Repeated Paradoxical Aggravation of Preexisting Psoriasis during Infliximab Treatment for Crohn's Disease

As more rheumatologists and dermatologists have begun to use biological agents such as TNF-α blocker, they have confronted an unexpected complication: psoriasis was paradoxically aggravated or induced by the TNF-α blocker. Although it is not a common complication of TNF-α blocker, this aggravation may be more common than previously thought. To our knowledge, most reports about TNF-α blocker-induced psoriasis have been limited to western countries while only a few cases have been reported in Korea and Japan. In addition, new onset of pustular psoriasis by TNF-α blocker has been reported more commonly than worsening of preexisting psoriasis. Now we report a patient whose preexisting psoriasis vulgaris was aggravated repeatedly after using the TNF-α blocker, infliximab, to control Crohn''s disease, which is a rare rheumatologic disease in Korea.     

Orofacial granulomatosis and erythema multiforme in an adolescent with Crohn's disease

The cutaneous manifestations of Crohn's disease are myriad. A 15‐year‐old girl presented with recurrent lip swelling and eventual development of diarrhea and targetoid macules on the palms, feet, and back. She was finally diagnosed with Crohn's disease in the setting of a clinical presentation and histopathology consistent with orofacial granulomatosis and erythema multiforme. We review the literature and summarize reported occurrences of these cutaneous diseases in children with Crohn's disease.     

Apocrine Carcinoma of the Groin Possibly Associated with Extramammary Paget's Disease

Apocrine carcinoma is a rare malignancy with invasive potential. It presents as painless, slow-growing, firm or cystic, red nodules with focal ulcerations. The tumor is capable of hematogenous dissemination to the liver, lungs, and bone as well as lymphatic spread. In addition, apocrine carcinomas cause intra-epidemial pagetoid spread. We report a case of an apocrine carcinoma related with extensive extramammary Paget''s disease (EMPD). The relationship between apocrine carcinoma and EMPD remains to be understood. Co-existing cases with apocrine carcinoma and EMPD are discussed to better understand the relationship between these two malignant apocrine tumors.     

Early oral presentation of Crohn's disease

Inflammatory changes of the oral mucosa in children can have a variety of infectious and non‐infectious etiologies. We report on a 10‐year‐old boy with progressive cobblestone‐like changes and erosions of oral mucosa over six months, which turned out to be early oral manifestations of Crohn disease.     

The potential role of serology in diagnosing chronic lymphogranuloma venereum (LGV): a case of LGV mimicking Crohn's disease

We present the case of a 26 year old HIV positive homosexual man who was managed for suspected Crohn's disease for over 1 year before lymphogranuloma venereum (LGV) was clinically diagnosed. He had presented with constipation, secondary to acute haemorrhagic proctitis, and subsequently had two chlamydia negative rectal smears, using direct fluorescent antibody (DFA) Chlamydia trachomatis staining. Positive chlamydial serology guided retrospective testing of an early rectal biopsy, which was found to have C trachomatis by polymerase chain reaction (Roche Cobas) and identified as LGV serovar L2 by the Sexually Transmitted Bacteria Reference Laboratory (STBRL), Health Protection Agency (HPA), Colindale, London. Chlamydial serology may have a role in identifying late stage LGV infection. Although no standardised test currently exists, consideration should be given to evaluating the role of chlamydial serology in establishing a diagnosis of LGV.     

Synchronous Orofacial Granulomatosis in a Patient with Brain Cavernous Hemangioma

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.     

Extramammary Paget's Disease of External Genitalia with Bowenoid Features

Extramammary Paget''s disease (EMPD) is an uncommon intraepithelial adenocarcinoma, primarily affecting the apocrine-bearing skin. Bowen disease is an intraepithelial squamous cell carcinoma having the potential to become invasive carcinoma. The histopathological concomitant features between EMPD and Bowen disease have been described. One theory is that primary EMPD arises multicentrically, within the epidermis from the pluripotent stem cells. Herein, we describe a case of EMPD that had bowenoid features, and review the previous cases associated with the origin of EMPD.     

Cutaneous Crohn's disease with superimposed psoriasis: A unique case with overlapping histology

Crohn's disease (CD) is an idiopathic, chronic inflammatory disorder of the gastrointestinal tract. We recently encountered a unique case in which a patient with longstanding CD presented with skin lesions with histopathologic features of both psoriasis and granulomatous inflammation suggestive of cutaneous CD. To our knowledge, this has not been described concomitantly in the same patient, in the same lesions. Review of the literature suggests that the intersection of these 2 histopathological reaction patterns may not be pure coincidence. Clinical‐pathologic correlation of this case will be discussed, along with a review of the potential mechanisms of this unique disease presentation.     

Cutaneous Crohn's disease treated with infliximab and 4 years of follow up

Metastatic Crohn's disease (MCD) is a rare, non‐contiguous cutaneous manifestation of Crohn's disease. To date, there have been only four reports in the literature of an effective treatment of this condition with infliximab and there are no long‐term follow‐up studies on adult MCD patients treated with infliximab. We present a case of MCD treated with infliximab with 4.5 years of follow up.     

