Small Macules Research Articles

Peutz-Jeghers Syndrome: A Comprehensive Review of Genetics, Clinical Features, and Management Approaches.

A relatively rare inherited condition known as Peutz-Jeghers syndrome (PJS) causes mucocutaneous pigmentation and gastrointestinal hamartomatous polyps. These polyps are non-cancerous, but the presence of PJS significantly increases the chances of developing various types of cancers, such as colorectal, pancreatic, gastric, and breast cancer. The purpose of this review article is to give an abbreviated summary of what is currently known about this syndrome, covering its clinical symptoms, pathophysiology, genetics, and management. PJS also raises the risk of getting many malignancies, especially gastrointestinal and pelvic cancers. Symptoms of the gastrointestinal tract brought on by hamartomatous polyps are frequent and include stool blockage, bleeding, and stomach pain. The pigmentation commonly appears as prominent bluish-black macules and frequently affects the skin and mucous membranes. Small macules and large regions of lentiginous pigmentation are both possible. Numerous areas, including the perioral area, buccal mucosa, fingers, and lips, exhibit pigmentation. Bowel obstruction and intussusception risk can be decreased by early identification and routine surveillance of gastrointestinal polyps. The gene serine/threonine kinase 11 (STK11) controls several biological functions, including cell polarity, growth, and proliferation. Genetic counseling is recommended for the affected individuals and their families. This can help assess the risk of passing on the condition to future generations and provide information about available reproductive options. Regular surveillance is crucial for managing the syndrome and reducing the risk of cancer development. Other syndromes and extra-gastrointestinal characteristics, such as somatic tumor polyps outside the gastrointestinal tract, are also linked to this syndrome.

Hyper- and Hypo-pigmented Small Macules on Face, Neck and Extremities: A Quiz

Abstract is missing (Quiz)

Interobserver variability in the classification of childhood maculopapular cutaneous mastocytosis.

Maculopapular cutaneous mastocytosis (MPCM) in children is classified in two variants: (i) monomorphic variant, presenting with the small macules or papules typically seen in adult patients; and (ii) polymorphic variant with larger lesions of variable size and shape, typically seen in children. The definition of polymorphic and monomorphic variants is mostly intuitive, and a validation of this classification has not been done. To study interobserver variability in the classification of MPCM in two groups of observers: mastocytosis experts and general dermatologists. Nineteen cases of childhood MPCM were shown blindly, for classification as monomorphic or polymorphic type, to 10 independent observers (eight dermatologists, one allergist and one haematologist) from Europe and North America with a vast experience in the management of paediatric mastocytosis. Also, the same cases were shown on a screen to 129 general dermatologists attending a meeting; their votes were registered by remote controls. The interobserver variability kappa coefficient (with 95% confidence interval) was calculated to measure the reliability of the correlation. The value of kappa interobserver variability coefficient for the group of 10 experts (95% confidence interval) was 0.39 (0.18-0.63), which is considered as 'fair'. The value of kappa interobserver variability coefficient for the group of 129 general dermatologists (95% confidence interval) was 0.17 (0.06-0.39), which is considered as 'slight'. A complete agreement of all 10 experts was achieved in only four of 19 cases (21.1%) The most voted choice was concordant between the two groups in only 11 of the 19 cases. We failed to validate the classification system of childhood MPCM in monomorphic and polymorphic types. While the rate of agreement was low for mastocytosis experts, it was nearly the agreement expected by chance in general dermatologists.

