actinic cheilitis (AC) presents as a diffuse clinical-histopathological alteration throughout the lower lip. OBJECTIVES: To analyze AC lesions using reflectance confocal microscopy (RCM) and evaluate criteria for the early diagnosis of incipient squamous cell carcinoma (SCC). METHODS: This prospective study comprised cases of clinically diagnosed AC. RCM was performed over the entire extension of the lower lip. The sites that showed the highest degree of morphological alteration by RCM, according to established criteria for AC and SCC, were biopsied. The RCM findings and histopathology were correlated to establish the precise and early diagnosis of SCC. RESULTS: A total of 61 cases that had been clinically diagnosed with AC were included. The RCM findings that correlated independently with SCC were: nonedged papillae (sensitivity 84% and specificity 88%, p < 0.0001, and OR 42), cell-filled papillae (sensitivity 82% and specificity 93%, p < 0.0001, and OR 71.3), inflammation (sensitivity 68% and specificity 68%, p = 0.0163, and OR 4.8), large and roundish cells in the lamina propria (sensitivity 65% and specificity 100%, p < 0.0001, and OR infinity), and nests in the lamina propria (sensitivity 54% and specificity 100%, p < 0.0001, and OR infinity). An independent histopathological analysis classified the cases as partial epithelial dysplasia (25 cases, 41%) and SCC (36, 59%), and in 57 cases (94%), the results agreed with the categorization that was rendered by the RCM exam. CONCLUSIONS: RCM can be used to monitor AC cases, guide the biopsy site, and identify the early progression of AC to SCC with good sensitivity and specificity.

Squamous Cell Carcinoma Arising in Ungual Lichen Planus: Report and Review.

BACKGROUND: Lichen planus (LP) can lead to severe scarring of the nail unit leading to anonychia. There are very few reports of squamous cell carcinoma (SCC) occurring in the lesions of ungual LP. OBJECTIVE: The aim of this study was to revise the previously reported cases of SCC appearing in ungual LP and to present a new case. METHODS: A PubMed search was performed with the terms "nail lichen planus" and "squamous cell carcinoma." Reported cases as well as a new case were depicted in a table. RESULTS: Only 2 indexed articles reporting 3 cases were found. All patients suffered of long-lasting scarring ungual LP. CONCLUSIONS: The occurrence of SCC in nail LP is rare. LP is not "premalignant" per se, but SCC might rarely arise in LP scars.

Orofacial Granulomatosis and Crohn Disease: Coincidence or Pattern? A Systematic Review.

BACKGROUND: To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD). SUMMARY: This review was conducted according to PRISMA guidelines and a search of the PubMed, MEDLINE, and Embase databases, and the Cochrane Library in March 2020, using keywords and MeSH terms associated with "orofacial granulomatosis," "Crohn disease," and their variants, with no language restrictions and across all age groups. All relevant articles were accessed in full text. Single case reports and articles on sarcoidosis, allergy, ulcerative colitis, and infectious diseases were excluded from the analysis. RESULTS: We retrieved 507 reports on OFG. The mean age at onset was 23.3 years (range 2-89 years). A total of 240 (47.3%) females and 267 (52.6%) males were included. CD was present in 93 children aged <16 years (68.3%) and in 43 adults (31.9%). In most cases, the OFG appeared before the CD. The most common clinical manifestations were intraoral mucosa abnormalities (n = 251; 49.5%), lower-lip swelling (n = 249; 49.1%), upper-lip swelling (n = 227; 44.7%), and gingivae (n = 193; 38.7%). Patients with concurrent CD were more likely to experience involvement of the buccal sulcus. Key Messages: OFG presents primarily as a solo entity. The OFG that was associated with CD was present in 93 children aged under 16 years (68.3%) and in 43 adults (31.9%). Childhood onset of OFG carries with it a higher risk of developing CD.

