Prolidase Deficiency Causing Recalcitrant Leg Ulcerations in Siblings.

ABSTRACT: Prolidase deficiency (PD) is a rare autosomal recessive genodermatosis with variable clinical manifestations. It results from a mutation in the peptidase-D gene that leads to abnormal activity of the prolidase enzyme, an important player in collagen catabolism. The authors report the case of two siblings presenting with dysmorphic features, disturbed blood panel, and recalcitrant leg ulcerations of several years' duration. Sequencing of the 15 exons and of the intron/exon junction regions of the peptidase-D gene revealed the presence of a homozygous pathogenic variant c.549-1G > A. An ointment with 5% proline and 5% glycine was compounded, and the patients were instructed to apply it once daily. A follow-up visit after 8 months revealed partial improvement of the ulcerations starting from the third month of treatment. These authors hope this case report sheds light on this disease and recommend it be incorporated into the differential diagnoses of chronic leg ulcerations, particularly those starting at a young age.

Guidelines for the Topical Treatment of Psoriasis Vulgaris in the Levant and Iraq Area.

Psoriasis vulgaris is a common chronic, inflammatory, multisystem disorder that affects approximately 1.5% to 3.4% of the population in the Middle East. The disease has an impact on the quality of life in a significant number of affected patients. The majority of patients (approximately 70%) have mild to moderate psoriasis that is manageable with topical agents, which generally show a high efficacy to safety ratio. Topical agents can be used alone when treating patients with limited disease or may be used as adjunctive therapy for patients with more extensive psoriasis undergoing systemic treatment. Treatment should also be customized to meet individual patients' needs. To optimize the topical treatment of psoriasis in the Levant and Iraq area, dermatology experts from Iraq, Jordan, Lebanon, Palestine, and Syria met and initiated a project to develop guidelines and recommendations for the topical management of psoriasis. The guidelines are based on literature evidence and experts' opinions. We present recommendations for the use of topical corticosteroids, vitamin D analogues, calcineurin inhibitors, tazarotene, salicylic acid, anthralin, and coal tar, as well as combination therapy, based on their efficacy and safety profiles.

Linear Morphea in the Context of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Infection: A Unique Dermatological Manifestation.

Morphea, a rare skin disorder characterized by localized areas of thickened and sclerotic skin, typically presents as circumscribed plaques. The linear variant, however, manifests as linear bands of sclerosis affecting the extremities, and its association with coronavirus disease 2019 (COVID-19) has not been documented until now. In this article, we present the case of a 22-year-old previously healthy female patient who contracted COVID-19 complicated by an erythroedema on the back of the right hand, extending notably to the forearm on the 10th day of the infection. Skin biopsy revealed dermal and septal hypodermal fibrosis with a mild lymphocytic interstitial infiltrate in the dermis consistent with morphea. Treatment with low-dose corticosteroids was started, and regular follow-up was established. An isolated recurrence of cutaneous symptoms was observed after the first COVID-19 vaccination (Sputnik V) administered five months after the initial infection, with spontaneous regression in 10 days. This clinical evolution underscores the importance of a comprehensive understanding of dermatological manifestations in COVID-19, particularly in the context of post-infection vaccination.

Childhood sarcoidosis: diagnostic issues.

Childhood sarcoidosis is a rare granulomatous disorder that affects several systems including the lungs, skin, eyes, and bones. On histopathology, non-caseating granuloma is the classic feature. We present a case of late childhood sarcoidosis of the skin, lungs, and bones demonstrating granulomas with fibrinoid necrosis, making distinction from tuberculosis difficult.

Dermatological manifestations associated with COVID-19 and COVID-19 vaccination: results from the Lebanese COVID registry.

BACKGROUND: A number of cutaneous manifestations and adverse cutaneous reactions have been associated with COVID-19 infection and vaccine. OBJECTIVES: A Lebanese national registry was established to characterize the dermatologic manifestations and adverse cutaneous reactions associated with COVID-19 infection and vaccination in a sample of the Lebanese population. METHODS: An observational cross-sectional study was conducted via a web-based clinical form distributed to physicians wishing to report their cases from May 2021 till May 2022. RESULTS: In total, 142 patients were entered in the registry, of which 133 were adults and nine were pediatric patients. The main dermatological manifestations reported with COVID-19 infection in the adult group were urticaria (32.9%), telogen effluvium (21.4%), morbilliform (10%), and papulosquamous (8.6%) eruptions. Urticaria was the most common adverse cutaneous reaction to the vaccine (33%). Interestingly, herpes zoster was triggered in 12 patients post vaccination in our series with this finding more frequently seen in patients above the age of 41 (P = 0.013). In the pediatric group, the most reported dermatological findings associated with COVID-19 infection were malar erythema (25%) and telogen effluvium (25%). One 16-year-old patient developed lichen planus after one dose of a COVID-19 vaccine. No deaths were reported in both age groups. CONCLUSION: This Lebanese registry adds more robust evidence that clinical manifestations of the COVID-19 virus and vaccine are diverse. More studies are necessary to establish the pathophysiology of these dermatological findings in the context of COVID-19 infection and vaccination.

