Doxycycline monotherapy for pyoderma vegetans: a case report.

Background: Pyoderma vegetans (PV) is a rare neutrophilic dermatosis of unknown etiology. Currently, there are no treatment guidelines for PV. Systemic steroids are often used as first-line therapy, but recurrence upon discontinuation or tapering is common.Materials and methods: We tested the efficacy of doxycycline at a dose of 200 mg/d to treat resistant PV.Results: After 4 weeks of treatment we noticed a significant improvement in the clinical appearance of PV.Conclusions: Our case demonstrates the potential utility of doxycycline as a systemic steroid-sparing agent in the treatment of PV.

Erythroderma with total scarring alopecia.

A woman in her 50s presented with total scarring ulcerated alopecia evolving for 10 years, and a recent budding tumour on the lower lip. Clinical examination revealed an associated exfoliated and infiltrated erythroderma with ulcerated cutaneous tumours of the legs, palmoplantar hyperkeratosis, hepatosplenomegaly and diffuse lymphadenopathies.Dermatoscopy of the scalp, leg tumours and infiltrated skin showed a typical yellowish background overlaid by arborescent vessels and whitish areas. Cutaneous biopsies of the same areas found sarcoidotic granulomas. Lip biopsy found an associated well-differentiated squamous cell carcinoma. Investigations confirmed the diagnosis of systemic sarcoidosis with an elevated converting enzyme level, mediastinal calcified lymphadenopathies in CT scan and deep localisations in positron emission tomography scan (thyroid, lymph nodes, mediastinum, liver, spleen and adrenal glands).The patient was treated with oral prednisone for sarcoidosis and intramuscular bleomycin followed by surgery and radiotherapy for squamous cell carcinoma. Sarcoidotic lesions healed, but a recurrence of her carcinoma led to death.

Dermatoscopic and clinical features of congenital or congenital-type nail matrix nevi: A multicenter prospective cohort study by the International Dermoscopy Society.

BACKGROUND: Congenital nail matrix nevi (NMN) are difficult to diagnose because they feature clinical characteristics suggestive of adult subungual melanoma. Nail matrix biopsy is difficult to perform, especially in children. OBJECTIVE: To describe the initial clinical and dermatoscopic features of NMN appearing at birth (congenital) or after birth but before the age of 5 years (congenital-type). METHODS: We conducted a prospective, international, and consecutive data collection in 102 hospitals or private medical offices across 30 countries from 2009 to 2019. RESULTS: There were 69 congenital and 161 congenital-type NMNs. Congenital and congenital-type NMN predominantly displayed an irregular pattern of longitudinal microlines (n = 146, 64%), reminiscent of subungual melanoma in adults. The distal fibrillar ("brush-like") pattern, present in 63 patients (27.8%), was more frequently encountered in congenital NMN than in congenital-type NMN (P = .012). Moreover, congenital NMN more frequently displayed a periungual pigmentation (P = .029) and Hutchinson's sign (P = .027) than did congenital-type NMN. LIMITATIONS: Lack of systematic biopsy-proven diagnosis and heterogeneity of clinical and dermatoscopic photographs. CONCLUSION: Congenital and congenital-type NMN showed worrisome clinical and dermatoscopic features similar to those observed in adulthood subungual melanoma. The distal fibrillar ("brush-like") pattern is a suggestive feature of congenital and congenital-type NMN.

[Sneddon-Wilkinson disease: a case report]. / Maladie de Sneddon-Wilkinson: à propos d'un cas.

Sneddon-Wilkinson disease is a benign amicrobial pustulosis belonging to the spectrum of neutrophilic dermatoses. It is characterized by evident stereotypic clinical features, including, in most cases, pustular lesions of the trunk and prominent skin folds. This pustulosis may be associated with other diseases (IgA monoclonal gammopathy, rheumatoid arthritis, neoplasms or other neutrophilic dermatoses) and therefore requires regular follow-up. This disease has a relapsing-remitting course. First line therapy is dapsone. We here report the case of a 49-year-old patient with amicrobial pustulosis (Sneddon-Wilkinson disease).

[Naevus unius lateris: a confusing entity (a case report)]. / Nævus unius lateris: un nævus déroutant (à propos d´un cas).

Naevus unius lateris is a rare congenital hamartoma, originating from the ectoderm. It is considered as a verruquous variant of the epidermal naevus. Given its extensive unilateral distribution, it is frequently associated with neurological, musculoskeletal, auditory and visual abnormalities. We here report the case of a 9-year-old child with nevus unius lateris associated with neurological and eye abnormalities.

Bullous pemphigoid-like rash revealing hypereosinophilic syndrome.

Hypereosinophilic syndrome (HES) is a rare haematologic disorder characterised by unexplained, persistent eosinophilia with organ involvement. We report the case of a 74-year-old patient who presented with a pemphigoid-like bullous rash as the only manifestation of an HES. This report highlights the importance of the dermatological manifestations of this potentially life-threating condition that may initially present as a skin-limited disease. Bullous pemphigoid (BP) is a very common autoimmune blistering dermatosis that may share common clinical and histological features or associate with an HES. HES should be considered when patients diagnosed with BP demonstrate atypical course under corticosteroids and/or recalcitrant blood eosinophilia.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus: two cases report.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus (TEN-like ACLE) is a rare manifestation of systemic lupus erythematosus (SLE). Because of its rarity, little is known about this entity. In this report, we describe a case of two women previously diagnosed with SLE that presented TEN-like skin lesions. The common elements in both patients were the initial disposition of the lesions on the photoexposed areas, the positivity of Nikolsky´s sign, the discrete mucosal attrition compared to that observed during TEN, and the simultaneous appearance of dermatological lesions with an extra-cutaneous flare of lupus disease. The skin biopsy in both cases showed epidermal necrosis with an identification of lupus band on direct immunofluorescence. Systemic corticosteroids were used with a good evolution after 2 weeks. Skin damage is an indicator of disease activity, and careful search for extracutaneous involvement is obligatory to prevent further complications.

