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Dear Editor, Granuloma annulare (GA) is a granulomatous, idiopathic, inflammatory skin disorder characterized by the formation of papules and plaques with annular and acral distribution. 1 GA is often limited and self-resolving, but in some cases, it can be generalized and refractory toThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

Differential diagnosis of dermatoses presenting with skin fragilityand blisters in photo-exposed areas is broad and includes porphyrias, autoimmune bullous diseases (ABD), and phototoxicities such as pseudoporphyria. 1 Pseudoporphyria is a bullous photodermatosis with a clinical presentation and histology similar to that of porphyria cutanea tarda (PCT), although with no porphyrin increase. 1,2 Pseudoporphyria is infrequent (<150 reported cases), probably underdiagnosed/underreported, and can represent a diagnostic challenge. 1,2 Here, we present a series of cases with pseudoporphyria and propose a diagnostic algorithm.

Monoallelic NLRC4 gain‐of‐function variants cause an inflammasomopathy with diverse clinical forms including infantile enterocolitis, recurrent macrophage activation syndrome, cold‐induced urticaria‐like lesions (or familial‐cold autoinflammatory syndrome, FCAS4), and painful subcutaneous nodules. Here, we identified a large family with six consecutive generations affected. Genetic analyses detected the heterozygous p.Ser445Pro NLRC4 variant in three patients, which has been previously reported in a Dutch family with FCAS4. We aimed to describe the clinicopathological features and the functional consequences of the detected NLRC4 variant. Patients presented an early‐onset (3 months–6 years) inflammatory disease characterized by recurrent panniculitis, fever and arthralgia. Histopathological examination showed perivascular and interstitial lymphohistiocytic infiltrates in the dermis and mixed panniculitis. Functional analysis supported the conclusion that the p.Ser445Pro NLRC4 variant leads to a constitutive activation of NLRC4‐inflammasome and increased plasma levels of IL‐18. Prompt recognition of early‐onset panniculitis through clinicopathological examination and laboratory biomarkers may allow targeted therapies.

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