Penile Crohn''s disease: a case report.

Metastatic Crohn's disease is a rare inflammatory condition characterised by cutaneous granulomatous lesions separated from the affected bowel by normal intact skin. Involvement of the genitalia in Crohn's disease is rare and consists of ulcerated lesions in almost all of the cases reported in the literature. We describe a case of penile involvement in a 27 year old man with a 5 year history of Crohn's disease. Should genital involvement precede the bowel disease, patients may consult the sexually transmitted disease service for this problem and the dermatovenereologists may be the first to formulate the diagnosis.     

Cutaneous metastatic Crohn disease

A 27-year-old patient had firm, subcutaneous nodules on both legs. Histological examination revealed that they were epitheloid granulomas with necrotic areas. After the exclusion of infectious granulomas this finding suggested an association with Crohn's disease. Clinical investigations showed an acute form of Crohn's disease. We interpreted our findings as a cutaneous granulomatous reaction in Crohn's disease. In the literature this is referred to as metastatic Crohn's disease.     

Granulomatous rosacea and Crohn's disease in a patient homozygous for the Crohn-associated NOD2/CARD15 polymorphism R702W

NOD2/CARD15 belongs to the N-terminal caspase recruitment domain family of proteins involved in regulating NF-kB activation in response to inflammatory stimuli transduced through Toll-like receptors. Mutations and polymorphisms in the NOD2/CARD15 gene reduce antibacterial responses and are associated with granulomatous inflammatory conditions such as Blau syndrome and early-onset sarcoidosis. The polymorphism R702W (arginine to tryptophan) is strongly associated with susceptibility to Crohn's disease in Caucasian populations. Skin abnormalities (other than cutaneous manifestations of Crohn's disease) have not been previously associated with R702W. We report on a female patient homozygous for R702W who developed granulomatous rosacea at the age of 12 years old. From the occurrence in the context of Crohn associated with R702W, we speculate that granulomatous rosacea may be an entity distinct from other forms of rosacea, which are associated with increased production of antibacterial proteins such as cathelicidin.     

Granulomatous rosacea associated with ulcerative colitis: 2 case reports

Inflammatory gastrointestinal disorders (ulcerative colitis and Crohn's disease) are often associated with cutaneous disorders, which occasionally may even precede the internal symptoms. Aside from the specific skin diseases, such as erythema nodosum or pyoderma gangraenosum, a number of unspecific skin eruptions may be found in both disorders. Here we report on 2 cases of ulcerative colitis associated with granulomatous rosacea.     

Lichenoid and granulomatous dermatitis associated with atypical mycobacterium infections

BACKGROUND: Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as well may be seen with lichenoid drug reactions, endogenous T-cell dyscrasias and as a feature of certain systemic diseases especially Crohn's disease and rheumatoid arthritis. RESULTS: We encountered three cases of lichenoid and granulomatous dermatitis in which the basis was one of primary cutaneous Mycobacterium infection. In all three cases acid fast stains revealed pathogenic organisms and as well cultures were positive for Mycobacterium kansasii in one case and Mycobacterium marinum in another. Other features included a prominent perineural and periadnexal lymphocytic infiltrate. CONCLUSIONS: The differential diagnosis of lichenoid and granulomatous dermatitis should also encompass primary cutaneous Mycobacterium infection in addition to the other more characteristic entities associated wtih this distinctive reaction pattern. Infection with Mycobacterium induces a TH1 dominant response which would hence produce an infiltrate.     

Giant cells in pyoderma gangrenosum

It has been claimed that pyoderma gangrenosum (PG) lesions may contain granulomatous foci when associated with Crohn's disease. To test this assertion, we obtained clinical histories and archived cutaneous biopsies from 34 PG patients. Thirteen of these patients had inflammatory bowel disease (IBD). Immunostaining with PGM1, a macrophage marker, revealed well-formed giant cells with three or more nuclei in biopsies from 6 of 13 patients with IBD. Five of the 6 biopsies came from patients with Crohn's disease and one from a patient with ulcerative colitis. Two were peristomal. In the 21 patients who had PG without IBD, no giant cells were seen. Thus, PGM1+ histiocytic giant cells within a PG lesion may be indicative of associated IBD (p = 0.006), particularly Crohn's disease.     

Cutaneous manifestation of Crohn disease

In a 20-year-old women having suffered from Crohn's disease for many years, we found granulomatous fistular ulcerations on her right distal lower leg. After histological verification of the clinical diagnosis, she was orally treated with metronidazole for 6 weeks with excellent results: The cutaneous lesions healed completely.     

Metastatic Crohn's Disease: A Case Report

Metastatic Crohn's disease (MCD) is a rare extraintestinal manifestation of Crohn's disease characterized by the histologic finding of granulomatous dermatitis at a site noncontiguous to the gastrointestinal tract. An adolescent had MCD of the face that was initially mistaken for severe, treatment-resistant acne. Histopathologic and microbiologic evaluation, combined with clinical examination and response to therapy, ultimately led to the correct diagnosis. Mycobacterium paratuberculosis is a suspected cause of Crohn's disease. Polymerase chain reaction was used to detect genomic DNA specific for the organism in biopsy specimens from the patient's cutaneous lesions. This study failed to demonstrate M. paratuberculosis in the specimens.     

