Characteristics of DRESS Syndrome in the Elderly: A Comparative Study of 55 Patients.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare drug reaction characterized by a skin rash, eosinophilia, and organ involvement. Objective: Our purpose is to focus on the clinical and epidemiological characteristics of DRESS in the elderly and to identify the incriminated drugs. Methods: This is a retrospective study including patients, hospitalized for DRESS with a RegiSCAR ≥4. The population was divided into 2 groups according to age: 65 years or older (G1) and <65 years (G2). The statistical study was performed using the comparative and multivariate analysis. Results: We included 55 patients (30.9% G1 and 69.1% G2). Skin manifestations were comparable in both groups. Lymphadenopathy was less common in G1 with a statistically significant difference (P = 0.012). Renal impairment was more frequent in the elderly with a statistically significant result (P = 0.005). DRESS in the elderly group was significantly associated with the occurrence of sepsis (P = 0.008). Allopurinol was the most common culprit associated with DRESS in G1 (P = 0.001). Relapses and recurrences were comparable in both groups (P = 0.71). Conclusions: DRESS in the elderly is associated with a high risk of complications, mainly kidney involvement and sepsis. Allopurinol is the most incriminated drug.

Follicular Basaloid Hamartoma and Associated Cutaneous Tumors: Clinical, Histopathological and Dermoscopic Aspects.

Background: Basaloid follicular hamartoma is an uncommun benign neoplasm of the hair follicule. The linear form of basaloid follicular hamartoma can be associated with basal cell carcinoma.OBJECTIVE: We report a case of a patient with multiple basal cell carcinomas developing on a congenital lesion allowing the diagnosis of basaloid follicular hamartoma on histopathology. Methods: Each lesion was evaluated by two dermatologists. All biopsy specimens were routinely stained with hematoxylin-eosin. Results: A 76 year-old-man consulted our dermatology departement for erythematous papules spread over the left lower limb. The lesion had been evolving since birth with flesh-colored to pinkish papules following Blaschko's lines from the root of the thigh to the foot. Three months before consultation, the papules increased in size leading to nodules with sometimes an ulcerated center. Dermoscopy under polarized mode displayed for ulcerated lesions (A) yellow-brownish crusts and ulceration surrounded by blue-grey ovoid nests, subtle short white streaks, brown dots and linear/arborising vessels. For nodular lesions (B), dermoscopic features are white pinkish hue, dotted and linear vessels, brown dots, blue-grey structureless areas and white prominent shiny streaks. There were some more erythematous inflamed and eroded areas in the background with a reversed honeycomb white network on dermoscopy (C), polymorphous vessels, whitish scales, ulcerations and milia-like cysts. The background lesion showed varied dermoscopic structures on a flesh colored slightly pinkish bottom (D). Histolopathology concluded for lesions A to an infiltrating and nodular basal cell carcinoma, (B) and (C) to fibroepithelioma of Pinkus and (D) to basaloid follicular hamartoma. Conclusion: Several case reports have documented dermoscopic features of a solitary basaloid follicular hamartoma. However, further studies are required to specify any reproducible features.

Inverted Follicular Keratosis: A Retrospective Study Over 10 Years.

Inverted follicular keratosis (IFK) is a rare benign tumour of the follicular infundibulum characterized by exo-endophytic growing. Generally, the diagnosis of IFK is histopathologically made because clinical differentiation from other lesions is difficult. We present a retrospective series with thirteen patients with histologically confirmed IFK to evaluate the epidemiological, clinical and histopathologic characteristics of IFK. The mean age of the patients at the time of the excision was 53 years with extremes ranging from 19 to 82 years. The sex ratio M/F was 3.3. The lesions affected the face in nine patients mainly the moustache, followed by the scalp in three cases and the arm in one case, and 92% of the localizations are sun-exposed. The diagnosis was never clinically evoked. The lesion had a pink colour in ten cases and was pigmented in two cases and hypopigmented in one case. More than half of the lesions (53%) had a keratotic centre. Histopathological examination showed endophytic intradermal proliferation of basaloid cells with a variable degree of squamoid differentiation. Horn cysts were present in all cases. According to our series, the IFK occurs predominantly in young men, in the face and more specifically in the moustache. Dermoscopy may suggest the diagnosis of the IFK. In fact, a histopathological examination is the gold standard for the diagnosis of the IFK and helps differentiate these benign tumours from possible malignant neoplasms.

miRNA implication in the pathogenesis and the outcome of Tunisian endemic pemphigus foliaceus.

