Recalcitrant Paraneoplastic Pemphigus Associated With Prostate Adenocarcinoma: Spectacular Evolution Under Androgen Deprivation.

Paraneoplastic pemphigus (PNP) is a rare, autoimmune, blistering condition defined by severe stomatitis, polymorphous cutaneous eruptions, and underlying neoplasms. PNP associated with solid cancer is extremely rare. An association with prostate adenocarcinoma remains exceptional. We describe a 69-year-old patient with recalcitrant PNP associated with prostate adenocarcinoma showing spectacular response immediately after associating hormonotherapy with conventional immunosuppressive drugs.

Predictors of poor response to oral propranolol in infantile hemangiomas.

BACKGROUND: Propranolol is the first-line treatment for infantile hemangiomas (IH). Cases of propranolol-resistant infantile hemangiomas are rarely reported. The purpose of our study was to investigate the predictive factors for poor response to propranolol. METHODS: A prospective analytical study was conducted between January 2014 and January 2022 including all patients with IH who received oral propranolol therapy at a dose of 2-3 mg/kg/day maintained for at least 6 months. RESULTS: A total of 135 patients with IH were treated with oral propranolol. Poor response was reported in 18 (13.4%) of the patients: 72% were girls and 28% were boys. Overall, 84% of the IH were mixed, and hemangiomas were multiple in three cases (16%), nasal tip hemangiomas accounted for four cases (22%), and 15 patients (83%) had segmental hemangiomas. There was no significant association between the age or sex of the children and type of response to treatment (p > 0.05). No significant association was found between the type of hemangioma and the therapeutic outcome as well as the recurrence after treatment discontinuation (p > 0.05). Multivariate logistic regression analysis revealed that nasal tip hemangiomas, multiple hemangiomas, and segmental hemangiomas were at greater risk of poor response to beta-blockers (p < 0.05). CONCLUSION: Poor response to propranolol therapy has rarely been reported in the literature. In our series, it was approximately 13.4%. To our knowledge, no previous publications have focused on the predictive factors of poor response to beta-blockers. However, the reported risk factors for recurrence are discontinuation of treatment before 12 months of age, mixed or deep type IH, and female gender. In our study, the predictive factors for poor response were multiple type IH, segmental type IH, and location on the nasal tip.

Atypical Cutaneous Viral Infections Reveal an Inborn Error of Immunity in 8 Patients.

Unusual viral skin infections might be the first clinical manifestation in children with an inborn error of immunity (IEI). We performed a prospective study from 1 October 2017 to 30 September 2021, at the Department of Pediatric Infectious Diseases and Clinical Immunity of Ibn Rochd University Hospital-Casablanca. During this period, on 591 patients newly diagnosed with a probable IEI, eight of them (1.3%), from six independent families, had isolated or syndromic unusual viral skin infections, which were either profuse, chronic or recurrent infections, and resistant to any treatment. The median age of disease onset was nine years old and all patients were born from a first-degree consanguineous marriage. By combining clinical, immunological and genetic investigations, we identified GATA2 deficiency in one patient with recalcitrant profuse verrucous lesions and monocytopenia (1/8) and STK4 deficiency in two families with HPV lesions, either flat or common warts, and lymphopenia (2/8), as previously reported. We also identified COPA deficiency in twin sisters with chronic profuse Molluscum contagiosum lesions, pulmonary diseases and microcytic hypochromic anemia (2/8). Finally, we also found one patient with chronic profuse MC lesions and hyper IgE syndrome, (1/8) and two patients with either recalcitrant profuse verrucous lesions or recurrent post-herpetic erythema multiforme and a combined immunodeficiency (2/8) with no genetic defect identified yet. Raising clinicians awareness that infectious skin diseases might be the consequence of an inborn error of immunity would allow for optimized diagnosis, prevention and treatment of patients and their families.

Mucocutaneous Manifestations in Kidney Transplant Patients: Risk Factors.