Metastatic ulcerative penile Crohn''s disease

Metastatic Crohn's disease (MCD) is a cutaneous granulomatous reaction which affects patients with bowel disease in areas of the skin distant from the affected bowel. Penile involvement is a very uncommon observation. We report a case of penile ulcerative MCD in a 37-year-old man, which requested surgical management. The lesion developed during reactivation of the bowel disease.     

Lichenoid and granulomatous dermatitis

BACKGROUND: The prototypic lichenoid eruptions, lichen planus (LP), lichenoid drug eruptions, secondary syphilis, and collagen vascular disease, are defined histologically by a band-like lymphocytic infiltrate in close apposition to the epidermis. We describe a novel form of lichenoid dermatitis with a granulomatous component. DESIGN: Skin biopsies from 40 patients demonstrating a band-like lymphocytic infiltrate with concomitant granulomatous inflammation were encountered over 4 years. Clinicians were contacted to elucidate underlying triggers and medical illnesses. RESULTS: A lichenoid dermatitis, a linear eruption, vasculitis, annular erythema, and erythroderma were among the clinical presentations. A drug-based etiology was implicated in 14 cases: the drugs included antibiotics, lipid-lowering agents, anti-inflammatory drugs, antihistamines, hydroxychloroquine sulfate, and angiotensin-converting enzyme inhibitors. Over one-third of patients with drug-related eruptions had other medical illnesses associated with cutaneous granulomatous inflammation, namely rheumatoid arthritis (RA), Crohn's disease, hepatitis C, diabetes mellitus, and thyroiditis. A microbial trigger was implicated in 12 patients in the context of infective id reactions to herpes zoster, Epstein-Barr virus (EBV), or streptococci, or active infections by Mycobacterium tuberculosis, M. leprae, fungi, and spirochetes. The remainder had hepatobiliary disease and RA without obvious exogenous triggers, cutaneous T-cell lymphoma (CTCL), and idiopathic lichenoid eruptions (i.e. LP, lichen nitidus, and lichen striatus). One patient with LP had underlying multicentric reticulohistiocytosis. The histiocytic infiltrate assumed one or more of five light microscopic patterns: (i) superficially disposed loose histiocytic aggregates; (ii) cohesive granulomata within zones of band-like lymphocytic infiltration with or without deeper dermal extension; (iii) a diffuse interstitial pattern; (iv) scattered singly disposed giant cells; and (v) granulomatous vasculitis. Additional features included lymphocytic eccrine hidradenitis in those patients with drug reactions, hepatobiliary disease, and antecedent viral illnesses, tissue eosinophilia and erythrocyte extravasation in drug hypersensitivity, granulomatous vasculitis in patients with microbial triggers, drug hypersensitivity or RA, and lymphoid atypia in lesions of CTCL or drug hypersensitivity. CONCLUSIONS: The cutaneous lichenoid and granulomatous reaction may reflect hepatobiliary disease, endocrinopathy, RA, Crohn's disease, infection, or a drug reaction. One-fifth of cases represent idiopathic lichenoid disorders. Lymphoproliferative disease or pseudolymphomatous drug reactions must be considered in those cases showing lymphoid atypia.     

Granulomatous and suppurative dermatitis at interferon alfa injection sites: report of 2 cases

It has previously been reported that interferon alfa injection sites may develop pyoderma gangrenosum, interface dermatitis, vasculitis, or, more commonly, ulcers characterized by intravascular thrombi and a mixed inflammatory cell infiltrate. We describe 2 patients in whom granulomatous and suppurative dermatitis developed at interferon alfa injection sites. These cases extend the spectrum of interferon alfa injection site reactions. The histologic and clinical similarities of these cases with pyoderma gangrenosum and cutaneous Crohn's disease are explored.     

Crohn''s disease presenting as a breast abscess: A case report

A case of cutaneous Crohn's disease (CD) involving the areola-nipple region of both breasts in a 45-year-old woman is reported. The lesion had initially been diagnosed and treated as a simple abscess. Histopathological examination, however, showed granulomatous inflammation with eosinophils suggesting extraintestinal CD. The patient also had a minor area of cutaneous CD localized at the umbilicus and severe anogenital lesions. Twenty-six years previously the woman had undergone a course of sulphasalazine treatment together with steroid enema for chronic inflammatory colitis, but the colitis had been inactive for 25 years. Treatment with sulphasalazine, metronidazole, prednisolone and azothioprine had only minor effect on the skin lesions. CD is a systemic disorder and the most prominent manifestations may be extraintestinal.     

Granulomatous lymphangitis of the scrotum and penis. Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation

BACKGROUND: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis. OBJECTIVE: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling. Biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) and Crohn's disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn's disease) is evident 1 year later. Literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn's disease, frequently with anal involvement and a minority, both with and without Crohn's disease, had orofacial granulomatosis. CONCLUSIONS: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn's disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn's disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn's disease.     

