Urethral keratosis caused by vitamin A deficiency: two case reports.

BACKGROUND: Dysuria in children can have various etiologies, including ureteric stones, phimosis, congenital obstructive posterior urethral membrane, and neurogenic bladders. However, there have been no reports of vitamin A deficiency (VAD) causing dysuria due to urethral keratosis. Here, we report two cases of urethral keratosis caused by a VAD. CASE PRESENTATION: An 8-year-old boy (Patient 1) and a 6-year-old boy (Patient 2) presented with multiple episodes of dysuria and epididymitis. Both patients had intellectual disabilities and autism, which prevented voiding cystourethrography. Therefore, the patient was admitted for cystoscopy under general anesthesia. Cystoscopy revealed urethral diastolic dysfunction and a large amount of desquamated epithelium obstructing the urethra in both patients, causing urinary obstruction and dysuria. Catheterization was repeated; however, the symptoms recurred after catheter removal. Although the cause of recurrent urinary obstruction could not be initially identified, an ophthalmologist found Bitot's spots and suggested the possibility of a VAD. Serum vitamin A levels were extremely low in both patients, leading to the diagnosis of urethral keratosis due to VAD. VAD can be attributed to an unbalanced diet resulting from intellectual disabilities or autism. Vitamin A replacement therapy improved both the urethral symptoms and cystoscopic findings. CONCLUSION: Dysuria due to VAD is extremely rare, and urethral keratosis as a cause of dysuria is likely the first report of its kind worldwide. VAD may develop due to an unbalanced diet in patients with intellectual disabilities or autism. Therefore, it is essential to consider VAD as a potential cause of dysuria in patients with intellectual disabilities and autism.

Smokeless tobacco keratosis.

Smokeless tobacco keratosis is a benign lesion characterized by the formation of white, gray, or pale macules or papules with wrinkling or rugae. It forms in the oral mucosa in response to the use of smokeless tobacco products. We present a 50-year-old man with an extensive history of smokeless tobacco use and development of the characteristic lesion. Shave biopsy showed typical changes of this benign condition and tobacco cessation was recommended.

Mechanic Hands/Hiker Feet in a Patient With Amyopathic Dermatomyositis and Interstitial Lung Disease.

ABSTRACT: A 30-year-old African American woman with a history of interstitial lung disease presented with bilaterally symmetrical, nonpruritic, scaling and fissuring, hyperpigmented, lichenified plaques on her hands and feet. She reported occasional erythema of her face, intermittent erythema, and irritation of her eyes but denied any muscle weakness. A biopsy of the plantar first toe showed hyperkeratosis, striking alternating ortho- and parakeratosis with underlying apoptotic bodies. There was psoriasiform acanthosis without suprapapillary thinning, numerous apoptotic keratinocytes in all layers of the epidermis extending into the corneum that were out of proportion with the minimal interface inflammation. Colloidal iron and Alcian blue stains showed increased dermal mucin deposition. Given the clinical, histopathological, and supportive serological findings (positive anti-KU and anti-SSA), a diagnosis of clinically amyopathic dermatomyositis with mechanic hand/hiker feet (MH/HF) was rendered. The pseudocheckerboard pattern of MH/HF has been previously reported in only 4 patients. The most frequent associations with MH/HF are dermatomyositis and antisynthetase syndrome; however, our patient was negative for antiaminoacyl transfer RNA synthetase antibodies, a required criterion to diagnose antisynthetase syndrome. It is imperative to recognize MH/HF clinically and histopathologically because it may be an early indication of developing dermatomyositis or other connective tissue diseases, which would guide further workup and screening for systemic involvement of the disease, including interstitial lung disease.

Acrokeratosis of Bazex as a sign of thyroid cancer: first description and review of thyroid-associated paraneoplastic dermatoses.

OBJECTIVE: Bazex syndrome is a rare paraneoplastic skin disorder of unknown pathogenesis. Cutaneous findings are usually noticed before the diagnosis of the underlying malignancy, more frequently squamous cell carcinomas of the upper aerodigestive tract or metastasis to cervical lymph nodes. Association with other malignancies has been reported. CASE REPORT: Herein, we describe a case in course of metastatic papillary thyroid carcinoma and review the relevant literature. RESULTS: A bibliographic search was conducted and a total of 8 studies concerning the association were reviewed. CONCLUSIONS: Physicians be aware of unexpected cutaneous conditions as a possible sign of underlying tumors.

Erythema ab igne with features resembling keratosis lichenoides chronica.