Psoriasis and Myasthenia Gravis: A Common Th-17 Pathway.

Psoriasis is a chronic inflammatory skin disease whose treatment arsenal is expanding by the day. However, when comorbidities coexist, therapy can be challenging. We report a case of a 55-year-old female with steroid-dependent myasthenia gravis who presented with a severe form of chronic plaque psoriasis. After the failure of topical corticosteroids and phototherapy, the patient was started on ixekizumab. This anti-IL-17 antibody led not only to the clearance of the psoriatic lesions but also to the remission of the myasthenic symptoms. While on this medication, the patient was able to taper down and discontinue the oral corticosteroids. The remission of the symptoms of myasthenia gravis during this treatment supports the role of IL-17 cytokines in the pathogenesis of this disease and adds it as a management option in steroid-dependent cases.

Adalimumab-Induced Psoriasis with Severe Alopecia.

A 52-year-old woman with a 10-pack-year smoking history presented with serious hair loss. She had a 10-year history of Crohn's disease associated with arthritis, for which she had been taking adalimumab. After 2 months of this treatment, she developed pustular lesions on her palms, soles, and scalp. Her gastroenterologist discontinued adalimumab injections, ordered skin biopsy, and switched her to ustekinumab 90-mg subcutaneous injections every 8 weeks. She then presented to our dermatology clinic, 2 months following the initiation of anti-IL12/23 medications, for her alopecia. She had no personal or family history of psoriasis. (SKINmed. 2022;20:136-138).

A Recurrent Cheek Mass.

Solitary infantile myofibroma is a benign fibrous tumor occurring in early childhood. Although rare, it is the most common benign fibrous tumor of infancy. The clinical course of the disease is almost uniformly good since most tumors regress spontaneously. When indicated, conservative surgical excision is the treatment of choice, with a low recurrence rate. We present a case of solitary infantile myofibroma that recurred after three attempts of surgical excision, questioning the reported recurrence rate and the standard of care in recurrent solitary infantile myofibroma.

Purpuric lesions induced by UVA1 spectrum (340-400 nm) phototesting in an adult with persistent and severe hydroa vacciniforme.

A 28-year-old man had presented a severe photosensitivity since his infancy. In March 2008, the clinical examination showed large crusts on the dorsum of his hands, on the edge of his ears with destruction of the underlying cartilage, and on his nose and cheeks. He also presented erythematosus fibrous scars on the temples. The diagnosis of hydroa vaccinforme was made. Phototesting including repeated UVA1 phototest was strongly positive with purpuric lesions from day 7 to day 10 and hypertrophic scars at day 67. A sequential histological study of the UVA1 triggered lesions was performed and showed bullous cleavage, dense inflammatory infiltrate in the whole dermis with numerous neutrophilic cells, nuclear dusts, superficial focal thrombosis of small blood vessels at day 10. We report an unusual case of hydroa vaccinforme with purpuric lesions leading to fibrous scars and with important infiltration of neutrophils in the dermis of the photoinduced lesions.

Bullous presentation of Henoch-Schönlein purpura in an adult.

Henoch-Schönlein purpura is a small vessel vasculitis occurring mainly in childhood and rarely in adulthood. Typical cutaneous eruption may begin as macular or urticarial erythematous lesions progressing to a palpable purpura. In adults, the disease has a propensity to be more severe and chronic and affects mainly the ankles and feet. Bullae, vesicles and ulcers are occasionally seen in this group. The prognosis depends on renal involvement, commonly seen in adults. We report a new case of Henoch-Schönlein purpura in a 36-year-old man presenting with a bullous eruption followed by the appearance of abdominal pain and hematuria.

Treatment of mammary and extramammary Paget's skin disease with topical imiquimod.

BACKGROUND: Paget's disease is an uncommon epidermal cancer, affecting all skin regions wherever apocrine glands are present. It is divided into extramammary (EMPD) and mammary Paget's disease (MPD). It can be confined to the epithelium or may be associated with an underlying adenocarcinoma. The diagnosis is confirmed by skin biopsy and the treatment depends on characteristics of the underlying cancer. Surgery is the first-line treatment. Imiquimod, a topical immunomodulator, approved its efficiency in several skin neoplasms and has been shown as a safe treatment for EMPD. However, it has never been reported for the treatment of MPD. OBSERVATIONS: We report on two cases of EMPD and MPD successfully treated with imiquimod 5% cream. CONCLUSION: This non-surgical method may be considered as a primary treatment when Paget's disease is not associated with an underlying malignancy. The good prognosis with a long-term survival, the anatomic localization and the poor general condition of elderly people may favor imiquimod as an alternative treatment. On the other hand, it will reduce the extent of excision when it anticipates surgery.

