Background Livedoid vasculopathy, characterised by painful ulcers and atrophie blanche, significantly affects the quality of life of patients. Data pertaining to the efficacy of various available treatment options for this condition is limited, especially in India. Aim The study aimed to evaluate the treatment outcomes, clinicodemographic features, and associated laboratory abnormalities in patients with livedoid vasculopathy at a tertiary care centre. Methods This retrospective observational study analysed the case records and clinical photos of all clinically and histologically proven cases of livedoid vasculopathy who received antithrombotic treatment and were followed up for a minimum of 6 months. The primary endpoint was the proportion of patients who achieved a pain visual analogue (VAS) score of zero and complete healing of livedoid vasculopathy ulcers at 3 months. The secondary endpoint was the proportion of patients who achieved a pain VAS score of zero and complete healing of livedoid vasculopathy ulcers at 6 months. Side-effects, improvements in the dermatological life quality index (DLQI), clinicodemographic features, and associated laboratory abnormalities were also analysed. Results Of the 26 patients who satisfied the inclusion and exclusion criteria, 20 were males. At 3 months, 65.3% (17) of patients achieved the primary endpoint. Of these, 11 had received rivaroxaban (10mg) once daily and six had received aspirin (150mg) once daily. At 6 months, 96.1% (25) of patients had complete ulcer healing and achieved a VAS pain score of 0. Of these, 11 patients had received rivaroxaban, eight had received a combination of rivaroxaban and aspirin, and six had received aspirin. The improvements in VAS and DLQI at 3 months and 6 months were significant. None of the patients had any adverse effects from the therapy. Limitations The small sample size and its retrospective nature were limitations of the study. Conclusion Monotherapy with rivaroxaban or aspirin can effectively heal the ulcers, successfully achieve pain control, and improve the quality of life in livedoid vasculopathy. However, a fraction of patients may need a combination of the two to achieve these therapeutic goals. Both monotherapy and combination therapy are safe and not associated with significant side effects.

Abstract Dermatitis artefacta (DA) is a self-inflicted psychocutaneous condition with varying morphologies. Herein we discuss four cases of DA with bullous lesions (Table). The first patient had right-hand dominance with involvement of a covered area, and spontaneous healing of bullous lesions, which led to suspicion of DA. The second patient was left-hand dominant with almost complete involvement of the right upper limb, with extensive scarring but minimal concern. She had visited multiple doctors and was a divorcee in her second marriage, after which the disease had started. The single most important clinical pointer towards her disease was presence of linear hyperpigmented macules at most proximal area of the arm. The third patient was an adolescent boy accompanied by his father; his mother had died 5 years earlier, after which these episodes had started. Older photographs showed uniform disc-shaped bullous lesions suggestive of burns. The fourth patient was a student preparing for a competitive exam. Some linear imprints suggestive of fingers were noticed over the left arm, which gave a suspicion of self-infliction. DA can be associated with conditions such as underlying depression, anxiety and/or borderline personality disorder. In patients with bullous lesions, it is important to rule out immunobullous disorders.TableSummary of clinical detailsCase1234Age (years), sex25, female30, female13, male21, femaleSiteLeft breastRight upper limbGeneralizedBilateral upper limbs and trunkPresentationPainful recurrent bullous lesionsFlaccid bullae with background of oedema and scarringMultiple round atrophic and/or dyspigmented scarsBright erythema with vesicles and bullaeClinical diagnosisLocalized BPLocalized BPDermatitis artefactaSevere irritant contact dermatitisHistopathologySubepidermal cleftNonspecificNot doneNot doneDirect immunofluorescenceNegativeNegativeNot doneNot doneFinal diagnosisBorderline personality disorderMajor depressive disorderImpulse control disorderAcute stressManagementOcclusive bandagingOcclusive bandagingCounsellingObservationBP, bullous pemphigoid.