A polymorphous cutaneous tuberculosis.

Cutaneous tuberculosis is uncommon, comprising 1%-2% of all extrapulmonary manifestations. It has a wide clinical spectrum. The clinical presentation depends on the route of the infection, the pathogenicity of the bacteria, and the immune status of the host. Association of multiple forms in a same patient is uncommon. We report a case of disseminated tuberculosis with polymorphous cutaneous lesions in an immunocompetent woman, combining scrofuloderma, gummas, and sporotrichoid lesions, along with tuberculous adenitis and bone tuberculosis.

Skin cancer knowledge and attitudes in the region of Fez, Morocco: a cross-sectional study.

BACKGROUND: The prevalence of skin cancers is constantly increasing in Morocco, and they have gradually become more aggressive due to a significant delay in the diagnosis. Our aim was to assess the levels of awareness and the influencing factors related to skin cancer knowledge in Morocco. METHODS: This cross-sectional study was carried out in Morocco through the medium of a validated questionnaire, which contained several items - demographics, skin cancer knowledge and attitudes towards skin cancer patients- during a period of 1 year (2014). RESULTS: Out of the 700 participants enrolled in the study, 17.9% had never heard of skin cancer, 32.5% had a low score of skin cancer knowledge, 66.7% had a moderate score, and only 0.85% had a high score of skin cancer knowledge. Further, 15.1% of the participants were under the assumption that this cancer is contagious. The sun was the most incriminated risk factor in skin cancer occurrence by 74.3% of the participants, and 57.9% of them believed that prevention is important through using various means of photoprotection. After univariate and multivariate analysis, the influencing factors related to the skin cancer knowledge in Morocco were: the socioeconomic status (P = 0.003, OR = 7. 3) and the educational level (p < 0.001, OR = 20. 9). CONCLUSIONS: Due to the lack of knowledge or the underestimation of skin cancer in our study population, efforts are needed to promote skin cancer surveillance behaviors in Morocco.

The TLR2 and TLR4 gene polymorphisms in Moroccan visceral leishmaniasis patients.

Visceral leishmaniasis (VL) is endemic in the Mediterranean basin and leads to the most severe form of Leishmania infection, lethal if left untreated. However, most infections are sub-clinical or asymptomatic, reflecting the influence of host genetic background on disease outcome. This study aimed to investigate possible association of TLR4 Asp299Gly, TLR4 Thr399Ile and TLR2 Arg753Gln polymorphisms with VL in Moroccan children. We enrolled 119 children with VL caused by Leishmania infantum as well as 138 unrelated children, 95 asymptomatic subjects and 43 healthy individuals who had no evidence of present or past infection. Polymorphisms were genotyped by polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) and amplification refractory mutation system assay (ARMS-PCR). Results showed significant differences in genotype Thr399Ile and recessive model frequencies between VL and delayed-type hypersensitivity (DTH+) groups (p=0.018, OR=0.414CI 0.195-0.880; p=0.029, OR=0.448CI 0.214-0.938], respectively) by having the amino-acid threonine polymorphism as a reference in the VL group. Concerning the Asp299Gly there were a significant associations when comparing VL vs DTH+ (Asp299Gly genotype p=0.002, OR=0.326CI 0.158-0.671, allele frequencies p=0.033, OR=0.396CI 0.164-0.959, recessive model p=0.002, OR=0.343CI 0.172-0.681) and DTH+ vs DTH- groups (Asp299Gly genotype p=2.160E-4, OR=3.065CI 1.672-5.618, Gly299Gly genotype p=0.047, OR=0.368CI 0.299-0.452, allele frequencies p=1.406E-7, OR=29.571CI 3.907-223.8, recessive model p=4.370E-14, OR=36.965CI 8.629-158.3), by having the aspartic acid polymorphism as a reference these results suggest that the allele A (savage) confer protection against the clinical manifestations but not against the infection. Furthermore, there was a significant association regarding the Arg753Gln genotype (p=0.002, OR=0.326CI 0.158-0.671), allele frequencies (p=0.033, OR=0.396CI 0.164-0.959) and when applying a recessive model (p=0.002, OR=0.343CI 0.172-0.681) in the VL vs DTH+ groups. The same results was observed when comparing DTH+ vs DTH- groups (p=4.136E-6, OR=0.211CI 0.104-0.428), allele frequencies (p=0.008, OR=0.327CI 0.137-0.779) and recessive model (p=1.748E-5, OR=0.244CI 0.124-0.480). The results provide evidence that allele C in Thr399Ile and allele G in Arg753Gln polymorphisms may lead to protection against the clinical disease. Our data provide insights into the possible role of TLR2 and TLR4 variations in VL susceptibility.