Study of vitiligo at a tertiary care hospital

Background: Vitiligo is a common acquired, progressive, multifactorial, depigmenting disorder characterized by the appearance of circumscribed white macules varying patterns, varying from small macules with scalloping borders to near total depigmentation of body, supposed to be due to chronic, progressive loss of functional melanocytes in the epidermis. This study was aimed to study vitiligo in our tertiary care hospital. Material and Methods: This prospective, observational and descriptive study was conducted in OPD patients clinically diagnosed as vitiligo during study period. Results: A total of 300 patients were included in the study after applying inclusion and exclusion criteria. Among these 179 (59.67%) were females and 121 (40.33%) were males. The female to male ratio was 1.5:1. The age at onset was found to be in the 11-20 age group in 104 (34.5%) patients. Most common duration was noted as between 1 to 5 years, 169 (56.5%) patients. A positive family history was present in 62 (20.5 %) patients In 67 patients triggering factor was noted. Koebner’s phenomenon was noted in 62 (22.2%) patients while leucotrichia was seen in 33 (11 %) patients in our study. Most common site affected was lower limb in 204 patients (68.17%), followed by upper limb in 194 (64.67%) patients. Clinically most common morphological pattern was vitiligo vulgaris noted in 52.5% patients. Acrofacial, segmental, universal, mucosal patterns were noted in 23.83 %, 7.67 %, 5.67 %, 1 % patients respectively. Conclusion: Vitiligo has a multifactorial origin, unpredictable triggers and progress of disease. Early age of onset, family history, HLA antigen, presence of leucotrichia, other skin problems are predictors for poor prognosis.

Melanoma on nevus spilus

The nevus spilus (NS), also known as speckled lentiginous nevus or nevus on nevus, is represented by a brownish macule on which small macules of darker shades appear. It is more common on the trunk and lower limbs. The general population's prevalence is 0.2% to 2.3%, and they have a benign character. The exact risk for malignant transformation is still unknown; thus, it demands a routine clinical-dermoscopic follow-up. We present a case of malignant melanoma on acquired nevus spilus, in which early an excision was performed, with no recurrence, highlighting the importance of follow-up of these patients

Case 4: A Preterm Infant with a Necrotic Ulcer Over the Perianal Area

A 1.07-kg male infant with severe growth restriction is born at 30 weeks’ gestational age to a 36-year-old gravida 3, para 2 mother by emergency cesarean delivery for nonreassuring heart tracing. The parents are of low socioeconomic status and are first-degree consanguineous relatives. The mother received steroids and her membranes ruptured at delivery. At birth, the infant’s cord clamping was delayed, and after an initial weak cry he underwent intubation for poor respiratory efforts. Apgar scores were 4 and 7 at 1 and 5 minutes, respectively. His initial stabilization included surfactant administration, mechanical ventilation with volume guarantee, catheter line placement (umbilical venous and arterial catheters), initiation of total parenteral nutrition, antibiotics, and inotropic support. On day 3, he underwent extubation and received noninvasive intermittent positive pressure ventilation (NIPPV), inotropic support was discontinued, and antibiotics were stopped, because his blood culture was sterile. He demonstrated steady clinical improvement in the next 3 days, was weaned from NIPPV to nasal continuous positive pressure, and nasogastric feeds progressed gradually. On day 6, the bedside nurse noticed a small macule and blister with surrounding erythema over the lateral aspect of his right thigh, which progressed rapidly in hours to blackish-gray discoloration of skin in an area 4 × 1 cm in size with a small hemorrhagic lesion in the center (Fig 1). The infant’s clinical condition worsened rapidly in the next 24 hours; he became lethargic, hypothermic, tachycardic, hypotensive with poor perfusion, and apneic with increased oxygen requirement. He underwent elective reintubation for recurrent apnea and mixed respiratory and metabolic acidosis. A limited sepsis screening (complete blood cell count and cultures from blood, skin wound, and urine) was performed. Empiric antibiotic treatment with piperacillin-tazobactam was commenced. The infant received a bolus of normal saline, and dopamine treatment was commenced for low mean blood pressure. …

Becker nevus syndrome presented with ipsilateral breast hypoplasia.