Introduction Mucocutaneous complications in kidney transplant patients are due to drug toxicity or immunosuppression. The main objective of our study was to determine the risk factors associated with their occurrence. Methods We conducted a prospective analytical study (January 2020- June 2021) including kidney transplant patients seen at the Nephrology Department. We described the characteristics of the patients who presented mucocutaneous complications and then compared them to those who didn't to deduce the risk factors. Statistical analysis was performed using SPSS 20.0 (p<0.05). Results Of the 86 patients recruited, thirty patients had mucocutaneous complications. The mean age was 42.73, with a male predominance (73%). Ten kidney transplants were performed from a living-related donor. All the patients received corticosteroids, Mycophenolate Mofetil, and the Calcineurin Inhibitor: Tacrolimus (76.7%) or Ciclosporin (23.3%). Induction was performed with Thymoglobulin (n=20) or Basiliximab (n=10). Mucocutaneous complications were dominated by infectious manifestations (53.4%): eight cases of fungal infections; six cases of viral infections: warts (n=3), herpes labialis (n=2), intercostal herpes zoster (n=1), and two cases of bacterial infections: atypical mycobacteria and boils. Inflammatory complications (36.6%) included acne (n=4), urticaria (n=3), rosacea (n=1), simple maculopapular exanthema (n=1), aphthous lesion (n=1), and black hairy tongue (n=1). Actinic keratosis, skin xerosis, and bruises were found in one patient respectively. The evolution with a symptomatic treatment was good in all the patients. After statistical analysis, the factors significantly associated with the occurrence of mucocutaneous complications were advanced age, male gender, anemia, HLA non-identical donor, as well as the use of Tacrolimus or Thymoglobulin. Conclusion Infectious mucocutaneous complications are the most common dermatological manifestations among renal transplant recipients. Their occurrence is related to advanced age, male gender, anemia, HLA non-identical donor, and the use of Tacrolimus or Thymoglobulin.

A Generalized Form of Lichen Planus Pemphigoid Induced by an Oral Antidiabetic.

Lichen planus pemphigoid (LPP) is a rare autoimmune bullous dermatosis, although it is frequently idiopathic, the induced form is rare and there are few inducing drugs. We report a case of LPP induced by a gliclazide. A 68-year-old female patient with type 2 diabetes on gliclazide for three months presented with an eight-week history of generalized erythematous-papular eruption. Then she developed blisters on her forearms with oral mucosa involvement. A diagnosis of gliclazide-induced LPP was made based on a skin biopsy and imputability. The patient was treated with systemic corticosteroid with an improvement. LPP is a rare entity; its diagnosis is a challenge as it represents an overlap between lichen planus and bullous pemphigoid.

A Lymphocytic Variant of Hypereosinophilic Syndrome Presenting With Isolated Cutaneous Manifestations.

Hypereosinophilic syndrome (HES) is a rare disease defined by a persistent increase in eosinophilic cells associated with organ damage without any underlying cause. Three variants have been identified: myeloproliferative, lymphocytic, and idiopathic syndrome. The symptomatology is variable because it depends on the involvement of different organs, including the circulatory system, skin, lungs, digestive tract, peripheral and central nervous system, and eyes. Although cutaneous involvement may frequently reveal an underlying HES. We report a case of a 52-year-old man with a 12-year history of skin lesions with intense pruritus. On examination, the patient presented with erythroderma, extensive infiltrated plaques, excoriated itchy papules, palmoplantar pustules, ear infiltration, periorbital edema, and cutaneous xerosis. Histopathology showed lichenoid dermatitis without epidermotropism. Inflammatory infiltrates in the dermis were principally composed of eosinophilic cells and lymphocytes. Serum immunoglobulin E and peripheral blood immunophenotyping showed atypical T lymphocyte proliferation CD4+CD3-, and clonal TCR gene rearrangement was in favor of lymphocytic HES. The patient was treated with prednisone (1 mg/kg/day) and pegylated interferon alpha with improvement. This case shows that HES should be suspected in patients with dermatological lesions and hypereosinophilia, without obvious cause. Elimination of secondary causes of eosinophilia, evaluation of deep organ involvement, and cytogenetic studies to assess prognosis are paramount. Pegylated interferon alpha 2a may be an effective treatment option for steroid-resistant or refractory patients with lymphocytic HES.

Verrucous Melanoma of the Scalp Initially Misdiagnosed as Seborrheic Keratosis.

Verrucous melanoma (VM) is a rare entity that presents diagnostic difficulty on both clinical and histopathologic grounds. Clinically, this tumor can be mistaken for a benign non-melanocytic lesion, particularly seborrheic keratosis (SK), as they both share several similarities, such as the homogenous pigmentation, the verrucous surface, and the roughly well-defined borders. In our patient's case, her verrucous lesion was initially misdiagnosed as SK by a general practitioner two months prior to her admission. Upon physical examination, the lesion was indeed suggestive of SK but a VM was not discarded. Biopsy revealed melanoma. Standard treatment of SK often includes electrodesiccation or cryotherapy, which potentially might worsen and delay the diagnosis of melanoma with subsequent implications for therapeutic management and prognosis. We report this case to increase awareness and knowledge about VM, which may lead to earlier diagnosis and improved outcomes.

The Proliferating and Malignant Proliferating Trichilemmal Cyst: An Anatomo-Clinical Study of Three Cases.

