Metastatic Crohn Disease: A Clinicohistological Appraisal From a Tertiary Care Center in India.

BACKGROUND: Granulomatous dermatitis due to noncontiguous involvement of Crohn disease (CD) of the gut has been described as metastatic CD (MCD). MCD is the rarest form of cutaneous manifestations of CD. This study aims to analyze the clinicohistological features of MCD in a tertiary care center of India. MATERIALS AND METHODS: A retrospective review of patients diagnosed clinically and histologically with MCD over past 5 years was performed. Data on cutaneous features, histological findings, and response to treatment were collected. RESULTS: Twelve patients (3 men and 9 women) with a mean age of 29 years were identified. All women had vulval involvement in the form of edema (80%), ulceration (60%), and fistula (20%). Among the 3 men, 2 had perineal and scrotal swelling and ulcer, whereas the third patient presented with leg ulcer. Intestinal CD was already diagnosed in 50% patients (5/10) at cutaneous presentation, whereas it was diagnosed subsequently in 30% (3/10) cases. Histological examination revealed nonnecrotizing granulomatous inflammation in the dermis in 11 patients (92%). Additional histological features included eosinophilic infiltrate (58%), panniculitis (33%), and vasculitis (33%). The patients were treated with various combinations of oral prednisolone, metronidazole, minocycline, azathioprine, and subcutaneous adalimumab with partial relief. CONCLUSION: MCD shows a wide spectrum of clinical presentation, with anogenital involvement being the most common. Histology reveals nonnecrotizing granulomas in the dermis in majority of the cases. The diagnosis is extremely challenging in patients without gastrointestinal involvement at presentation, and thus, a high index of suspicion is imperative.

Filaggrin gene mutations in hand eczema patients in the Indian subcontinent: A prospective case-control study.

BACKGROUND: There are no Indian studies on the association between filaggrin gene (FLG) mutations and any dermatosis, including hand eczema. OBJECTIVES: To determine the prevalence of FLG mutations in Indian hand eczema patients, and examine associations between such mutations and any aetiological type of hand eczema. MATERIALS AND METHODS: A total of 163 patients and 86 controls were included. Patients were categorized into aetiological subtypes of hand eczema. FLG polymorphisms (S2889X, 2282del4, R501X, and Q2417X) were determined in patients and controls, and correlated with subtypes. RESULTS: The prevalences of FLG mutations were 33.7% in cases and 3.5% in controls. Mutations in S2889X constituted 96.4% of all FLG mutations. No carrier of R501X and Q2417X mutations was identified. Among 55 patients with mutations, irritant contact dermatitis (ICD) with or without atopy was found in 22 patients, allergic contact dermatitis (ACD) with or without atopy was found in 12, and idiopathic hand eczema was found in 12. There was a significant association of FLG mutations with ICD with or without atopy, ACD without atopy, and idiopathic subtypes. FLG mutations were associated with more severe hand eczema. CONCLUSIONS: S2889X mutation is commoner in patients than in controls. FLG polymorphisms are associated with specific subtypes of hand eczema and severe disease.

Generalized granulomatous dermatitis following Mycobacterium w (Mw) immunotherapy in lepromatous leprosy.

Mycobacterium w (Mw) vaccine is a heat-killed suspension derived from a nonpathogenic, cultivable, atypical mycobacterium named Mycobacterium indicus pranii. Mw immunotherapy has been reported to be efficacious as an adjunct to multidrug therapy multibacillary regimen in leprosy patients with high bacillary index. Cutaneous reactions are predominant adverse effects associated with the administration of vaccines. Cutaneous adverse effects ascribed to Mw vaccine are generally limited to the site of injection. We herein describe two cases of lepromatous leprosy who developed an unusual generalized cutaneous reaction following Mw immunotherapy. A high index of suspicion is needed to identify such manifestations in leprosy cases to avoid misdiagnosis of a relapse or a reaction and for appropriate treatment.

Isolated benign primary cutaneous plasmacytosis in an adult Indian male.

