BACKGROUND:Rituximab is effective in both systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and cutaneous sclerosis. However, most studies report improvement in skin sclerosis as a secondary outcome, leaving a gap in the literature on rituximab's dermatological impact. OBJECTIVES:To evaluate rituximab's effectiveness in reducing skin sclerosis. METHODS:This retrospective study was conducted at a tertiary care centre in northern India. Records of patients with SSc from 2016 to 2023 were reviewed. Clinical assessments, investigations and standard treatment for end-organ damage were undertaken according to clinical presentation and the European Alliance of Associations for Rheumatology guidelines. Rituximab was administered in two doses of 1 g each, administered 2 weeks apart to patients with a baseline modified Rodnan skin score (mRSS) of ≥ 14 with or without ILD or a baseline mRSS of ≥ 7 and < 14 in the presence of ILD, as per a departmental protocol. Patients were divided into two groups: those receiving rituximab (the rituximab group) and patients who were not treated with rituximab (the non-rituximab group). Treatment response was evaluated based on change in mRSS and overall survival. RESULTS:Among 98 patients with SSc, 37 received rituximab and 61 were in the non-rituximab group. The rituximab group had a higher percentage of patients with diffuse skin sclerosis (DSS) [70% (26/37) vs. 30% (18/61)] and a higher mean baseline mRSS [20.1 (SD 9.4) vs. 11.5 (SD 8.0)]. There was a significantly greater mean percentage reduction in mRSS [47.9% (SD 27.4) vs. 31.2% (SD 31.4), P = 0.01] in the rituximab group compared with the non-rituximab group. Overall survival (Kaplan-Meier curve analysis) was 66% at 140 months in the rituximab group compared with 53% at 95 months in the non-rituximab group (P = 0.85). Patients with limited skin sclerosis (LSS) had better survival (78% at 100 months) compared with those with DSS (47% at 140 months, P = 0.05). On binomial logistic regression analysis, a greater percentage reduction in mRSS was the strongest predictor of survival (P = 0.04). CONCLUSIONS:Rituximab treatment resulted in a significant reduction in skin sclerosis even in patients with more severe baseline skin sclerosis, although it did not demonstrate any survival benefit.

Epidermal naevi arise because of cutaneous mosaicism. The epidermolytic variant of this naevus is a result of a postzygotic mutation in the KRT1 or KRT10 gene. Occasionally, this mutation can manifest in progeny as generalized KRT10-nonsyndromic epidermal differentiation disorder (nEED)-epidermolytic (formerly epidermolytic ichthyosis). We report an infant with KRT10-nEDD-epidermolytic and his father had an KRT10-nEDD-mosaic (formerly verrucous epidermal naevi) with a similar mutation in KRT10.

Bullous pemphigoid (BP) is known to be associated with various comorbidities such as diabetes mellitus and neurological disorders. However, its association with human immunodeficiency virus (HIV) has been occasionally reported. We hereby report a case of a middle-aged female with BP as well as HIV infection and explore the association between the two conditions.

A 27-year-old woman presented to the outpatient dermatology clinic with complaints of nonitchy reddish papules over the trunk and thighs for 2 months. The rash was sudden in onset and rapidly progressive. She also complained of cough with expectoration along with low-grade fever for 6 months. She had experienced a weight loss of 12 kg during this time. General examination revealed bilateral cervical lymphaden­opathy with firm, nontender lymph nodes, the largest lymph node measuring 2 × 2 cm. On cutaneous examination, multiple erythematous to violaceous flat-topped follicular papules arranged in a grouped configuration were observed on the abdomen, lower back, chest and inguinal area. The papules measured 2–3 mm and were associated with minimal scaling. On dermoscopy, pale to whitish monomorphic large dots were observed in the perifollicular regions with central blackish follicular openings or plugs and mild scaling in some areas. A 3.5-mm punch biopsy was obtained from the lesion on the abdomen, which on histopathological examination revealed perifollicular epithelioid cell granulomas along with multinucleated giant cells in the upper dermis. Lymphocytic cuffing was present around the granulomas along with mild perivascular lymphocytic infiltrate in the mid dermis. The epidermis was unremarkable. Ultrasound examination of the cervical region revealed bilaterally enlarged cervical lymph nodes (right > left) at level 1–5, with matting observed in the right cervical area (level 4). Necrotic changes were also visualized. Fine needle aspiration cytology (FNAC) from a right posterior cervical lymph node showed many well-formed epithelioid cell granulomas with polymorphous reactive lymphoid cells in the background. Ziehl–Neelsen stain was positive for acid-fast bacilli from the FNAC smear; however, mycobacterial culture was negative. Mantoux test showed a strong positivity, measuring 25 × 25 mm. High-resolution computed tomography scan of the chest was performed, which showed patchy consolidation with multiple centrilobular nodules and interstitial septal thickening of the right lung along with mediastinal lymphadenopathy. A final diagnosis of lichen scrofulosorum with pulmonary tuberculosis (TB) and cervical TB lymphadenitis was made. The patient was subsequently started on weight-based antitubercular therapy (ATT) comprising isoniazid (225 mg), rifampicin (450 mg), pyrazinamide (1200 mg) and ethambutol (825 mg). There was significant clearance of the cutaneous lesions after 2 months of ATT and the patient continues to be under follow-up. Lichen scrofulosorum is a rare tuberculid that occurs in children and young adults. As it can occur in both pulmonary and extrapulmonary forms of TB, a thorough history, cutaneous and systemic examination, and histopathology, microbiology and imaging are essential for prompt diagnosis and early initiation of ATT.

