Weber-Christian disease, or idiopathic panniculitis, is a rare disease that was first discovered by Pfeifer in 1892 and later better described clinically and histologically by Weber and Christian in 1925 and 1928 [3]. It mainly affects adults and predominantly females. Clinically, it is characterized by recurrent episodes of fever and painful subcutaneous nodules with recurrent purplish lesions of small diameter, usually 1 to 2 cm, on an erythematous base, mainly located on the lower limbs (thighs, legs, and hips). The lesions soon become softer, forming cysts that leave an atrophic hyperpigmented scar [4]. In the present case, lesions were more frequently located on both thighs, the hips, and the lower abdomen. Multisystem involvement, respiratory problems (cough, dyspnea, chest pain), cardiac or renal problems (proteinuria, hematuria), hepatosplenomegaly, digestive system problems (abdominal pain and bloating, diarrhea, digestive bleeding, mesenteric involvement), and rarely neurological (cerebellar) bleeding are associated with high morbidity and mortality rates [5].

The patient in the present case was diagnosed with Weber-Christian disease after neutrophil infiltration and all etiologies of lobular panniculitis (enzymatic causes, antitrypsin α1 deficiency, infectious panniculitis) were excluded. 

Neutrophilic Panniculitis (NP) is a clinical-histological entity that is grouped together with several dermatoses characterized by similar clinical manifestations, presenting commonalities and possibly being associated with systemic diseases and hemopathies. In fact, NP may precede the possibility of an underlying Myelodysplastic Syndrome (MDS), which may be detected even during clinical follow-up, or may be a warning sign of progression to acute myeloid leukemia [6]. The rate of association between NP and MDS was found to be significantly higher, reaching 75% in a series of 8 cases [7]. Weber-Christian disease or idiopathic recurrent febrile lobular panniculitis is more frequently noted in the literature in adults and is rarely observed in children, but according to most authors it is a nonspecific entity, possibly encompassing other more specific types of panniculitis [8].

The incidence and prevalence of Weber-Christian disease are unknown, both in the United States and internationally. Weber-Christian disease is rare in adults and even rarer in children. However, albeit rarely, it does occur in children. It has been most commonly reported in people in the fourth to seventh decades of life, with 75% of cases occurring in women after the second decade of life [9]. For example, in a review of children and adolescents in Brazil over a 20-year period (1983-2002), 35 cases of pediatric and adolescent panniculitis were identified, with only 6 cases meeting the criteria for Weber-Christian disease [9].

As in the present case involving the follow-up of a patient with a second attack of Weber-Christian disease, cutaneous findings may be an indicator of an underlying malignant disease. The preferred treatment for idiopathic panniculitis is corticosteroid therapy [10]. In most cases, it will help control exacerbation and dissolve skin lumps, but starting this treatment may mask any underlying disease for a while. In patients with mainly isolated skin manifestations, the clinical course may be characterized by exacerbations and remissions of cutaneous lesions for several years before the disease subsides.