Linear IgA Dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy

Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The patient experienced unsatisfactory partial control of skin and intestinal symptoms despite the use of adalimumab, mesalazine, prednisone and dapsone for some months. After total colectomy, he presented complete remission of skin lesions, with no need of medications during two years of follow-up. A review of previously reported cases of the association is provided here and the role of ulcerative colitis in triggering linear IgA dermatosis is discussed.     

A Case of Vitiligo after Kawasaki's Disease

Vitiligo is a common skin disease, but its pathogenesis has not been fully determined, though an autoimmune etiology is considered likely. Kawasaki disease (KD) is an acute multisystem vasculitis of childhood associated with coronary arteriopathy, and is diagnosed based on clinical criteria. Furthermore, vitiligo has been associated with several other diseases, but no report has been issued about the relationship between vitiligo and Kawasaki''s disease. The author''s report the case of an 8-year-old male child that presented with depigmented lesions, which developed from the desquamative skin lesions of Kawasaki''s disease.     

Pediatric patients with orofacial granulomatosis likely to subsequently develop intestinal Crohn's disease: Brief Report

Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition presenting as perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn's disease. Our retrospective review identified 7 patients diagnosed with OFG between 2000 and 2018 at a tertiary pediatric hospital. Four of the 7 patients subsequently developed Crohn's disease with a median delay of 3.1 years (range 0.4-6.9 years). This indicates that gastroenterology evaluation with long-term monitoring for intestinal Crohn's disease is warranted.     

A Case of Sweet's Panniculitis Associated with Spinal Metastasis from Prostate Cancer

Sweet''s panniculitis is a rare variant of Sweet''s syndrome in which neutrophilic infiltrate can be found either in the subcutaneous fat or in both the dermis and the subcutaneous tissue. Due to the rarity of this entity, the association between Sweet''s panniculitis and malignancies is inconclusive, but cases of Sweet''s panniculitis have largely been associated with hematological malignancies. Herein, we present a case of Sweet''s panniculitis accompanied by bone metastasis from prostate cancer. Clinicians should be aware that Sweet''s panniculitis may be associated with malignancies of solid organs.     

A Case of Kikuchi's Disease Presenting with Unique Facial Involvement

Kikuchi''s disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. When KD is involved in a skin lesion, it presents with various shapes, but rarely manifests with symmetrically distributed, erythematous, firm nodules only on the face. In this report, we describe a patient with KD and unique skin manifestations.     

Photodynamic Therapy for Bowen's Disease of the Vulva Area

Bowen''s disease is a squamous cell carcinoma in situ and has the potential to progress to a squamous cell carcinoma. The authors treated two female patients (a 39-year-old and a 41-year-old) with Bowen''s disease in the vulva area using topical photodynamic therapy (PDT), involving the use of 5-aminolaevulinic acid and a light-emitting diode device. The light was administered at an intensity of 80 mW/cm² for a dose of 120 J/cm² biweekly for 6 cycles. The 39-year-old patient showed excellent clinical improvement, but the other patient achieved only a partial response. Even though one patient underwent a total excision 1 year later due to recurrence, both patients were satisfied with the cosmetic outcomes of this therapy and the partial improvement over time. The common side effect of PDT was a stinging sensation. PDT provides a relatively effective and useful alternative treatment for Bowen''s disease in the vulva area.     

Photodynamic Therapy in Bowen Disease of the First Web Space of the Hand

Bowen disease (BD), or intraepithelial squamous cell carcinoma (SCC), may progress to an invasive SCC. Although surgery is preferred because of the low recurrence rate, it can result in hypertrophic scarringor contracture, particularly in lesions on the hands. We report a case of BD in the first web space of the hand, which was treated with ablative fractional laser-assisted photodynamic therapy (AFXL-assisted PDT). After multiple AFXL-assisted PDT sessions, the lesion showed no clinical or pathological abnormalities. Thus, we believe that PDT can be an alternative treatment for BD occurring in the web space of the hand.     

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Elastosis perforans serpiginosa (EPS) is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers. Penicillamine, which is one of the clear triggers for EPS, is a heavy metal chelator that is primarily used for disorders such as cystinuria and Wilson''s disease. It may cause alterations in the dermal elastic tissue such as pseudo-pseudoxanthoma elasticum, acquired cutis laxa, EPS and anetoderma. Herein we present a case of cutis laxa and EPS in a 34-year-old man who was previously on a long-term, high-dose of penicillamine for Wilson''s disease. The combination of EPS and cutis laxa induced by penicillamine has rarely been reported and we report the first such case in Korea.     

An Unusual Clinical Presentation of Kimura's Disease Occurring on the Buttock of a Five-year-old Boy

Kimura''s disease is a rare inflammatory disorder of unknown etiology primarily seen in young Asian males. It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E (IgE) levels. Clinically, the subcutaneous nodules occur predominantly in the head and neck. However, we report the case of a five-year-old boy presenting with an asymptomatic solitary brown pigmented nodule on the left buttock diagnosed with Kimura''s disease. Here we describe the diagnosis and treatment of this unusual case.