BACKGROUND:The introduction of the World Health Organization's multidrug therapy (WHO-MDT) has significantly advanced leprosy treatment. Although dapsone is a fundamental component of MDT, it presents risks of adverse drug reactions (ADR), including the potentially fatal dapsone hypersensitivity syndrome (DHS). METHODS:Consequently, we conducted a retrospective observational study by reviewing the records of patients registered at the leprosy clinic within the department of dermatology at a tertiary care center from 2010 to 2022. We included all patients who experienced ADR due to dapsone that necessitated its discontinuation, with particular emphasis on those who developed DHS. RESULTS:Among 1598 leprosy patients treated with standard WHO-MDT, 45 patients developed ADR requiring cessation of dapsone (2.82% over 12 years). The most common ADR was abnormal liver function tests in 14 patients (0.87%) and anemia in 13 patients (0.81%), including one case each of hemolytic anemia and methemoglobinemia. Neuropsychiatric adverse effects were observed in five patients (0.31%). The incidence of DHS was 0.75% (12 patients), with maculopapular rash being the most frequent cutaneous manifestation. These patients were subsequently treated with MDT without dapsone, along with standard care for the ADR, resulting in no mortality or serious morbidity. CONCLUSION:While dapsone remains a cornerstone in the treatment of leprosy, the potential for clinically significant though infrequent adverse reactions highlights the need for vigilant monitoring and increased awareness among healthcare providers of the diverse manifestations of dapsone-related side effects.

BACKGROUND:There is a lack of literature on stabilizing and repigmenting potential of oral tofacitinib in active vitiligo. OBJECTIVE:To compare the efficacy of oral tofacitinib with oral mini-pulse therapy in active vitiligo. METHODS:This prospective, randomized, investigator-blinded trial recruited patients aged 18-60 with active nonsegmental vitiligo. Participants were randomized to receive dexamethasone 2.5 mg twice a week (group A) or oral tofacitinib 5 mg twice daily (group B) for 24 weeks, followed by 12 weeks of observation. The primary outcome was the proportion of patients showing ≥50% improvement in vitiligo extent score (VES) at 24 weeks. RESULTS:Sixty patients were recruited (30 per group), with 49 completing 36 weeks. At 24 weeks, the proportion achieving ≥50% VES improvement was similar (16.7% vs 20.0%, P = .833). By 36 weeks, group B had a significantly greater VES decrease (31.5 ± 24.9% vs 16.7 ± 34.8%, P = .031). Group A had higher treatment failure at 12 weeks (20% vs 3.3%, P = .049), but comparable stabilization rates at 24 weeks (63.3% vs 83.3%, P = .171). LIMITATIONS:Limited sample size, single-center design, and lack of double-blinding. CONCLUSION:Tofacitinib is more effective than oral mini-pulse (dexamethasone 2.5 mg twice a week) for treating active nonsegmental vitiligo.

Atopic dermatitis (AD) is a complex immune-mediated disease characterised by recurrent eczematous lesions and pruritus, which adversely affects the quality of life (QoL). Genetic factors, environmental factors, immune dysregulation, and skin barrier dysfunction contribute to its pathophysiology. Non-pharmacological management strategies aim to preserve the skin barrier, address immune dysregulation, and minimise triggers. In this review, wediscuss various non-pharmacological interventions, including allergen (aeroallergens, food allergens, and contact allergens) avoidance, bathing-related measures, moisturisers, clothing choices, therapies targeting the skin microbiome, and allergen-specific immunotherapy, in addition to education and psychotherapy. Non-pharmacological therapies are essential for the holistic management of AD, but their effectiveness varies, highlighting the need for further research and tailored approaches to individual patient needs.