Background: The proliferating and malignant proliferating trichilemmal cysts (MPTC) are rare adnexal tumors. We report 3 cases through which we will detail the anatomo-clinical characteristics of these tumors. Cases: Two patients, 60 and 56 years old, consulted for multiple scalp nodules, one of which had changed with the appearance of a central ulceration. The removal of the remaining scalp nodules was in favor of PTCs. The third patient presented with an ulcerative lesion occupying the vertex. Skin biopsy found trichilemmal-type keratinization associated with areas of necrosis concluding with a MPTC. Discussion: The PTC is a transitional form between the trichilemmal cyst (TC) and the MPTC. The increase in the size of a TC and ulceration are sufficient signals to suspect this evolution.

Papulonodular Mucinosis Associated With Subacute Lupus.

Papulonodular mucinosis is a rare but well-documented finding associated with systemic and cutaneous forms of lupus erythematosus (LE). It occurs exceptionally in association with subacute cutaneous lupus erythematosus (SCLE). Its etiology and pathogenesis remain to be elucidated. Herein, we report a case of papulonodular mucinosis associated with SCLE in a middle-aged woman. On physical examination, she presented with multiple flesh-coloured asymptomatic papules and nodules on the trunk and upper extremities. A biopsy specimen taken from a nodule showed mucin within the dermis with hypodermis and perivascular lymphocytic inflammation. Considering that the proportion of patients with cutaneous lupus mucinosis who progress to systemic lupus is uncertain, we suggest following these patients closely for evidence of multisystem disease.

Superficial Acral Fibromyxoma: Clinicopathologic Analysis of Five Cases.

Superficial acral fibromyxoma is a relatively rare benign slow-growing soft-tissue tumor, first described by Fetsch's group [Hum Pathol. 2001;32:704-14]. Since then, around three hundred publications have concerned this relatively new entity. The tumor involves peri- and subungual regions of fingers and toes in middle-aged adults with slight male predominance. This acral fibrous tumor is poorly known, and in certain cases, histology results may suggest myxoid dermatofibrosarcoma, which carries a completely different prognosis. In this article, we discuss the clinicopathologic features of this acral fibrous tumor through the report of 5 cases including 1 particular clinical presentation that revealed as a retronychia in a young woman.

Subungual Exostosis: A Case Series of 48 Patients.

BACKGROUND: Subungual exostosis is an unusual benign nail tumor, mostly located on the big toe. It generally affects young people and manifests as uncomfort during footwear. METHOD: A monocentric retrospective study was conducted at the outpatient consultation for nail disorders at the Department of Dermatology of the University of Casablanca, Casablanca, Morocco, between April 2006 and October 2019. RESULTS: We diagnosed subungual exostosis in 48 patients, including 25 men and 23 women, with an average age of 20 years. The subungual exostosis was located on the hallux in 34 cases, the second toe in 10 cases, and the index finger in 2 cases. Nail trauma was found in 27 patients. The tumor was entirely excised in all the patients. CONCLUSION: Large series of subungual exostosis are reported by orthopedic surgeons. However, our outpatient consultation for nail disorders allowed the diagnosis and appropriate management of subungual exostosis.

First Subungual Cryptococcosis Described in an HIV Patient with Disseminated Cryptococcosis.

BACKGROUND: Cutaneous cryptococcosis occurs in 10-15% of patients with disseminated cryptococcosis. It typically presents as papulonodular molluscum-like lesions, but it can also produce a wide variety of lesions. Cryptococcal infection of the nail unit has never been reported. CASE REPORT: A 28-year-old woman with a history of HIV with disseminated cryptococcosis in complete remission was referred to evaluate a subungual swelling of the right middle finger. Examination revealed an ulcero-burgeoning nodule over the right middle finger's subungual area with onycholysis, eschar, and erosion. An excisional biopsy was performed. Histopathological analysis demonstrated multiple histiocytic granulomas centered by encapsulated yeast cells. Culture grew Cryptococcus neoformans var. neoformans. After 9 months of follow-up, there was no recurrence of the lesion. DISCUSSION: It is the first reported case of nail involvement in the course of cutaneous cryptococcosis. Definitive diagnosis required pathology and culture. Cryptococcal infection of the nail unit was recalcitrant to systemic therapy while the remaining infection cleared. Our case report suggests that surgical excision associated with systemic therapy is the best treatment approach for subungual cryptococcosis. Recognition of rare manifestations of cutaneous cryptococcosis, such as ours, is essential because HIV cases increase continuously.

[Cutaneous complications following hematopoietic stem cell transplantation]. / Complications cutanées post-allogreffe de cellules souches hématopoïétiques.

