Familial erythema nodosum

Erythema nodosum (EN) is a hypersensitivity reaction associated with many diseases. We describe a family in which 4 sisters had acute or recurrent EN. HLA typing showed a common haplotype in the affected members of the family. A review of familial EN and HLA distribution in EN is presented.     

HLA-DRB1 associations in biopsy proven erythema nodosum.

OBJECTIVE: To examine the HLA-DRB1 associations of patients with erythema nodosum (EN), establish HLA-DRB1 differences among patients with idiopathic and secondary EN, and identify the HLA-DRB1 associations with specific conditions presenting with EN. METHODS: We conducted a retrospective study of 100 patients (83 women) with biopsy proven EN diagnosed for a defined population in Northwest Spain. Patients were classified into idiopathic or secondary if skin nodules occurred in the context of a well defined disease, or there was a precipitating event in close temporal relationship with the onset of EN. Patients and controls were HLA-DRB1 genotyped from DNA using molecular based methods. RESULTS: At the time of diagnosis no precipitating events or underlying diseases were identified in 35 cases and, due to this, they were considered to be idiopathic. Although strong associations do not appear to exist between HLA-DRB1 and unstratified EN, the idiopathic group showed a significantly lower frequency of HLA-DRB1*04 phenotype compared with controls. Among the patients with secondary EN, the patient subgroup with sarcoidosis exhibited a significantly increased frequency of HLA-DRB1* 13 compared with healthy controls. CONCLUSION: Idiopathic and some groups of secondary EN seem to have different HLA-DRB1 associations. These differences may have prognostic value in identifying patients with specific conditions associated with this syndrome.     

Histoid leprosy with erythema nodosum leprosum

Histoid leprosy is a particular variant of lepromatous leprosy presenting as cutaneous or subcutaneous nodular and/or plaque-like lesions arising form apparently normal skin. It is characterized histologically by spindle-shaped histiocytes in interlacing bundles and whorls, containing numerous intact and rod-shaped Mycobacterium leprae. It can occur de novo or secondary in patients treated for a long course by dapsone alone. We describe a case of lepromatous leprosy treated according to the national Moroccan protocol who developed histoid lesions during his treatment by dapsone. The patient responded well to fluoroquinolone, rifampicin and clofazimine, with however, the occurrence of erythema nodosum leprosum.     

Risk factors for erythema nodosum leprosum

A retrospective study of new borderline lepromatous and lepromatous patients reporting for multidrug therapy (MDT) for leprosy at the Anandaban Leprosy Hospital, Kathmandu, Nepal, over an 8-year period was conducted to determine the prevalence of erythema nodosum leprosum (ENL), the time and frequency of reactions, and clinical and laboratory parameters associated with ENL. An overall prevalence of ENL in this cohort of 19% was found. One third of these reactions occurred in patients before MDT was given, one third in the first 6 months and one third after 6 months of treatment. Nearly 1 in 10 of the ENL reactions occurred in patients who had completed 2 years of MDT; 45% of patients with ENL had more than one episode. Data collected at the patients' first presentation was used to identify four major risk factors. Patients with lepromatous disease, skin infiltration or a bacterial index (BI) of > 4+ were at significantly increased risk. Patients older than 40 were at significantly decreased risk of ENL. There was a linear relationship in the risk of ENL with an increasing BI and an inverse relationship to increasing age. These observations should enable clinicians to recognize patients at first presentation who will be likely to develop ENL.     

Necrotic course of erythema nodosum leprosum

During 2 years, 25 lepromatous patients were hospitalised in the Hansen complications room of Institut Marchoux. Between these patients, 9 developed a Necrotic Erythema Nodosum Leprosum. A review of the observed clinic effects is established and 3 types of signs are isolated and discussed: necrotic extension after big nodes on chest and arms, a punch crater complicating small nodes, and a sclerous xylodermia on arms and legs. The course of this complication is estimated about six months average, with pauses and relapses with general and subjectives symptoms. The final course shows side effects: anemia, denutrition, functional disabilities of joint movements and cutaneous straps. In the group of 9 patients, 3 died. We did not find relations between the necrotic phenomenon and therapy or occurred diseases. The best drug to stop the necrotic processus is thalidomide 400 mg daily and decrease 100 mg when the signs fall near normality. One or two mg/kg each day coricosteroides were tested: the effect is unconstant, the duration of action is shorter. Side effects occur rapidly and patient will become corticostero?d dependent. The importance of bath with disinfectants is high. We did not observed surinfection.     

Giant cell arteritis of the skin simulating erythema nodosum.

Cutaneous involvement in giant cell arteritis is quite uncommon. A patient is described who presented with pretibial skin lesions clinically indistinguishable from erythema nodosum which, on biopsy, showed subcutaneous pannicular giant cell vasculitis. Cutaneous manifestations of giant cell arteritis are subsequently reviewed.     

Recurrent erythema nodosum: a diagnostic dilemma

Erythema nodosum is the most frequent clinico‐pathological variant of the panniculitides. We describe a case of a young lady who suffered from recurrent attacks of erythema nodosum over the anterior side of the lower extremities which was confirmed by histopathological finding. She was treated with corticosteroid and immuno‐suppressive agents without much improvement. She responded well to oral colchicine.