Lichen planus pemphigoides (LPP) is a rare autoimmune bullous disorder that is rarer in children characterized by bullae on lichen planus like papules and the normal looking skin. Clinical, histopathological and direct immunoflourescence evaluation is important for the diagnosis of this entity. We report a case of LPP in a 5-year-old girl child probably triggered by an episode of preceding varicella. Diagnosis was confirmed by histopathology and immunofluorescence examination. Histological findings were typical of lichen planus from the biopsy taken from a violaceous to erythematous papule and characteristic of bullous pemphigoid in the biopsy taken from a bullous lesion with evidence of immunoglobulin G and C3 deposition along the basement membrane zone on direct immunofluorescence.
Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare autosomal recessive disorder characterized by various dermatological and systemic manifestations such as lipoatrophy, hypertriglyceridemia, hepatomegaly, acanthosis nigricans, and acromegaloid features. BSCL type 2 is more common and severe, with onset in the neonatal period or in early infancy. The locus for BSCL2 has been identified on chromosome 11q13. Early recognition and differentiation from other congenital generalized lipodystrophies help in the initiation of appropriate preventive and therapeutic measures such as lifestyle modification and pharmacotherapy that helps postpone the onset of metabolic syndrome. We report BSCL type 2 in two siblings with several cutaneous manifestations like acanthosis nigricans, hypertrichosis, prominent subcutaneous veins, and increased lanugo hair.
Elastosis perforans serpiginosa (EPS), characterized by transepidermal elimination of fragmented elastic fibers, clinically presents as hyperkeratotic papules. EPS is classified into three types: (1) Idiopathic; (2) reactive, with associated connective tissue diseases such as pseudoxanthoma elasticum (PXE), Ehlers–Danlos syndrome, cutis laxa, Marfan syndrome, osteogenesis imperfecta, Down's syndrome; (3) the one that is induced by D-penicillamine. A rare association of EPS with PXE, which is primarily a defect of transmembrane transporter protein with accumulation of certain metabolic compounds and secondary calcification of elastic fibers has been documented in the literature. We report a case of PXE with associated lesions that were histopathologically compatible with EPS.
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