Merkel cell number and distribution, and CD200 expression in patients with lichen planopilaris and discoid lupus erythematosus.

BACKGROUND: Immune mechanisms are considered to be responsible for the pathogenesis of cicatricial alopecia in lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) diseases. CD200 has an immunomodulatory function in hair follicles. The functions of Merkel cells (MCs) in hair follicles remain to be fully understood. OBJECTIVE: This study aimed to determine the number and distribution of MCs as well as CD200 expression in patients with DLE and LPP. METHODS: Using immunohistochemistry, the number and distribution of MCs (staining with CK20) and CD200 expression in biopsy specimens of LPP and DLE patients were compared with control group patients. RESULTS: The number of follicular MCs, total MCs, mean follicular MCs, and CD200 expression were significantly lower in the case groups compared to the control group. In CD200- cases, the number of follicular MCs and mean follicular MCs were significantly lower than in CD200+ cases. Retrospective design, lack of data regarding the history of alopecia in the control group, and unknown stage of disease in patients were the limitations. CONCLUSION: MC loss might play a role in immune privilege collapse in hair follicles. This study is novel in terms of investigating MCs in DLE and LPP patients.

Early recognition of chronic rejection in a face allotransplant patient with alopecia.

The features of chronic rejection (CR) in full-face vascularized composite allotransplantation (VCA) are not well-known. Herein, we report a full-face transplant patient that experienced two episodes of acute rejection (AR) and three episodes of AR/CR over the course of 6-years. The patient noticed a small, round patch of hair loss in his beard 9 months after the second AR episode, which occurred 21 months post-transplantation. Biopsy of the alopecic patch showed lichen-planopilaris-like features, which were suggestive of early CR. Despite an increase in immunosuppressive dosages, the alopecia progressed. Following the second and third AR/CR episodes, the alopecia became more pronounced, with the addition of hyperpigmentation as well as sclerosis and telangiectasia. The findings of multiple biopsies showed CR. Based on these findings we think that alopecia with lichen-planopilaris-like histopathological features similar to grade III AR features, particularly in hair follicles appears to be an early finding of CR in the presented patient. The findings further indicate that follicular involvement may be a significant feature of CR in VCA patients and that it can present prior to sclerosis, vasculopathy, or loss of adnexa. The present case is uniquely important because of the distinctive presentation of CR, with hair follicles clinically and histopathologically affected, leading to progressive and irreversible alopecia with lichen-planopilaris-like histopathology.

Epidermodysplasia Verruciformis After Hematopoietic Stem Cell Transplantation in a Patient With Severe Combined Immunodeficiency Syndrome.

ABSTRACT: Epidermodysplasia verruciformis (EV) is a rare dermatologic disorder that is characterized by skin-colored-to-light brown flat, discrete or confluent papules resembling verruca plana. EV is divided into 2 forms: a classical genetic form and an acquired form. Classical genetic EV is caused by mutations in EVER1 and EVER2 genes. Acquired EV develops in immunocompromised patients such as HIV-positive patients and transplant recipients. Patients with a prior history of hematopoietic stem cell transplantation (HSCT) have tendency to develop generalized verrucosis. We report an extraordinary case of disseminated epidermodysplasia verruciformis seen in a 7-year-old boy diagnosed with severe combined immunodeficiency syndrome who had undergone HSCT. He had plane, brown papules involving his face, forearms, neck, anterior chest, nape, back, and knees. Cutaneous biopsy showed typical characteristic findings of EV: large cells with gray-blue cytoplasm and keratohyaline granules of different sizes in the granular and spinous layers. Herein, we present an unusual case of disseminated EV in a HSCT patient with typical histopathologic findings and treatment options.

A New Experimental Lymphedema Model: Reevaluating the Efficacy of Rat Models and Their Clinical Translation for Chronic Lymphedema Studies.

BACKGROUND: Our aim was to create a new rodent hind limb lymphedema model lacking the fibrosis effect induced by radiotherapy and subjected to the inhibition of lymphangiogenesis via sirolimus (rapamycin) to maintain a chronic lymphedema model and investigate its reliability for human treatment modalities. METHODS: Forty-two Sprague-Dawley rats were randomly assigned to 7 groups: (1) surgery control, (2) vehicle-surgery control, (3) vehicle control, (4) rapamycin control, (5) surgery with 1 mg/kg per day rapamycin, (6) surgery with 1.5 mg/kg per day rapamycin, and (7) surgery with 2 mg/kg per day rapamycin. All surgeries were performed on the right hind limbs, with the left hind limbs also considered as a control. The drug and its solvent were administered daily into the relevant groups intraperiteonally. The presence of lymphedema was investigated by weekly limb circumference measurements, microcomputed tomography, fluorescence lymphography using indocyanine green dye, and microscopic evaluation at the end of the sixth week to determine any histological changes in the hind limbs. RESULTS: In group 1, lymphedema was observed for 2 weeks (P = 0.032), whereas in groups 5, 6, and 7, lymphedema lasted for 3 weeks (P < 0.05.) Fluorescence using indocyanine green revealed that the edema was totally resolved after 6 weeks of surgery by a well-developed superficial lymphatic organization instead of the normal distinct vessel structure. Histologically, groups 1, 5, 5, and 7 demonstrated a significant increase in both the number of macrophages (P < 0.001) and newly formed lymphatic vessels in the right side surgically treated hind limb (P < 0.05). CONCLUSIONS: Despite the extreme surgical destruction and lymphangiogenesis inhibition in the rat model, the sustained lymphedema did not last >3 weeks. Because of the rapid neolymphangiogenesis in murines and a different wound healing mechanism, they should not be considered as an appropriate model for research on human lymphedema in first place.