Pemphigus foliaceus (PF) is a bullous autoimmune skin disease diagnosed through sera and skin analyses. PF severity is associated with maintained anti-Dsg1 sera levels and its prognosis is unpredictable. MicroRNA (miRNA), dynamic regulators of immune function, have been identified as potential biomarkers for some autoimmune diseases. This study aimed to assess the miRNA expression of miR-17-5p, miR-21-5p, miR-146a-5p, miR-155-5p and miR-338-3p using quantitative real-time PCR in peripheral blood mononuclear cells (PBMC) and lesional skin samples from untreated and treated PF patients (both remittent and chronic) over 3 months. Overall, miRNA expression was significantly higher in PBMC than in biopsy samples. Blood miR-21 expression was increased in untreated patients compared to controls and had a diagnostic value with an AUC of 0.78. After 6 weeks, it decreased significantly, similar to anti-Dsg1 antibodies and the PDAI score. In addition, a positive correlation was observed between cutaneous miR-21 expression and the disease activity score. Conversely, cutaneous expressions of miR-17, miR-146a and miR-155 were significantly higher in treated chronic patients compared to remittent ones. The cutaneous level of miR-155 positively correlated with pemphigus activity, making it a potential predictive marker for patients' clinical stratification with an AUC of 0.86.These findings suggest that blood miR-21 and cutaneous miR-155 can be used as supplemental markers for PF diagnosis and activity, respectively in addition to classical parameters.

Trichofolliculoma: A Case Series.

Trichofolliculoma (TF) is a rare benign adnexal follicular tumor, described as hamartoma with follicular differentiation according to some authors. It typically appears during adulthood on the face or scalp as an isolated nodule with protrusion of central tufted hairs. We present a retrospective series of eleven patients with histologically confirmed TF to evaluate epidemiological, clinical, and histopathologic characteristics. The mean age at excision was 46 years with extremes ranging from 20 to 75 years. The sex ratio M/F was 0.37. Clinical presentation was a papule or nodule with an average diameter of 6,7mm (2-15 mm), firm with central pit in 54 percent and visible emerging vellus hairs in 18 percent of cases only. The localization was on the face in seven cases (63.6%) and only four cases were located outside the face (scalp [n=2], sub mammary fold [n=1] and shoulder [n=1]). Histologically, a cystically dilated hair follicle containing keratinous material with several mature and immature branched follicular structures is described in all cases. According to our series, TF occurs predominantly in women without age predilection, in the face. Central tufted hairs are only found in a minority of cases corresponding histologically to many secondary vellus hair follicles. In fact, histopathological examination is the gold standard for the diagnosis because clinical diagnosis could be challenging. Histology and dermoscopy may vary according to the age of the lesion. To date, only few case series have been published.

Lichenoid lesions around the anus: An unusual site of Hailey-Hailey disease.

The anal region is an unusual site of Hailey-Hailey disease. It manifests with lichenoid lesions with crusted erosions around the anus. It should be differentiated from condylomata acuminata, extramammary Paget disease, and bowenoid papulosis.

A rare case of melanoma erysipeloides.

Malignant melanoma presenting as an inflammatory skin metastasis has been described but is an exceedingly rare phenomenon. We report a case of inflammatory metastasis of cutaneous melanoma (CM).

A verrucous tumor of the leg: Atypical case of Syringocystadenoma Papilliferum.

Syringocystadenoma Papilliferum (SCAP) is a benign adnexal tumor. Most of the cases present with a solitary lesion in the head and neck region at birth or in early childhood. In fact, only two cases of SCAP on the lower leg have been reported in the literature. We report an atypical case by its location and its clinical aspect.

Pigmented and polypoid tumor of the pubis.

