Congenital triangular alopecia (CTA) is a rare disorder with incidence of 0.11% in dermatological examinations. It presents as triangular, mostly unilateral, noncicatricial type of alopecia on the temporal region of the scalp. It is usually under reported due to its asymp-tomatic and nonprogressive nature. Diagnosis is clinical, aided by a classification proposed by some Authors and dermoscopy. It should be considered as a differential diagnosis in cases of nonscarring alopecia mainly alopecia areata (AA), with which it is often confused. This prevents unnecessary use of topical/intralesional corticosteroids, main treatment for AA. Two cases of CTA in 1 ½ and 2 year old girls are reported to highlight this rare entity.
LPP is a rare variant of LP that is infrequently encountered in children. In a retrospective analysis of 316 pediatric patients with LP, only nine (2.8%) were diagnosed with LPP (4). Similarly, in a recent retrospective study of 76 pediatric patients with LP in South India, only one (1.3%) was diagnosed with LPP (6). It is commonly seen in middle-aged patients with high skin phototypes. LPP presents as chronic, acquired, violaceous to brownish-gray, oval macules, with poorly-defined borders (7). The lesions appear more frequently in the neck and face, but they can also affect the upper and lower extremities, and torso as well. LPP rarely affects the oral mucosa, scalp, palms, soles, and nails. Flexural involvement is seen in around 20% of cases with a concurrent photo-exposed distribution (5). LPP-inversus is a variant of LPP, limited to intertriginous and flexural areas, and sparing sun-exposed areas (3, 5). A skin biopsy will confirm the diagnosis. The histopathology findings include hyperkeratosis and vacuolar degeneration of the basal layer, dermal band-like or perivascular infiltrate, and melanophages. Superficial pigmentary incontinence can be seen (7). The main differential diagnosis of LPP is erythema dyschromicum perstans (EDP) or ashy dermatosis. EDP presents as non-pruritic, blue-gray macules and patches, with or without raised erythematous borders in photo-exposed areas and trunk. Histopathology reveals lichenoid features within the peripheral active border. A perivascular infiltrate and numerous melanophages can be seen. The clinical features and histopathology of LPP and EDP can overlap, so much so as to suggest that the two conditions can be part of the spectrum of a unique entity. Other differential diagnoses include post-inflammatory hyperpigmentation, pigmented contact dermatitis, fixed drug eruption, and urticaria pigmentosa, among others (2, 7). LPP treatment is difficult, with limited results in most cases. The available topical treatments include medium to high potency corticosteroids, calcineurin inhibitors (mostly tacrolimus 0.1% ointment) and skin lightening creams containing hydroquinone and retinoids in conjunction with an anti-inflammatory treatment (3, 7, 9). Some Authors (3) recommend that in order to prevent steroid atrophy, corticosteroids should be used for a short period (2-4 weeks). Later on they could be followed by a calcineurin inhibitor over a longer period (3-6 months). In recalcitrant or widespread cases, systemic treatments with or without topical therapy can be used. They include oral corticosteroids, dapsone, and isotretinoin (7, 9, 10). The use of narrow band ultraviolet B (NB-UVB) phototherapy in LPP has been recently reported. Complete resolution of lesions was achieved after 20 exposures and no adverse effect was observed (1).
Background. Nail lichen planus is considered to be rare in children. Information about its prognosis is lacking. Objectives. To review the epidemiological and clinical features, response to treatment and follow-up of 12 children with nail lichen planus. Methods. Retrospective study involving 12 children younger than 16 years with a clinical and histopathological diagnosis of nail lichen planus, seen from April 2006 to December 2019 at outpatient consultation for nail disorders of the Department of Dermatology of the University Hassan II of Casablanca, Morocco. Results. Data on 12 children were collected, with an average age of 10.6 years (8-16 years). A male preponderance was observed (7/12). The mean duration of the disease was about 16 months. None of the children had skin or mucosal lesions of lichen planus. In all nail lichen planus cases, nail bed involvement was associated with nail matrix damage. In all cases nail biopsy confirmed the diagnosis. Intramuscular triamcinolone acetonide was used in all patients. Apart from the patients with pterygium, most of the children improved. Conclusions. Because of the risk of permanent scarring in nail lichen planus, early diagnosis is essential. Nail biopsy is a relatively simple, safe and useful procedure with a minimal scarring risk, even in children. Treatment should be implemented immediately.
Midline skin malformations are very common in the newborn; in most cases, the latter are isolated skin lesions of little clinical importance; on the other hand, in some cases they are a clue to the diagnosis of possible malformations of the underlying tissues with severe prognosis. An example of this second occurrence is the supraumbilical raphe. Closure on the midline occurs around the eighth week of gestation when the mesoderm merges on the midline. When this mesodermal fusion does not occur, the overlying ectodermal epithelium undergoes necrosis. The supraumbilical raphe is believed to result from this lack of midline ecto-mesodermal interaction, followed by scar repair (6). The umbilical raphe can be an isolated skin defect or associated with sternal cleft, Cantrell pentalogy with cardiac malformations up to ectopia cordis, vascular dysplastic syndrome and PHACES syndrome (4). The supraumbilical raphe can be associated with hemangiomas in the absence of other malformations (2). However, other malformations are more frequently present, as in PHACES syndrome. In the latter case, the umbilical raphe can represent the first and only sign at birth; hemangioma can in fact manifest itself after days or weeks, while posterior fossa malformations and encephalic and cervical vascular dysplasias can be only unveiled with imaging techniques; if ignored, vascular dysplasias can be unveiled even after decades by stroke episodes (1). These cases reopen the discussion of the relationship between malformations and hemangiomas. Although usually independent, it is possible that vascular malformations may be responsible for hemangioma: in these cases the hemangioma could be the expression of an exaggerated angiogenic response to the condition of hypoxia caused by vascular dysplasia (5). However, it is also possible that the same pathogenetic factor, maybe of maternal origin, acting at different times, could be responsible for both early malformations, which occur during the first three months of gestation, and hemangiomas, which occur in the perinatal period. There are data from the literature (3) that really show the presence in the same family of limb defects secondary to vascular malformations in some relatives and hemangiomas in others. The actual case series confirms that midline supraumbilical raphe and hemangioma may be present in the same individual as in the first case. However, it also emphasizes that the two disorders can occur separately in two twins.