Citation: Gupta P, Dev A, Vinay K, Bishnoi A, Sendhil Kumaran M, Parsad D. Validating a Novel Technique of Dermatoscopic Imaging or Sequential Comparison of Target Lesions in Vitiligo. Dermatol Pract Concept. 2024;14(1):e2024003. DOI: https://doi.org/10.5826/dpc.1401a3

Abstract An 8-year-old male child presented with a history of multiple vesicles and bullae on the ear, nose, upper trunk, lower legs and feet for the past 4 years. There was no history of photosensitivity. There was no history of similar conditions in family members. On examination, the child had erosions with haemorrhagic crusting on the above-mentioned sites. The lesions were healing with hypopigmentation and scarring. A few milia-like lesions were seen on the nose and ears. Superficial tiny erosions with haemorrhagic crusting were present on the lip mucosa and tongue. Dystrophy of the left great toe was also noted. Differential diagnoses of Kindler syndrome, porphyria cutanea tarda, bullous pemphigoid and chronic bullous dermatosis of childhood were considered. A 3-mm punch biopsy from the vesicle revealed epidermis with psoriasiform hyperplasia and hyperkeratosis. The upper dermis showed a perivascular and perifollicular lymphomononuclear infiltrate with multiple eosinophils. A perilesional 3-mm punch biopsy was sent for direct immunofluorescence, which revealed at least three IgG linear deposits along the dermoepidermal junction in an N-serration pattern. There were no IgA or IgM deposits. A genetic analysis showed no mutation in the FERMT1 gene. Porphyrin levels in the blood, urine and stool were normal. Based on the histopathology and direct immunofluorescence findings, the patient was diagnosed with childhood bullous pemphigoid. Weight-based azathioprine was started and there was improvement in the lesions within 2 months of initiation of therapy. Bullous pemphigoid is a subepidermal blistering disorder that commonly affects older patients (60–80 years), but it can rarely affect children. It demonstrates considerable clinical and histopathological overlap with other acquired and/or congenital blistering disorders. A high index of suspicion coupled with appropriate investigations are imperative for diagnosis. Histopathology and direct immunofluorescence are more cost-effective. In a limited-resource setting, this can eliminate the need for more expensive tests, such as genetic analysis.

Abstract Dermatological manifestations in chronic granulomatous disease (CGD) include skin infections and sterile cutaneous inflammatory lesions. The objective of this study was to assess the clinical and histopathological features of noninfectious skin lesions in patients with CGD. This was a cross-sectional observational study from January 2022 to June 2023, carried out at a tertiary care centre. Forty-eight patients with CGD who were registered in the paediatric immunology clinic were screened for cutaneous noninfectious lesions during their routine follow-up. We describe the frequency of cutaneous noninfectious lesions among patients with CGD, along with their clinicodemographic and histopathological features. Noninfectious inflammatory skin lesions were present in 11 of 48 (23%) patients with vCGD. The mean age of this cohort (n = 11) was 19.8 years (SD 5.2). Centrofacial papulopustular lesions and erythematous infiltration involving the nose and the perinasal area (nose sign) were observed in 5 of 11 patients. Five patients presented with yellowish-orange, discrete, papular lesions involving the face and trunk, mimicking acne-like eruptions. Two patients had lesions of hidradenitis suppurativa. Histopathological examination revealed ill-formed granulomas with a mixed inflammatory infiltrate in the patients with facial lesions, while three patients with acneiform eruptions showed well-formed granulomas. The median age at onset of symptoms of CGD (48 vs. 7 months, P = 0.005; difference between groups 35 months, 95% confidence interval 12–78) and the median age at diagnosis of CGD (150 vs. 48 months, P = 0.008, difference between groups 72 months, 95% confidence interval 22–132) were significantly higher in patients with CGD who developed noninfective, inflammatory skin lesions compared with those without such skin lesions. The proportion of patients with NCF1 mutation was significantly higher in patients with noninfective inflammatory skin lesions (73% vs. 27%, P = 0.011). No correlation was seen between the type of skin manifestation and pathogenic mutation. In conclusion, inflammatory noninfectious cutaneous lesions can occur in CGD in the form of perinasal infiltrated papules and pustules (nose sign), as well as acne­iform lesions, and can be clues towards clinical diagnosis.

This cross-sectional study examines cutaneous inflammatory presentations of chronic granulomatous disease.

The authors declare no conflict of interest.