Becker nevus syndrome (BNS) is a rare epidermal nevus syndrome characterized with Becker nevus and ipsilateral breast gland hypoplasia or other skin, skeletal and/or muscle tissue disorders. A 24-year-old woman presented with brown, irregular bordered patch with a diameter of approximately 10 cm which consisted of several small macules on the left breast skin. The ultrasonography and magnetic resonance imaging revealed left breast hypoplasia. Histopathological examination demonstrated minimal acanthosis, papillomatosis, increase in basal layer melanin and hypertrophy of the erector pili muscle. Immunohistochemical staining was positive for androgen in the epidermis, dermal stromal cells and skin appendages. Depending on the clinical and histopathological findings, the patient was diagnosed as BNS. Diagnosis of BNS needs careful examination of pigmented macules and patches since non-hairy BN may be easily overlooked. Patients with BN should be evaluated for associated abnormalities of BNS, in which the severity and extend of ectodermal involvement may differ from patient to other.

Direct Melanocytes Transplant from Normal Donor Area into Vitiliginous Recipient Area by Dermabrasion Technique

Background: Vitiligo is an autoimmune pigmentory disorder, that affects all age group that is treated by many medical treatments but some of them might need surgical therapy. Objective: To evaluate the dermabrasion technique in the treatment of vitiligo by direct transfer of melanocytes from the dermabraded normal donor area to the vitiliginous recipient area. Patients and Methods: This is a case interventional study was done in Department of Dermatology/Baghdad Teaching Hospital from February 2011-March 2012. Nine Patients with vitiligo were enrolled in this study with different clinical types of vitiligo including 5 segmental, 2 generalized and 2 localized. The donor and recipient areas were anesthetized at the same time with xylocain alone. Dermabrasion of recipient area was done first by manual abrader and left for few minutes until the oozing was stopped. Then the donor area was similarly dermabraded and the dermabraded tissue including the epidermis and superficial epidermis was immediately transferred into the recipient area and dressing was applied. Removal of the dressing was done after 10 - 14 days from the operative time. Follow up was done every 2 weeks in the first month then monthly for six months to record the result of implantation and repigmentation. Results: The re-pigmentation started one month after the operation as small macules and this increased gradually over time: the mean rate of re-pigmentation was 13% at 2 months, 27.8% at 4 months and 36.78% at 6 months. In addition, sun light exposure was applied to enhance re-pigmentation. The pigmentation was diffuse and not follicular in shape. Conclusion: Direct transfer of melanocytes from normal donor area into vitiliginous recipient area by dermabrasion technique was easy, rapid and non-costly and gave 36.78% mean rate of pigmentation at 6 months follow up and without complications.

Asymptomatic Progressive Hyperpigmentation in a 16-Year-Old Girl

REPORT OF A CASE A 16-year-old girl presented with a four-year history of asymptomatic, hyperpigmented, macular eruptions initially arising on the upper extremities and then slowly progressing to involve the entire body, sparing only the scalp, palms, soles, and mucous membranes. The lesions reportedly first appeared as small hyperpigmented macules with a palpable, erythematous border. The macules would enlarge slowly by peripheral extension and coalescence. The patient reported extensive areas, especially on the upper extremities, of erythematous, annular, raised borders that would disappear within days. Once pigmented lesions appeared, they persisted. The patient's general health was excellent, and there was no family history of cutaneous disease. Physical examination revealed uniformly bluishgray macules of varying intensity, ranging in size from small macules to large polycyclic patches (Fig 1). Lesions involved the face, neck, trunk, and extremities, sparing only the palms, soles, mucous membranes, and scalp. On the upper extremities, some of

CUTANEOUS MANIFESTATIONS OF PERIARTERITIS NODOSA

Periarteritis nodosa is an inflammatory disease affecting the smaller arteries and arterioles. Almost all the organs and tissues of the body may be involved, giving rise to a variable train of symptoms, depending on where the condition has particularly established itself. The vascular changes may result in a partial or complete cutting off of the blood supply causing impairment of function or localized areas of cell death. Alkiewicz¹collected from the literature between 1866 and 1933 36 cases in which the skin was affected. In 16 of these there were small macules (<i>kleine Flecken</i>), which were purpuric in 7; in 11 there were nodules; in 15, papules or small nodules; in 7, severe hemorrhages, and in 5, necrosis. In a fairly exhaustive survey of the literature from 1912 to the present we have been able to add 23 cases to those published by Alkiewicz. With over 200 reports in