Subcutaneous nodular sarcoidosis and systemic involvement successfully treated with doxycycline.

Subcutaneous nodular sarcoidosis is a rare cutaneous manifestation of systemic sarcoidosis. We report a new case in a 45-year-old woman with a 7-year history of subcutaneous nodules and a new onset of dyspnea. She was treated with corticosteroids but her disease recurred upon withdrawal. A 6-month course of doxycycline in a dose of 200 mg/d led to complete remission. We also emphasize the value of systemic work-up and regular screening in such cases.

[The syndrome of bazex or acrokeratosis paraneoplastica]. / Le syndrome de bazex ou acrokératose paranéoplasique. Revue de la littérature.

Bazex syndrome or acrokeratosis paraneoplastica is a rare dermatosis usually associated with a squamous cell carcinoma of upper aerodigestive tract or with metastases in the cervical lymph nodes. It presents as psoriasiform violaceous scaly patches over extremities, tip of the nose, earlobes and helices. This syndrome evolves in three stages parallel to the growth of the tumor, thus the utility of recognition of the syndrome which may unmask the associated neoplasia at an early stage. Treatment consists of treating the underlying neoplasia whether by surgery, chemotherapy and/or radiotherapy. Recurrence of cutaneous lesions may reveal the recurrence of the tumor.

Onychomycosis in Lebanon: a mycological survey of 772 patients.

Accurate diagnosis of onychomycosis is based on clinical findings, direct microscopic investigation and mycological culture. If the diagnosis is not confirmed by culture and improvement does not occur, it is impossible to tell whether this represents treatment failure or an initial incorrect diagnosis. The aim of this study was to identify the major organisms involved in onychomycosis with emphasis on the importance of culture in treating onychomycosis. The study was performed at the Lebanese University, Beirut, Lebanon over a 5-year period (2000-2004). Clinically suspected patients were referred to our mycology laboratory for KOH test and culture. The study included 772 patients (520 women, 252 men). Cultures were positive in 54.3% of cases (predominantly male). The ratio of onychomycosis in toenails/fingernails was 1.9. In toenails, dermatophytes were found in 77.1% of cases, Candida in 18.9% and moulds in 4%. In fingernails, Candida was found in 81% of cases, dermatophytes in 18.1% and moulds in 0.9%. The most commonly isolated dermatophytes were Trichophyton mentagrophytes (36%), T. rubrum (27.5%) and T. tonsurans (26%). Pathogens involved in onychomycosis change according to each geographical area. Therefore, treatments should be based on studies carried out in the same region.

Angiolymphoid hyperplasia with eosinophilia: efficacy of isotretinoin?

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign but potentially disfiguring vascular lesion. It is usually characterized by dermal and subcutaneous nodules, primarily in the head and neck region. Spontaneous regression is common, but persistent or recurrent lesions may require treatment. Several treatments have been reported but surgery is the most efficient one. METHODS AND RESULTS: We report a 32-year-old man presenting with multiple nodules on the cheeks, preauricular region and the scalp and who received treatment with isotretinoin (0.5 mg/kg/day) for 1 year with complete resolution of one of his scalp nodules. The rest of the lesions remained stable and were treated with surgical excision without recurrence. CONCLUSION: Isotretinoin may play a role in the treatment of ALHE due to its antiangiogenic properties via a reduction of vascular endothelial growth factor (VEGF) production by keratinocytes.

Photoprotection in teenagers.

BACKGROUND: It is very common to apply a 'template photoprotection' without taking into consideration the background of a specific population. In Lebanon, so far, no preliminary survey has been conducted before launching an educational photoprotection campaign, revealing the need of the people according to their background. MATERIALS AND METHODS: In April 2004, a specific questionnaire was administered to 940 adolescents, aged from 14 to 18 years. The questionnaire comprised 18 multiple-choice questions that established the approximate phototype of teenagers, awareness of ultraviolet (UV) hazard, presence of sufficient information regarding the risk of sun exposure (school, doctor, media) and the use of measures of photoprotection (clothes, sunscreens). RESULTS: The incidence of sunburn in teenagers was high (85.42%) despite their awareness of the risks of unprotected sun exposure. The information regarding sun damage seems to be insufficiently delivered at school and by doctors. The main source of information was television. It was obvious that our adolescents underestimated the value of clothing to protect themselves. The use of clothing comes at the second place after sunscreens. However, the application of sunscreen seems to be inadequate. CONCLUSIONS: While solar protection has become part of routine beach behavior for adolescents, there is room for improvement with better application of sunscreen and more use of clothing, especially wearing hats, and seeking shade. However, it is difficult to appreciate the impact of publicity campaigns on the risks of sun exposure in reducing the incidence of melanoma.