Erythema ab igne (EAI) is an asymptomatic dermatosis that develops in response to chronic exposure to low-grade heat. Characteristic findings on histopathology include epidermal atrophy, dermal elastosis, atypical histiocytes, and melanin and hemosiderin deposition. Reactive endothelial changes and prominent vascular proliferation are variable. Keratosis lichenoides chronica (KLC) is a rare lichenoid hyperkeratotic dermatosis. Acanthosis with parakeratosis and a lichenoid interface dermatitis with lymphocytes, histiocytes, and plasma cells are characteristic findings of KLC. Although its etiology remains unclear, KLC has been reported to occur in response to heat. Herein, we report a case of EAI with features resembling KLC.

Treatment of toes as an integrated part of infection control for advanced lower limb lymphedema.

The aim of this study was to compare the incidence of infection and verrucous hyperkeratosis in patients who underwent surgery for advanced lymphedema according to the algorithm designed by the senior author, and were treated concurrently with/without toe treatment. A case series (Between 2004-2015) of 46 patients with unilateral advanced lower limb lymphedema was reviewed. Lymphoscintigraphy was used for evaluation of lymphedema severity. The ICG lymphography was used for staging. Fibrosis and skin induration were reflected by the tonicity. They were divided into two groups: (1) patients who underwent further treatment of toes according to the algorithm, and (2) patients who did not have toe-related treatment. Infection episodes and verrucous hyperkeratosis were recorded. There were 21 and 25 patients in Groups 1 and 2, respectively. All lymphoscintigrams showed severe dermal backflow with severe stagnation by 2.5 h after injection of Tc-99 colloid. All patients were stage IV or V. Tonicity values of skin were <60. Group 1 was reduced to an average of 0.6 episodes per year in the past year of follow-up, and Group 2 was reduced to an average of 1.5 episodes per year in the past year of follow-up (p <0.001). The average frequency of preoperative cellulitis was 3.6 episodes per year. The occurrence of verrucous hyperkeratosis was observed in 14.3% and 32% of Groups 1 and 2, respectively. The incidences of cellulitis and verrucous hyperkeratosis were significantly lower in Group 1 than in Group 2 (p <0.001). To achieve successful control of infection, they should be treated carefully according to the strategy described above.

Chronic Oral Lesions.

Chronic oral mucosal lesions can be associated with several mucocutaneous diseases. This article reviews the autoimmune and immune-mediated, reactive, genetic, and infectious diseases that may present with chronic oral and/or cutaneous manifestations and provides a rational approach to diagnosis and management.

Huriez syndrome associated with basal cell carcinoma. A case report.

Huriez syndrome is a rare cancer-prone genodermatosis confined to the hands and feet connects with an increase in squamous cell carcinoma on affected skin. Its diagnosis is complex due to not well defined symptoms and since only few cases are described in literature. The differential diagnoses are many and the treatment is focused only on symptoms control and tumours eradication. Our case report is highly interesting because add new knowledge about this disease describing a new important feature of the syndrome. For the first time in literature we describe the arising of basal cell carcinoma from affected skin. KEY WORDS: H, Basal cell carcinoma, Huriez syndrome, Palmoplantar keratoderma.

Forming diagnostic criteria for vulvar lichen planus.

BACKGROUND/OBJECTIVES: Vulvar lichen planus is a debilitating skin condition usually complicated by delayed diagnosis due to its highly variable clinical appearance and inconsistent histopathological characteristics. This study aims to devise a clinical diagnostic tool for the disease and to correlate this with histopathology findings. METHODS: The retrospective single-centre chart review was conducted for patients presenting between January 2010 and December 2019. Clinical features were compared between 243 women with clinically suspected vulvar lichen planus with available histopathology, 50 patients with biopsy-proven vulvar lichen sclerosus and 50 patients with culture-proven chronic vulvovaginal candidiasis. Features which significantly differentiated between conditions were further studied using multivariate nonlinear regression analyses to formulate a score-based diagnostic criteria. Criteria was then applied to the remaining patients with inconclusive biopsies (classified as 'normal', 'non-specific' or 'suggestive or lichenoid') to determine sensitivity and specificity. RESULTS: The clinical features that significantly differentiated the conditions were the presence of erosions (P < 0.001), glazed erythema (P < 0.001), oral involvement (P < 0.001), pain/burning sensation (P < 0.001) and hyperkeratotic border (P < 0.001). A score ≥2 correlated with a histopathological diagnosis of vulvar lichen planus with a sensitivity of 100%. The specificity was 92% and 88% when compared against vulvar lichen sclerosus and chronic vulvovaginal candidiasis, respectively. Sensitivity was 97%, 97% and 93% in suggestive, nonspecific and normal histopathological subgroups, respectively. CONCLUSIONS AND RELEVANCE: The proposed criteria may aid clinicians in diagnosing patients if histopathology is inconclusive. Nonspecific and suggestive findings on biopsy for patients with ≥2 features on diagnostic criteria are comparable to a conclusive biopsy.