A novel mutation of keratin 5 in epidermolysis bullosa simplex with migratory circinate erythema.

Epidermolysis bullosa simplex migratory circinate erythema (EBS-Migr) is an uncommon subtype of EBS. We report a case of EBS-MIGR with a novel heterozygous pathogenic mutation in exon 9 (frameshift deletion c.1650delC) and likely benign heterozygous mutation in exon 2 (missense c.591C > A) of keratin 5. This novel pathogenic mutation in KRT5 expands the molecular spectrum of this rare subtype of EBS.

A case of bullous pemphigoid induced by vildagliptin.

Bullous pemfigoid (BP), an autoimmune disorder, can also be induced by some medications. Vildagliptin is a new drug used to treat diabetes mellitus (DM). Recently, a few cases of vildagliptin-induced BP have been described in the literature. We report a patient with BP in which vildagliptin was thought to be as a possible causative agent. The awareness of BP development risk during gliptin therapy can prevent unnecessary usage of systemic drugs with serious side effects.

Childhood-Onset Keratosis Lichenoides Chronica Accompanied by Severe Hair Loss.

A 37-year-old woman attended the dermatology outpatient clinic because of recent hair loss from the eyebrows and axillae. Her past medical history revealed mild generalized erythema and hyperpigmented papules and plaques since childhood. On dermatologic examination, there were flat-topped, purple to brown hyperkeratotic lichenoid papules and linear plaques on the elbows, trunk, and buttocks, some of which coalesced into hyperpigmented reticular plaques on the axillae, neck, and groin. Mild erythema was noted. There was thinning and loss of hair of the eyebrows; severe loss of hair was noted in the axillae and genital regions (Figure 1). One of the lichenoid papules was biopsied. The specimen showed histopathologic findings of focal parakeratosis, irregular acanthosis, an increased granular layer, and focal vacuolar degeneration of the basal layer. Necrotic keratinocytes were also observed. Hyalinization and abundant melanin in the papillary dermis and marked congestion of blood vessels were noted (Figure 2). Clinicopathologic correlation of the case was consistent with keratosis lichenoides chronica (KLC).

Primary Nasal Tuberculosis in a 10-Year-Old Girl.

Nasal tuberculosis is a rare clinical entity which mainly presents in elderly people. Nasal tuberculosis has always been considered to be secondary to tuberculosis of the lungs, and in rare instances it is a primary infection, usually when mycobacteria are inhaled. We describe the case of a 10-year-old girl who was successfully treated for primary nasal tuberculosis. This patient is one of the very few children who have been reported to have primary nasal tuberculosis.

Is Intracytoplasmic Sperm Injection Success Affected by the Testicular Histopathology in Nonobstructive Azoospermic Patients?

OBJECTIVE: To investigate whether the histological changes observed in testicular tissue might be correlated with pregnancy outcome in intracytoplasmic sperm injection-testicular sperm extraction (TESE) cycles in nonobstructive azoospermic patients. Embryo quality was also tested in various types of histology. STUDY DESIGN: We retrospectively analyzed the pathology results of 209 testicular biopsies from nonobstructive azoospermic patients in our assisted reproductive technologies clinic. According to the final pathology reports, the study group was divided into the following categories: Group A (Sertoli-cell-only), Group B (maturation arrest), Group C (hypospermatogenesis), Group D (normal spermatogenesis), and Group E (mixed pattern). RESULTS: Spermatozoa were identified in 114 of 209 cases (54.5%). Sperm retrieval and presence of motile spermatozoa after TESE was highest in normal spermatogenesis and lowest in Sertoli-cell-only groups. Fertilization rate was highest in the normal spermatogenesis and hypospermatogenesis groups. Embryo grades on day 3 were comparable between groups. Clinical pregnancy per embryo transfer was also similar. Both fertilization and clinical pregnancy rates were statistically higher in favor of motile spermatozoa group. CONCLUSION: Testicular histopathology not only gives information regarding the probability of retrieving sperm at TESE, but also is a good predictor of fertilization. Once a motile spermatozoon is identified, the chance of clinical pregnancy might improve probably due to relatively good optimal microenvironment within the testis.