Case Reports in Dermatology最新文献

Introduction: Bart's syndrome is an uncommon inherited congenital disorder associating congenital cutaneous aplasia of the extremities and inherited epidermolysis bullosa. Bilateral and symmetrical involvement of the limbs is exceptionally described on black skin. In most cases, the diagnosis is clinical; however, the management remains very difficult and the extended forms are a real therapeutic challenge. We report 2 cases of Bart's syndrome observed in a sub-Saharan African country (Senegal, Dakar).

Case presentation: It was about 2 premature female and male newborns. On physical examination, the girl presented with a total absence of skin on the limbs, associated with cutaneous detachment of the trunk representing a detached and detachable skin surface of 46%; the boy underwent a total absence of skin of more than 50% of the skin surface. The diagnosis of Bart's syndrome was set based on the typical clinical aspect. The blood count and CRP were normal for the girl whereas it revealed some disorders for the boy. The 2 newborns were urgently admitted to an incubator, and the intensive care was started with hyperhydration, anti-staphylococcal prophylaxis, and daily dermatological care with antiseptic baths and fatty dressings.

Conclusion: Bart's syndrome is an uncommon genodermatosis characterized by a clinical triad associating congenital cutaneous aplasia of the extremities, inherited epidermolysis bullosa suspected in the presence of bubbles, and areas of cutaneous fragility and nail deformity. All types of which can be associated with this syndrome. The easy clinical diagnosis but the difficult management encumber the vital prognosis of our cases.

Green nail syndrome (GNS) is a persistent greenish pigmentation of the nail plate, originally described in 1944 by Goldman and Fox, due to Pseudomonas aeruginosa infection. Recently, pulmonary co-infection of P. aeruginosa and Achromobacter spp. has been described in patients with cystic fibrosis. Achromobacter xylosoxidans is a multidrug-resistant (MDR) pathogen involved in lung and soft tissue skin infections. Both Achromobacter xylosoxidans and P. aeruginosa are mainly found in humid environments or in water. There are no recognized co-infections due to P. aeruginosa and A. xylosoxidans in the skin and appendages. We describe two cases of GNS, the first due to P. aeruginosa associated with Achromobacter xylosoxidans; the other due to MDR P. aeruginosa, both successfully treated with topical ozenoxacin 1% cream daily for 12 weeks. The clinical management of GNS can be confusing, especially when the bacterial culture result is inconsistent or when non-Pseudomonas bacteria are isolated. In our case, due to the co-infection of P. aeruginosa and Achromobacter spp., local treatment with ozenoxacin - the first nonfluorinated quinolone - could be a safe and effective treatment in case of MDR nail infections. Further studies are required to evaluate clinical isolation from nail infections and the co-presence of P. aeruginosa and A. xylosoxidans.

Poroid hidradenoma (PH) is a rare benign adnexal tumor of eccrine differentiation. It is the rarest of the four described variants of poroid neoplasms. PHs characteristically share a hybrid of the architectural features of the hidradenoma, namely, tumor cells are entirely intradermal with both solid and cystic components, and the cytologic characteristics of the poroid neoplasms, containing predominantly poroid and cuticular cells. Many published reports of PH since its original discovery in 1990 state that "very few" cases of PH can be found in the literature. Here, we have identified a total of 75 published accounts of PH, including the case presented here, as well as the associated patient demographics, lesion characteristics, treatment, and outcomes. We suggest that while uncommon, PH is likely not exceptionally rare and could be an underreported diagnosis.

Neurofibromatosis type 1 is a genetic disorder impacting approximately 2.5 million people worldwide, often leading to development of numerous benign yet disfiguring cutaneous neurofibromas (cNF). Removal of cNF is limited to excision or laser ablation with common post-operation complications and scarring. The current case explores a new approach to removal or reduction of cNF by a minimally invasive and pain-reduced treatment modality. A 40-year-old female patient with numerous cNF across her body underwent a single treatment using a 20 MHz dermatologically focused ultrasound device on seven selected cNF on the upper back. Each cNF was treated in a single session of 20-60 s without anesthesia due to manageable pain. Only one minimal adverse reaction in the form of dyspigmentation in a single treated tumor was noted from treatment or during the healing of a thin scab that formed on each cNF a few days after treatment. At the 12-month follow-up, four out of seven treated cNF showed full remission, two showed partial or significant reduction in tumor volume, while two did not respond to treatment. The reason for the variability is not fully understood, but speculations include difference in tissue content, e.g., due to tumor age. The method is concluded to be a promising candidate for a new safe and minimally invasive treatment that can potentially be used for single-session removal/reduction of a large number of cNF. Further research should focus on refining treatment parameters and strategies to enhance response predictability.

Fungal infections can be challenging to diagnose, but doctors of every specialty may encounter this issue. They can be mistaken for other common dermatoses such as eczema or psoriasis and inadvertently be treated with topical corticosteroids or calcineurin inhibitors. This may lead to tinea incognita, a term used to describe a fungal infection with an altered clinical appearance, which may confuse the clinician even further. This case report presents a 54-year-old previously healthy man with a 4-month history of a painful and pruritic rash in the genitoinguinal region. The patient's general practitioner had unsuccessfully attempted to treat the rash with topical terbinafine, econazole-triamcinolone, and betamethasone-fusidic acid, in addition to peroral dicloxacillin capsules. On examination, there were multiple red-bluish nodules and pustules coalescing into infiltrating erythematous plaques on both thighs and in the pubic region. Fungal cultures were negative, but the clinical features together with the history of prolonged use of combined topical steroids and antifungals raised suspicion of a deep fungal infection. Histopathological skin examination revealed deep suppurative and granulomatous folliculitis with ruptured hair follicles which was consistent with a diagnosis of Majocchi's granuloma. Treatment with itraconazole capsules was initiated, and after a 16-week course of systemic antifungal therapy, the rash resolved. In conclusion, our case report presents a case of Majocchi's granuloma, which is a great mimicker, especially for non-dermatologists. It is therefore important that the diagnosis is considered as a differential diagnosis, even though a patient has previously been treated with a topical antifungal.