Skin Signs of Rheumatoid Arthritis and its Therapy-Induced Cutaneous Side Effects

Rheumatoid arthritis (RA) is a systemic inflammatory disorder that primarily affects the joints, but may exhibit extra-articular, including cutaneous, manifestations such as rheumatoid nodules, rheumatoid vasculitis, granulomatous skin disorders, and neutrophilic dermatoses. A large burden of cutaneous disease may be an indication of RA disease activity and the need for more aggressive treatment. Many of the therapeutic agents used to treat RA can also result in cutaneous adverse effects, which pose their own diagnostic and therapeutic challenges. Anti-TNFα agents, in particular, have a wide variety of adverse effects including psoraisiform eruptions, granulomatous conditions, and cutaneous connective tissue disorders. Herein we provide an update on the clinical presentations and management of RA-associated cutaneous findings as well as drug-induced cutaneous effects, with particular attention to the adverse effects of biologic disease-modifying agents.     

Metastatic Cutaneous Crohn's Disease in Children: Case Report and Review of the Literature

Abstract:  Metastatic cutaneous Crohn's disease is a rare complication of Crohn's disease, especially in the pediatric population, and can present a diagnostic dilemma. Most of the reported cases of metastatic cutaneous Crohn's disease in childhood have concurrent gastrointestinal symptoms and/or perianal disease to aid in the diagnosis. We present a case of a 13½-year-old girl whose initial symptom of Crohn's disease was asymptomatic labial swelling. An overview of metastatic cutaneous Crohn's disease in childhood is also provided.     

Metastatic Crohn's disease: a histopathologic study of 12 cases

Perhaps, the most intriguing cutaneous sequela of Crohn's disease (CD) is 'metastatic' CD, defined as sterile granulomatous skin lesions arising at sites discontinuous from the gastrointestinal tract. Though various histopathologic patterns have been described, a lack of a large series has precluded a comprehensive characterization and distinction from the pathologic differential diagnoses. The histopathology features of 12 new cases of metastatic CD were reviewed. Non-supperative granulomata with a slight cuff of lymphocytes in a nodular or diffuse pattern with an associated superficial and deep perivascular mixed inflammatory infiltrate was the most common pattern. Other common features included an accompanying infiltrate which was often rich in eosinophils, and ulceration of the overlying epidermis. These features are emphasized as potentially useful in distinguishing this entity from its greatest mimicker, cutaneous sarcoidosis.     

Metastatic Crohn's disease mimicking genital pyoderma gangrenosum in an HIV patient

The differential diagnosis of ulcerative genital lesions in patients with high risk sexual habits can be a challenge even for dermatologists. We present the case of a 27-year-old HIV-positive male with a history of recalcitrant genital ulcers. Microbiology studies were negative. A skin biopsy and a sample from a perineal fistula showed granulomatous infiltrates. The patient was treated with prednisone, metronidazole and aminosalicylates, showing complete resolution of the lesions in a few weeks. The clinical picture and histological findings are consistent with the diagnosis of cutaneous metastatic Crohn's disease. Although infrequent, metastatic Crohn's disease should be suspected in cases of recalcitrant ulcerative conditions, even in the absence of intestinal disease.     

手背皮肤转移性Crohn病一例

患者男，62岁，右手背红肿1年余。1年前，患者发现右手手背起红肿结节并逐渐增多。既往患Crohn病近20年。体检：右手背可见3个红色肿胀性皮下结节，质实，压痛不明显，局部淋巴结未触及。红结节处组织病理显示：表皮无明显变化，真皮内可见结核样浸润性非干酪样肉芽肿改变。诊断为皮肤转移性Crohn病。患者目前仍继续我院消化内科沙利度胺+硫唑嘌呤+美沙拉嗪的治疗方案，且内脏及皮损均有改善，皮损处仅给予强效糖皮质激素软膏外用对症处理，目前患者仍在我科及消化内科长期随诊。     

Cutaneous reactions at sites of herpes zoster scars: an expanded spectrum

Several types of cutaneous lesions have previously been described at the sites of herpes zoster scars. We describe 16 patients with cutaneous lesions which had developed on herpes zoster scars. Biopsies were taken from these lesions, and a polymerase chain reaction assay was used to detect the viral genome in paraffin-embedded specimens. Histopathological findings enabled diagnosis of nonspecific granulomatous dermatitis in five patients, granulomatous vasculitis in two patients, lichen sclerosus in two patients, and pseudolymphoma, keloid, sarcoidal granuloma, granuloma annulare, granulomatous folliculitis, lichen planus and cutaneous Rosai–Dorfman disease, each in one patient. Varicella-zoster virus DNA was not identified in any of the patients. Granulomatous folliculitis, lichen sclerosus and cutaneous Rosai–Dorfman disease have not previously been described in herpes zoster scars, but they are three new cutaneous reaction patterns that may have developed within these scars. Our investigations indicate that the cutaneous reactions appearing in herpes zoster scars are not due to the persistence of varicella-zoster virus DNA within the lesions.