The histopathological spectrum of Melkersson-Rosenthal syndrome: Analysis of 47 cases.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of recurrent orofacial edema, relapsing facial paralysis and plicated tongue. Histopathological features of MRS have not been extensively analyzed. METHODS: This study investigated the histopathological aspects of oral lesions from 47 MRS patients. RESULTS: The most common biopsy site was the upper lip, followed by the lower lip, gingiva and palate. The most important findings were ill-defined and well-formed granulomas. Lymphoplasmacytic inflammatory infiltrate was seen in early and late stages of MRS. Edema, fibrosis, vasodilatation and congestion were the most common finding in the lamina propria. Gingival and palate exams also demonstrated granulomatous infiltrates. Regarding the evolution time of the disease, we demonstrated that, in initial phases, there is a lymphoplasmacytic inflammatory infiltrates, followed by a granulomatous infiltrate and, subsequently, fibrosis. CONCLUSION: Histopathological examination of oral lesions is helpful for the diagnosis of MRS; the absence of granulomatous inflammation does not exclude the diagnosis of syndrome. Clinical and histopathological analysis of the rare gingival and palate lesions is important, since all histopathological findings of the disease were detected in these sites.

From strawberry gingivitis to palatal perforation: The clinicopathological spectrum of oral mucosal lesions in granulomatosis with polyangiitis.

BACKGROUND: Reports of oral manifestations of granulomatosis with polyangiitis (GPA) usually refer to single-case reports; "strawberry gingivitis" has been increasingly reported. OBJECTIVE: To study the clinicopathological findings of four patients in which the diagnosis of GPA was suspected from the observation of their oral lesions and compare these to existent data. METHODS: Retrospective study of a case series. RESULTS: One patient presented typical "strawberry gingivitis" with localized disease and negative ANCA results. Two patients presented rapidly growing oral ulcers associated with systemic compromise and high ANCA levels. One patient presented with a chronic granulomatous lesion that leaded to palatal perforation. CONCLUSION: Oral manifestations of GPA may vary from rapidly evolving lesions in acutely ill patients to chronic and locally destructive lesions in slowing developing disease. These differences are also evident in the histopathological findings.

Cutaneous sarcoidosis: clinico-epidemiological profile of 72 patients at a tertiary hospital in São Paulo, Brazil.

BACKGROUND: Sarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking. OBJECTIVES: To describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018. METHODS: Clinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. RESULTS: Cutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient. LIMITATIONS: Insufficient data in medical records. CONCLUSIONS: This series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.

CHILD syndrome: successful treatment of skin lesions with topical lovastatin and cholesterol lotion.

CHILD syndrome (Congenital Hemidysplasia, Ichthyosiform erythroderma, Limb Defects) is a rare X-linked dominant disease. The authors report a 2-month-old patient presenting with typical features of CHILD syndrome that was treated with a topical solution containing cholesterol and lovastatin, with complete clearance of her CHILD nevus. The changes in skin lipid metabolism that explain the CHILD ichthyosiform nevus and their correction through topical application of cholesterol and lovastatin are discussed.

CHILD syndrome: successful treatment of skin lesions with topical lovastatin and cholesterol lotion

Abstract: CHILD syndrome (Congenital Hemidysplasia, Ichthyosiform erythroderma, Limb Defects) is a rare X-linked dominant disease. The authors report a 2-month-old patient presenting with typical features of CHILD syndrome that was treated with a topical solution containing cholesterol and lovastatin, with complete clearance of her CHILD nevus. The changes in skin lipid metabolism that explain the CHILD ichthyosiform nevus and their correction through topical application of cholesterol and lovastatin are discussed.

Ointment Pseudo-Cheilitis: A Disease Distinct From Factitial Cheilitis. A Series of 13 Patients From São Paulo, Brazil.

BACKGROUND: The terms exfoliative cheilitis, factitial cheilitis, and morsicatio labiorum are used to describe self-inflicted lesions of the lip. OBJECTIVE: Here we report and analyze clinical, pathological, and therapeutic data on 13 patients with a form of factitial cheilitis that we believe should be considered a separate entity. RESULTS: Eight patients were male and 5 patients were female. All patients reported pain and presented with crusts consisting of dried saliva and topical medications adherent to the surface of the lips. These patients expressed great concern with their condition, and reported several previous, ineffective treatments. Simple reassurance only was not effective; saline compresses alleviated symptoms for a few patients. Most patients were lost to follow-up. Biopsies were not performed on all patients. CONCLUSION: This particular type of cheilitis artefacta has been previously described, but some features described herein are new and allow a specific approach: patient's behavior, the "protrusion sign," and possible improvement with antidepressants. For these cases, we propose the term ointment pseudo-cheilitis because it comprises the nature of the attached material and the lack of true inflammation. Further psychiatric characterization is a logical next step in further characterizing this difficult-to-treat condition.