BACKGROUND: Hematopoietic stem cell allograft is a treatment for patients with severe constitutional or acquired hematopoietic system diseases. This act is always linked to complications requiring multidisciplinary care. Our study describes the post-allograft cutaneous complications. METHODS: A prospective study was conducted at the Hematology department of "20 Août Hospital" in Casablanca during a period going from January 2018 to December 2020; including all patients who presented acute or chronic cutaneous complications post-allograft. RESULTS: Twenty-five patients were included. All patients received induction chemotherapy (Busulfan/Fludarabine or Busulfan/Melphalan). A skin infection was found in 8 patients : four cases of Malassezia folliculitis, one case of perineal zona, one case of genital herpes, one case of varicella and one case of Candida sepsis. The acute graft versus host reaction was found in 3 patients, revealed by an erythematous rash all over the body. The chronic graft versus host reaction was found in five patients on a lichenoid form. Nine patients had a hyperpigmentation of the folds followed by detachment in the same areas, concluding to a Busulfan toxidermy. DISCUSSION: Hematopoietic stem cell allograft has many complications. The literature mainly specifies hematological and digestive complications, while skin complications are little described. Our series is special by reporting different types and mechanisms of skin complications that can occur; with a predominance of skin graft-on-host reactions and infections. It also reports an unusual Busulfan toxidermy.

Spontaneous regression of angiolymphoid hyperplasia with eosinophilia (AHLE): A case report.

INTRODUCTION: and importance - Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign microvascular proliferation. It is clinically characterized by intradermal or subcutaneous papules and/or nodules. Treatment consists mainly of surgery or laser therapy, with a high recurrence rate. Spontaneous regression is rare but possible. CASE PRESENTATION: A 72-year-old man presented for an erythematous plaque on his scalp. Physical examination demonstrated a large angiomatous indurated subcutaneous plaque. The lesion was not pulsatile. The doppler ultrasound revealed no underlying vascular malformation. The biopsy revealed an ALHE. No further treatment was carried out. During the follow-up, the lesion gradually subsided. After one-year follow-up, the lesion had regressed with no sign of recurrence. CLINICAL DISCUSSION: There are different treatment modalities for AHLE management, including surgical excision, cryotherapy and lasers. However, none of these modalities has provided consistent results and recurrence after treatment is common.). Rare instances of spontaneous regression have been reported. CONCLUSION: Given the rarity of cases of spontaneous resolution, ALHE may be considered as a chronic condition, especially since recurrences are frequent. A "wait and see" approach can be proposed in some specific situations.

[Stewart-Treves syndrome]. / Syndrome de Stewart-Treves.

Détention d'armes et permis de chasser. Les armes à feu sont la cause de plus d'un millier de décès chaque année, représentant ainsi un objectif de santé publique conséquent. Pour les armes à feu de type B et C, le tir sportif et le permis de chasse, le médecin traitant joue un rôle crucial, délivrant à chaque fois un certificat selon des modalités bien particulières.

[Generalized old world leishmaniasis: first Moroccan case in an immunocompetent adult?] / Leishmaniose généralisée de l'Ancien monde : Premier cas marocain chez un adulte immunocompétent ?

Background: Post-kala-azar dermal Leishmaniasis (PKDL) is a rare skin syndrome observed after treatment of visceral Leishmaniasis (VL) with pentavalent antimonial organic salts, never described in Morocco before. Here we report a case in an immunocompetent adult. Case: A 36-year-old-man from Tata in southern Morocco, with a history of visceral Leishmaniasis 2 years before and treated with meglumine antimoniate and amphotericin B with good clinical course, was hospitalized in dermatology for an erythematous papulo-nodular closet of the face. Six months ago, he presented oral mucosa involvement, then 3 months later, cutaneous lesions appeared on the face. The dermatological examination revealed a papulo-nodular erythematous closet extending to the nose and both cheeks, crusty and lupoid lesions on the forehead, around the eyes and chin, associated with an ulcerative and painless lesion on the heeL. The examination of the oral mucosa revealed an ulceration of the posterior third of the tongue and a papillomatous aspect of the soft palate. The skin biopsy and smear found some amastigote forms of Leishmania bodies. ITS1 PCR was positive (genus Leishmania). The HIV serology was negative. The diagnosis of PKDL was then evoked. The patient received intra-muscular injections of meglumine antimoniate with good progress. Conclusion: To our knowledge, this is the first case of generalised leishmaniasis suggesting PKDL reported in a Moroccan immunocompetent adult.

Nail Mycosis Fungoides: A New Case.

Ungueotropic mycosis is a rare form of mycosis fungoides. We present the case of a 32-year-old female patient with advanced tumor stage mycosis fungoides, presenting a phanerial involvement with lymphoid infiltration of the nails and scalp confirmed by histology and immunohistochemistry.

Oral White Sponge Nevus: An Exceptional Differential Diagnosis in Childhood.

White sponge nevus is an autosomal dominant skin disorder characterized by white, irregular, diffuse plaques mainly affecting the oral mucosa. Histological findings of white sponge nevus are characteristic but not pathognomonic. We report a case of an oral white sponge nevus in a 6-year-old girl, which poses a problem in differential diagnosis with oral candidiasis. No treatment was performed because of the benign and asymptomatic nature of the lesions.