Creation and Validation of Classification Criteria for Discoid Lupus Erythematosus.

Importance: Classification criteria are the standardized definitions that are used to enroll uniform cohorts for research studies. They emphasize high specificity and are distinct from diagnostic criteria. No universally recognized classification criteria currently exist for discoid lupus erythematosus (DLE), which has led to problematic heterogeneity in observational and interventional clinical studies across the field. Objective: To create and validate classification criteria for DLE using 12 previously defined candidate criteria items. Design, Setting, and Participants: For this diagnostic study, candidate criteria items were prospectively applied by dermatologists and dermatopathologists at clinical visits of patients with DLE or a condition that could be confused for DLE, termed a DLE mimicker, at academic dermatology practices across the United States, Poland, Japan, and South Korea. Data were collected from December 1, 2017, to February 1, 2019, and analyzed from March 1 to September 19, 2019. Main Outcomes and Measures: Clinical features among these 2 groups were calculated and compared with χ2 or Fisher exact tests. Candidate models were identified using best subsets logistic regression analysis. Improvement tests, fit statistics, and discrimination were considered to choose a final model. Results: Nine sites contributed 215 patients, 15 of whom had missing or incomplete data. The final model for DLE classification criteria includes only clinical variables: atrophic scarring (3 points), location in the conchal bowl (2 points), preference for the head and neck (2 points), dyspigmentation (1 point), follicular hyperkeratosis and/or plugging (1 point), and erythematous to violaceous in color (1 point), with an area under the receiving operating characteristic curve of 0.91 (95% CI, 0.87-0.95). A score of at least 5 points yields a sensitivity of 84.1% and a specificity of 75.9% in the classification of DLE, with increasing scores yielding higher specificity. Conclusions and Relevance: These findings provide the initial validation of classification criteria for DLE for use in observational and clinical trials.

Follicular spicules of multiple myeloma.

Follicular spicules are a very rare but highly characteristic cutaneous manifestation of multiple myeloma. The spicules typically appear as hyperkeratotic horns in the follicular openings of the face, most commonly on or around the nose and forehead. The pathophysiology of this condition has not been fully elucidated and remains an active area of research and debate. Herein we describe a patient who presented with follicular spicules in the context of unintentional weight loss, anemia, and elevated inflammatory markers. We discuss the diagnostic work-up for such a presentation, review the classification of follicular spicules of multiple myeloma, and describe approaches to manage this uncommon skin condition.

Eruptive milia and acneiform hyperkeratosis with comedones (pseudo-epidermal cysts) within tattoos.

BACKGROUND: Milia are small, hard, white superficial epidermal cysts measuring a few millimetres that can occur during skin healing due to occlusion of pilosebaceous units. Milia rarely occur on tattoos. However, cases of allergic reactions with hyperkeratosis and open comedones have been described in the literature, sometimes under the term "epidermal cysts". PATIENTS AND METHODS: We saw three patients who developed milia, including a 32-year-old man with eruptive milia 10 weeks after getting a black, red and green tattoo on his upper arm. Topical tretinoin was applied. We encountered two further cases of eruptive milia on black/grey tattoos. A fourth patient presented a massive hyperkeratotic reaction with retention comedones on the red/pink area of a tattoo. DISCUSSION: The occurrence of milia and acneiform allergic reactions after tattooing is rare. We collated a total of 13 cases from the literature, of which 8 involved milia. This condition occurred within 3 months following tattooing, with no particular correlation with any given colour, and generally without any allergic reaction (except in one case). Reactions comprising excessive acneiform hyperkeratosis and open comedones were noted with pink and red inks and were a complication in a setting of allergic inflammatory reaction. However, the histopathology of these reactions is poorly described in the literature. It seems inappropriate to diagnose the condition as "epidermal cysts" since the lesions are not in fact simple cysts but rather retention lesions occurring during an inflammatory reaction and are thus different from post-traumatic milia.