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This article discusses a case of VEXAS syndrome, a genetic autoinflammatory disorder that presents with various cutaneous manifestations, hematological abnormalities, and systemic inflammation. The case involves a 54-year-old Indian male who presented with red, raised, and mildly painful skin lesions, along with other symptoms such as fever, weight loss, and joint pain. The patient had a history of nodular skin lesions, scleritis, and diabetes mellitus. Laboratory evaluations revealed abnormalities in blood counts and bone marrow biopsy confirmed myelodysplasia. Genetic analysis confirmed the diagnosis of VEXAS syndrome. Treatment options for VEXAS syndrome are limited, with oral glucocorticoids being the most reliable. The article emphasizes the importance of dermatologists being aware of the cutaneous manifestations of VEXAS syndrome and collaborating with hematologists for multidisciplinary care. [Extracted from the article]

The Indian Association of Dermatologists, Venereologists, and Leprologists (IADVL) has established the I-CuReD registry in India to collect data on rare skin disorders. The registry has recorded 323 patients with genodermatoses, with epidermolysis bullosa and congenital ichthyosis being the most common diseases. The article provides detailed information on the demographics, symptoms, and diagnostic methods used for these patients. The study found a lower number of cases compared to other countries and highlights the need for further research in this area. The authors acknowledge the limitations of their study and hope for more comprehensive studies in the future. [Extracted from the article]

Linear and annular lupus panniculitis of the scalp is a rare form of lupus panniculitis recently reported in literature. It presents as linear or annular or arciform areas of nonscarring alopecia of the scalp with minimal surface changes. We report a 4‑year‑old Indian female child with arciform erythematous plaque over the forehead extending on the scalp with nonscarring alopecia with annular erythematous plaque over the cheek. Histology showed lobular lymphocytic panniculitis with abundant mucin deposition. Antinuclear antibody and systemic screen for lupus were negative. After treatment with oral corticosteroids, complete remission was achieved with good regrowth of scalp hair with no relapse during the follow‑up period of 6 months. This is the youngest reported case of linear and annular lupus panniculitis of the scalp. [ABSTRACT FROM AUTHOR]

When the host immunity decreased due to nutritional factors, stress, etc., lymphatic spread to the draining inguinal lymph nodes and contiguous infection of overlying skin resulting in the formation of discharging sinuses and scrofuloderma followed. A 5-year-old Indian girl presented with a verrucous plaque over the right knee, followed by pus-discharging sinuses with underlying nodules over the right inguinal region for 6 months. Based on the classical morphology, a positive Mantoux test, corroborating histopathology, and positive acid-fast bacilli from lymph nodes, a diagnosis of tuberculosis verrucosa cutis (TBVC) with scrofuloderma and underlying tubercular lymphadenitis was made. [Extracted from the article]

Linear and annular lupus panniculitis of the scalp is a rare form of lupus panniculitis recently reported in literature. It presents as linear or annular or arciform areas of nonscarring alopecia of the scalp with minimal surface changes. We report a 4-year-old Indian female child with arciform erythematous plaque over the forehead extending on the scalp with nonscarring alopecia with annular erythematous plaque over the cheek. Histology showed lobular lymphocytic panniculitis with abundant mucin deposition. Antinuclear antibody and systemic screen for lupus were negative. After treatment with oral corticosteroids, complete remission was achieved with good regrowth of scalp hair with no relapse during the follow-up period of 6 months. This is the youngest reported case of linear and annular lupus panniculitis of the scalp., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Indian Dermatology Online Journal.)