Primary cutaneous plasmacytosis (PCP) is an uncommon reactive lymphoplasmacytic disorder of uncertain etiology. It has been mainly described in patients of Japanese descent, with only few reports in Caucasians and Chinese. We present a case of isolated benign PCP in a 45-year-old man, who clinically manifested with a localized ulcerated nodule overlying a hyperpigmented plaque on the upper back. To the best of our knowledge, PCP from India has not been described before.

Disseminated cutaneous BCG infection following BCG immunotherapy in patients with lepromatous leprosy.

Cutaneous complications of Bacillus Calmette-Guerin (BCG) vaccine, especially in the form of generalised disease, are uncommon and mostly occur in immunocompromised individuals. There is a paucity of data on the cutaneous adverse reactions secondary to BCG immunotherapy in leprosy. We report two unique cases of disseminated cutaneous BCG infection following immunotherapy in patients with lepromatous leprosy. To our knowledge, cutaneous BCG infection presenting as widespread lesions after immunotherapy and confirmed by isolation of Mycobacterium bovis by polymerase chain reaction (PCR) has not been described. A high index of suspicion is required when leprosy patients who receive BCG immunotherapy develop new lesions that cannot be classified as either reaction or relapse, and diagnosis may be confirmed on histopathology and PCR.

Childhood leprosy through the post-leprosy-elimination era: a retrospective analysis of epidemiological and clinical characteristics of disease over eleven years from a tertiary care hospital in North India.

BACKGROUND: Children are believed to be the most vulnerable group for leprosy and childhood leprosy reflects disease transmission in the community as well as the efficiency of ongoing disease control programmes. Objectives: To study the epidemiological and clinical trends of childhood cases of leprosy at a tertiary care hospital in North India during 2001-2011. METHODS: A retrospective study was undertaken analysing the clinic records of children with leprosy less than or equal to 18 years registered at the leprosy clinic of this institute over an 11-year period. Demographic and disease characteristics including age, sex, history of contact, duration of disease, clinical pattern, bacteriological and histopathological parameters, reactions and disabilities were noted from a predesigned format. RESULTS: A total of 1225 cases of leprosy were registered during this period, of whom 59 (4·81%) were children. The mean age of the patients was 10·06 ± 3·35 years with a male preponderance (3·9: 1). History of close contact with a leprosy case was present in 15 (25·4%) patients. Mean duration of illness before diagnosis was 18·5 months (range: 1-70 months). Borderline tuberculoid (BT) was the commonest clinical type in 40 children (67·8%), followed by lepromatous (LL) in 7 (11·9%), borderline lepromatous (BL) in 6 (10·1%), pure neuritic (PNL) in 2 (3·4%), tuberculoid (TT), mid-borderline (BB), histoid and indeterminate leprosy in 1 patient (1·7%) each. Lesions were located over upper extremity in 32 (54·2%), lower extremity in 29 ( 49·2% ), face in 27 ( 45·8%) and trunk in 26 ( 44·1%) patients. A single lesion was observed in 23 (39%), 2-5 lesions in 12 (20·3%) and more than five lesions in 22 (37·3%) children. The slit skin smear was positive in 17 (28·8%) patients. Lepra reactions were observed in 20 patients (33·9%), of whom 14 (70%) had Type 1, and six (30%) had Type 2 lepra reaction. Thickened peripheral nerve trunks were present in 48 (81·4%) children, of which, 27 (56-3%) had more than one thickened nerve and 21 (43·7%) had only a single nerve involved. Neuritis occurred in 9 (15·3%) and disability (both· grade 1 and 2) at the time of diagnosis was noted in 24 (40-7%) patients. Six (10·2%) children defaulted from treatment. Three cases (5·1%) of relapse were observed. CONCLUSIONS: Childhood leprosy cases and their complications continue to present in alarming numbers in India and this suggests possible gaps in the national programmes aimed at leprosy elimination. We stress the importance of continuous and sustained efforts for early case detection in the community in general, and close follow-up of susceptible children amongst household contacts of leprosy cases in the post-leprosy-elimination era.