This case report discusses immunoglobulin-induced erythema multiforme (EM), a rare and understudied condition that requires further investigation. The report presents the case of a 69-year-old woman with a history of chronic lymphocytic leukemia who developed an acute hypersensitivity reaction to intravenous gamma globulin medication. The patient received intravenous immunoglobulin (IVIG) to normalize and stabilize her immunoglobulin levels and reduce the risk of recurrent infections due to her immunodeficiency with predominantly antibody defects. However, after the second administration of the medication, the patient experienced an acute skin rash and was admitted to the hospital for treatment. The treatment plan included systemic desensitizing therapy, systemic antihistamine therapy, corticosteroid therapy, and local therapy. After a course of therapy, the patient's skin condition improved, and her overall well-being improved. However, due to the acute hypersensitivity reaction, the IVIG therapy was discontinued. The multidisciplinary team of specialists concluded that the patient had developed EM. The discussion provides an overview of EM, including its causes, clinical presentation, diagnostic tools, and therapy principles. The discussion also describes the use of human IVIG preparations in treating various immunodeficient and inflammatory diseases, highlighting the importance of monitoring patients for adverse effects. The case of this patient underscores the potential risks associated with immunoglobulin therapy and emphasizes the need for healthcare providers to remain vigilant for adverse reactions. By promptly diagnosing and treating EM, healthcare providers can minimize its impact on patients' overall well-being.

Spinal dysraphism (SD) refers to the abnormal fusion of dorsal midline structures during embryogenesis. It encompasses a variety of congenital spinal defects, ranging from an overt defect in which neural tissue is exposed with no overlying skin (open SD) such as myelomeningoceles to skin-covered malformations (closed or occult SD). A 13-year-old boy presented with recurrent multiple painless ulcers and erosions over the tips of the toes, mainly involving the right foot with hemorrhagic crusts for 5 years. A review of systems revealed back pain, urine incontinence, and numbness in his right knee. He was diagnosed with peripheral neuropathic ulcers and tethered cord syndrome secondary to SD and confirmed by MRI. He underwent cord detethering and lipoma resection as well as expectant therapy with satisfying outcomes. Physicians should consider early diagnosis of SD to avoid later neurological complications of SD (traction and/or pressure on the spinal cord) when infants are presented with such anomalies: MRI, close follow-up, and neurosurgical intervention may be recommended.

Primary cutaneous marginal zone lymphoma (PCMZL) is a rare form of B-cell lymphoma that primarily affects the skin. Chronic antigen stimulation has been implicated in its development, with cases associated with various triggers. We present a case of PCMZL following chronic inflammation caused by long-term hair dyeing. A 75-year-old woman with a history of repeated inflammation and itching after hair dyeing for 30 years presented with persistent red-to-violaceous patches and plaques on her scalp. Despite receiving topical corticosteroid treatment for 10 years, the lesions remained. Pathological examinations confirmed the diagnosis of PCMZL. The patient achieved complete remission after radiotherapy. This case underscores the potential link between chronic inflammation and the development of PCMZL.

Porokeratosis is a group of well-known clinically distinct entities, characterised by different clinical aspects, but sharing a single common histological aspect, namely the cornoid lamella. Usually, porokeratosis occurs in the limbs and trunk, while it rarely involves the face, especially as an exclusive, single, and solitary lesion. We report the case of a 52-year-old Caucasian woman, with an 11-month history of a 2-cm slowly growing solitary, keratotic lesion on her left cheekbone. The patient did not present other cutaneous lesions on the face, as well as in other body sites. A cutaneous biopsy showed epidermal hyperplasia with multiple, sharply defined cornoid lamella, associated with an underlying attenuation of the granular layer and scattered dyskeratotic cells in the spinous layer. The superficial dermis underneath showed a mild lymphocytic infiltrate and fibrosis with remodelled collagen bundles. A final diagnosis of solitary facial porokeratosis was made.

Lichen planus is a chronic inflammatory disorder that may affect the skin, nails, and/or oral mucosa. Bullous lichen planus is a rare variant of lichen planus, which is even less common in the nails. We present a case of nail bullous lichen planus, in a 48-year-old male presenting with a 10-month history of onychodystrophy of all ten fingernails. A longitudinal excision of the left thumbnail was performed, with histopathology consistent with lichen planus with focal transition to bullous lichen planus. He was treated with intralesional triamcinolone injections to the fingernails monthly, with improvements noted after three treatments. Our patient's nail bullous lichen planus manifested with longitudinal ridging, white-yellow discoloration, onycholysis, subungual hyperkeratosis, and v-shaped nicking. Histopathological findings included classical lichen planus changes, as well as formation of subepidermal bullae, colloid bodies, and extensive inflammatory infiltrate. Increased awareness and high index of suspicion for this condition are necessary, given the often late diagnosis reported in previously published cases.