Seborrheic keratosis is a common benign epidermal tumor occurring in patients aged over 50 years. It is located preferentially in the trunk, head and neck. The genital location is rare. We report a case of 59 year-old-men presenting a seborrheic keratosis of the pubis.

A case of a verrucous and pseudotumoral mass on the leg that resolved with cryotherapy.

Various clinical forms of cutaneous leishmaniasis can be encountered such as: ulcerated, lupoïd, sporotrichoïd and other rare forms (eczematiform, erysipeloid, psoriasiform, verrucous, and pseudotumoral). We report an atypical presentation of verrucous and pseudotumoral cutaneous leishmaniasis that resolved following a course of cryotherapy.

Slow-growing pedunculated nodule.

There have been limited reported cases of pedunculated basal cell carcinoma(BCC). Our case is original, and it combines two rare aspects of CBC: Pedunculated and giant. This uncommon aspect is rarely encountered.

Compound blue nevus: A misleading pigmented melanocytic tumor.

Compound blue nevus had clinical and histological similarities with other heavily pigmented melanocytic tumor, like the pigmented epithelioid melanocytoma. Distinctive genomic aberrations have allowed differentiating it. The defining characteristic of blue nevi family is the presence of activating mutations in the G protein α-subunits, GNAQ and GNA11.

[A rare cause of AA amyloidosis: Hereditary epidermolysis bullosa]. / Une cause rare de l'amylose AA : les épidermolyses bulleuses héréditaires.

INTRODUCTION: Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis. OBSERVATIONS: Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases. CONCLUSION: Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

Hymenal polyps in two infants.

Pathological conditions of the hymen are rare in everyday medical practice. Hymenal polyps are polypoid formations originating from the hymenal rim and are benign and disappear spontaneously within a few weeks of onset. We report two cases of hymenal polyps in two infants.

Malignancy in a retrospective cohort of 17 patients with Dermatomyositis or Polymyositis in southern Tunisia.

INTRODUCTION: The prognosis of dermatomyositis (DM)/ polymyositis (PM) in adults is partly related to their association with neoplasia. The aim of our study was to report the epidemiologic, clinical, paraclinical, therapeutic and evolutionary aspects of DM associated with malignancy in patients from Sfax, south eastern of Tunisia. METHODS: A retrospective cohort study of patients with DM or PM admitted in Dermatology and Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2015. Cases of DM or PM associated with malignancy were retained. RESULTS: Seventeen cases (13.5%) of DM or PM associated with malignancy were noted. Fourteen patients had DM and 3 patients had PM. The Sex ratio M / F was 0.3 and the mean age at diagnosis was 56.5 years. In DM patients, malignancy preceded the myositis in 64.2% of cases. In PM patients, only one patient was known to have breast cancer and the myositis revealed the cancer for 2 others. Treatment consisted of corticosteroids associated with methotrexate in 4 cases. Outcome was fatal in 5 cases (29.4%), due to the underlying cancer in 3 cases. Swallowing disorders related to DM/PM were responsible for death in 2 cases. CONCLUSION: There are no specific clinical or biological features in paraneoplastic DM. In our series, breast neoplasm represented the first cancer associated with DM. Cancers of nasopharynx, colon and urinary tract had the second position.

Paucisymptomatic Dermoid Cyst with Fatal Outcome.

Dermoid cysts of the central nervous system can cause devastating complications because of the mass effect of meningitis due to sinus tract. We report the case of a 5-month-old girl who presented with a crusted lesion of the occipital region of the scalp. Clinical examination noted skin abnormalities suggestive of occult dysraphism. Magnetic resonance imaging (MRI) was recommended, however, 40 days after this evaluation, and before the MRI could be performed, the girl presented with neurologic complications. Unfortunately, the diagnosis of dermoid cyst was made after the onset of severe complications that led to her death. The findings in this case emphasize the importance of more prompt MRI evaluation, particularly in cases where cranial or spinal dysraphism is suspected to have any connection to the skin as a pit or tract. Should we perform an urgent MRI for any cutaneous sign of dysraphism to avoid a dramatic evolution?