A 42-year-old female teacher presented to the dermatology outpatient department with diffuse skin thickening and breathlessness. She had multiple asymptomatic elevated lesions all over her body for the last 5 years. She complained of reduced mouth opening and thinning of fingers. She had received various medications previously, including topical and oral corticosteroids and homeopathic medication, without significant improvement. Cutaneous examination showed generalised symmetric skin-coloured to waxy papules of 2–5 mm in size distributed over the face, neck, trunk and limbs. The papules were present over indurated skin and were linearly located on the face, neck and extremities (Figure 1a,b). The baseline investigations, such as the hemogram, biochemistry profile, viral serology and serum lipid profile, were within normal limits. Investigations revealed features of monoclonal gammopathy of undetermined significance: Serum protein electrophoresis showed monoclonal hypergammaglobulinemia and an M spike in the gamma globulin region, and the kappa:lambda ratio was normal. A cardiology consultation revealed moderate tricuspid regurgitation (TR) and pulmonary arterial hypertension. Pulmonary investigations such as chest X-ray, high-resolution tomography, and pulmonary function tests were within normal limits. A skin biopsy showed dermal mucin deposition, increased collagen and fibroblast proliferation, confirming the diagnosis of scleromyxedema (Figure S1). Treatment was initially started with 2 g/kg of intravenous immunoglobulin (IVIG) (total 100 g) monthly for 6 months, along with thalidomide 100 mg capsules twice daily. For the TR and pulmonary arterial hypertension, she was started on sildenafil 20 mg tablets twice daily along with eplerenone. The patient felt symptomatically better with improved breathlessness, and the skin papules started to regress. Following this improvement, the frequency of IVIG was decreased to once every 2 months for 6 months, then once every 3 months for 6 months and then once every 6 months for 1 year. After 2.5 years, the skin was almost completely normal (Figure 2a,b), with a significant reduction in the induration and resolution of the papules. Her mouth opening had significantly improved, and the Dermatology Life Quality Index reduced from 20 to 6. During the treatment, no significant side effects were noted. Currently, she is receiving IVIG every 6 months and is taking thalidomide 100 mg once daily. Scleromyxedema, a mucin deposition disorder, is characterised by a generalised papular eruption on a sclerodermoid background because of increased fibroblast proliferation, fibrosis and mucin deposition. It is commonly associated with monoclonal gammopathy [1-3]. Various treatments for scleromyxedema have been reported, such as corticosteroids, melphalan, thalidomide, IVIG, retinoids, extracorporeal photophoresis and autologous stem cell transplantation. There are no definitive guidelines on the best approach to treatment [2]. A recent systemic review reported IVIG to be the most commonly used treatment for scleromyxedema. The improvement rates for the skin, systemic involvement and associated paraproteinemia were found to be 69%, 63% and 75%, respectively. In addition, it demonstrated a very good safety profile of IVIG [4]. An open-label study demonstrated response in all patients following IVIG therapy, with the largest improvement observed in the treatment-naïve group [5]. The exact mechanism of action of IVIG in scleromyxedema is unknown; the plausible hypotheses for its effectiveness include IVIG's ability to block Fcγ receptor–mediated phagocytosis, inactivation of antibodies by anti-idiotype antibodies, elimination of circulating immune complexes and regulatory effect on cellular immune responses [4]. Thalidomide is used as an adjunct to IVIG in cases of partial response or for maintenance therapy to decrease the frequency of IVIG treatments. The mechanism of action of thalidomide in the treatment of scleromyxedema is due to its antiangiogenic, anti-inflammatory and immunomodulatory properties—the same rationale for its use in refractory multiple myeloma [4]. In our case, adding thalidomide helped decrease the frequency of IVIG administration without affecting disease remission. In addition, the patient experienced no untoward side effects as a result of the combination therapy. In conclusion, we report a rare case of scleromyxedema successfully treated with IVIG and thalidomide and provide two-and-a-half-year follow-up data. This report highlights the role of thalidomide as a maintenance therapy after the initial control of the disease by IVIG. Informed written consent was obtained from the patient to publish her photographs. The authors declare no conflicts of interest. The data that support the findings of this study are available from the corresponding author upon reasonable request. Figure S1. Histopathology shows dermal mucin deposition, increased collagen, and fibroblast proliferation (H&E, ×100). Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

In this manuscript we have shown the clincal images of a patient with papillon lefevre syndrome confirmed with genetic analysis. Knowledge of such syndromes is important as they can be clinically suspected in backgrund of other findings like periodontitis.

Pediatric eczema, which is mainly constituted by atopic dermatitis, is a prevalent chronic inflammatory skin condition in children characterized by dry, itchy, and inflamed skin. It significantly impacts quality of life and poses a therapeutic challenge due to its relapsing nature and varying severity. Nonpharmacologic treatments, especially moisturizers and cleansers, are essential components in managing pediatric eczema. Two key skincare categories with significant dermatologic relevance are cleansers and moisturizers, including their role in pediatric eczema. Moisturizers are a vital component of a dermatologist’s armamentarium, yet surprisingly little is written about them, and even less is truly understood. The market is flooded with a wide array of skincare products, many of which lack solid scientific validation. Although often dismissed as mere cosmetics, these products play a recognized role in managing various skin conditions. In today’s dermatological practice, it is essential for professionals to have a thorough understanding of the mechanisms of action, appropriate usage, dosing, and potential side effects. Cleansers may consist of alkaline soaps or milder synthetic detergents, commonly referred to as syndets, which are less damaging to the skin barrier. Syndets tend to cause less skin irritation and dryness, likely due to their lower propensity to denature proteins. This effect is attributed to the charge density of surfactant aggregates that resemble micelles and bind to proteins. This review explores the role of moisturizers and cleansers, highlighting their mechanisms, types, usage recommendations, and impact on disease control and quality of life.