Familial Cases of PENS: A Review of the Literature.

Papular epidermal nevus with "skyline" basal cell layer (PENS) is a keratinocytic nevus that can occur sporadically or has a familial transmission. There are 5 families reported with PENS, in which there are 2 family members affected with each case. We present the sixth familial case, with the peculiarity of being the first time in which there are 3 family members with PENS, while reviewing the other cases described until now. In addition, we present a new histopathological finding, an inflammatory lichenoid infiltrate on the upper dermis in PENS lesions. This finding could be the result of trauma to the biopsied lesion, or it may represent a new inflammatory histological variant.

Pediatric Kaposi's sarcoma associated with immune reconstitution inflammatory syndrome.

Immune reconstitution inflammatory syndrome (IRIS) represents paradoxical immune-mediated inflammation in response to an infecting pathogen, occurring after initiation of antiretroviral therapy (ART), concomitantly with immune system recovery. It has also been described in Kaposi's sarcoma (KS). We report a case of a 9-year-old Guinean girl, who developed Kaposi's sarcoma, following introduction of ART. KS associated with immune reconstitution inflammatory syndrome is rare, especially in children, but with the increased use of ART is becoming more prevalent.

Drug-induced accelerated nodulosis: review of the literature.

Accelerated nodulosis, the rapid progression/extension of preexisting nodules, is a recognized complication of immunomodulatory therapy, occurring mostly in patients with rheumatoid arthritis treated with methotrexate. As of today, its physiopathology remains incompletely understood, and there are no standardized guidelines regarding its management. Here, we conduct a literature review of the reported cases of drug-induced accelerated nodulosis and add our case of a 79-year-old female with an atypical clinical presentation.

Deep dermatophytosis caused by Trichophyton rubrum in immunocompromised patients.

In immunosuppressed patients, dermatophytosis can be more invasive, affecting the dermis and subcutaneous tissues. The authors describe the cases of two patients with kidney and heart transplanted, respectively, that developed a deep dermatophytosis caused by Trichophyton rubrum, confirmed by culture and DNA sequencing. Both patients had concomitant onychomycosis, and both were treated with itraconazole for about two months, which was interrupted due to pharmacological interactions with the immunosuppressive drugs and switched to terbinafine, leading to clinical resolution within four months. Deep dermatophytosis should be considered when dealing with immunocompromised patients, especially when a superficial dermatophytosis is present. Oral treatment is necessary and terbinafine is a preferable option in solid organ transplant recipients because it has less pharmacological interactions.

Borderline CD30+ cutaneous lymphoproliferative disorder: report of a case with expression of cytotoxic markers and response to clarithromycin.

CD30+ cutaneous lymphoproliferative disorders (CLPDs) are usually characterized by a benign clinical course. The prognostic value of cytotoxic markers in these lymphomas has not been evaluated in large series. We describe a case of borderline CD30+ CLPD with cytotoxic phenotype, presenting in a 22-year-old male patient as an ulcer on the forearm. He reported having had similar ulcers on the buttock and thigh that spontaneously regressed over the course of 1 year. The lesion resolved with a single course of clarithromycin; a subsequent lesion, too, responded to clarithromycin, and no recurrences or systemic involvement have been documented in the 9-month follow-up. A conservative approach in the management of CD30+ CLPD is recommended. We believe that the anti-inflammatory and apoptotic effects of clarithromycin on T cells may have hastened the remission process.

Cutaneous Rosai-Dorfman disease: a challenging diagnosis.

Rosai-Dorfman disease is a rare benign histiocytic proliferative disease of unknown cause that, in exceptional cases, presents with lesions confined to the skin. Clinically variable types of lesions such as papules, nodules and plaques have been reported. We present a case of a 27-year-old woman with a 1-year history of erythematous papular and nodular lesions on the malar and right axillary regions, previously misdiagnosed as acne. She reported no fever, malaise or weight loss, while physical examination and laboratory workup were normal. Bacteriological and mycobacteriological cultures were negative. Histopathological findings showed dense infiltration of inflammatory cells involving the entire dermis, consisting of large macrophages with emperipolesis, S100 and CD68 positive, neutrophils, eosinophils, lymphocytes and plasma cells. The patient was treated with oral prednisolone without improvement. Dapsone was subsequently initiated with favourable clinical response. The present article aimed to emphasise the clinical and histological differential diagnosis and share the treatment experience.

Brown plaques on the lower back.

A 12-year-old girl presented with a 2-year history of a 10 cm area of asymptomatic brown coalescent plaques of coalescent, linearly arranged papules on the lower back. No other physical or systemic abnormality was detected. Routine laboratory investigations, including thyroid function tests, were normal. The histopathological examination revealed hyperkeratosis, papillomatosis, and elongated rete ridges in the epidermis. On special staining (Alcian blue), mucin deposition was seen in the papillary dermis. The remaining dermis was normal. A diagnosis of Mucinous Nevus was made.

Lupus miliaris disseminatus faciei: a case report.

UNLABELLED: A 33-year-old man presented to our clinic with asymptomatic red-brown, dome-shaped papules, distributed bilaterally on the central area of the face (forehead, lower portions of the eyelids, nasolabial folds, and perioral areas) these had evolved over a period of about 1 year. A skin biopsy, taken from a lesion on the forehead, revealed an epithelioid cell granuloma with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells. The chest X-ray and the results of the laboratory studies were within normal limits; the Mantoux test was negative. The patient was treated with minocycline 100 mg/day for 4 months. There was significant clinical improvement, but papular lesions remained on the forehead. Later on, treatment with systemic steroids for 7 months resulted in the resolution of most lesions. COMMENT: Lupus miliaris disseminatus faciei (LMDF) is an uncommon, chronic, inflammatory dermatosis characterized by red-to-yellow or yellow-brown papules of the central face, particularly on and around the eyelids. Originally, LMDF was considered to be a variant of lupus vulgaris or a tuberculid because of the histology, but there has been no evidence to date supporting a link to tuberculosis. Some authors consider LMDF to be a granulomatous forms of rosacea. However, our case supports the concept that it is a distinct entity.