The differential diagnosis of angioedema is extensive because several disorders can mimic this clinical feature. Here is reported the case of a 12-year-old boy who presented with recurrent self-limited episodes of non-painful, asymmetrical, swollen lips, without urticaria. After about 1 year lips edema became persistent and associated with gingival hypertrophy and bilateral zygomatic edema. After exclusion of the most likely hypothesis, the histological findings led to the diagnosis of orofacial granulomatosis, which is a very rare disease in adults, and even less frequent in pediatric patients. The Authors emphasize the importance of an adequate differential diagnosis, since the underlying etiology can be a rare disease requiring a specific treatment.
Radiotherapy is still used today in the treatment of infantile hemangioma (IH) in the case of particular localizations such as the pituitary fossa, when other treatments are not possible (1). On the other hand, it is not actually indicated in cutaneous IH, even if it was still used until the ’60s. Among its side effects there is chronic radiodermatitis (6), which today is observed mainly in women undergoing radiotherapy for breast cancer. The possibility of developing radiodermatitis depends on various factors, including the proximity to the skin of the radiotherapy target, the radiation energy, the overall dose of radiation and its treatment scheme, and the size of the skin surface exposed to radiation. Chronic radiodermatitis is histologically characterized by fibrosis of the dermis with disappearance of the hair follicles and glands, and rarefaction of the vessels; some residual vessels can be very dilated. The epidermis can be acanthotic or atrophic and characterized by numerous dyskeratotic cells. However, a more fearful side effect of radiotherapy for IH is the late onset, even after 6 decades (8) of tumors (2, 3, 7), including angiosarcoma (5) and melanoma (4). Besides malignant tumors there are also benign proliferations. The atypical vascular proliferations on radiodermatitis are benign tumors consisting of branched and dilated vessels or irregular vascular lacunae delimited by endothelium; degeneration into angiosarcoma has been described in 3-6% of cases (8). The considerable delay between irradiation and the appearance of these proliferations seems to be linked to the low doses of X-rays used in the treatment of hemangioma (8). The current case was presented due to the rarity of atypical vascular proliferations and to remind us that neoformations on radiodermatitis are not always malignant.
Among the acquired diseases which are distributed along Blaschko’s lines there are linear psoriasis and lichen striatus: in addition to the same distribution, which is linked to a condition of mosaicism, they are both acquired inflammatory diseases and usually asymptomatic, sometimes creating problems of differential diagnosis.
Staphylococcal scalded skin syndrome (SSSS) is characterized by superficial blistering of the skin caused by exfoliative toxins of Staphylococcus aureus. SSSS predominantly affects children under five-year-old. We present a case of a 30-day-old female infant who developed circumscribed flaccid blisters within erythematous skin, with positive Nikolsky’s sign, without mucosal involvement. The ruptured blisters resulted in desquamation and scalded skin, then extending rapidly. The diagnosis of SSSS was achieved clinically and the patient was treated with intravenous cloxacillin. However, despite the given treatment in limited resources setting, the patient died due to sepsis. This case report highlights the difficulties in treating SSSS in limited-facilities hospital setting and underlines its life-threatening complications, such as sepsis. It is also aimed at raising awareness of SSSS as a possible differential diagnosis of superficial blistering and exfoliative skin disorders, especially in children population.
Skin picking disorder (SPD) is not a dermatological problem but a clinical picture causing a damage on the skin tissue and characterized by picking the skin intensely and repetitively. Psychiatric disorders such as depression, bipolar disorder and obsessive-compulsive disorder can play a role in its etiology. Neurodermatitis is included in the skin picking disorder spectrum and can be considered its synonym. Clinically, it is characterized by single or multiple lichenified plaques, which are often hyperpigmented and excoriated, and accompanied by prominent skin lines. The psychiatric factors play a role also in nodular prurigo, which is characterized by severely itchy nodules. A 5-year-old male patient, who had been diagnosed with atopic dermatitis, was brought to our outpatient clinic by his family with the complaints of compulsive scratching, which was responsible for severe bleeding. The patient was diagnosed with nodular prurigo/neurodermatitis with SPD. The rarity in the pediatric literature of case reports discussing the association with SPD in a frequently encountered clinical picture such as atopic dermatitis creates a concern that SPD cases are not diagnosed in children. This case has been presented to attract attention to the fact that atopic dermatitis and SPD can be observed together although they are two different clinical pictures and to underline that there should be no delay in diagnosis of SPD since there are also psychiatric disorders in the background.
Neonatal Herpes simplex virus (HSV) infection is a potentially life threatening disease. We describe a case series of 4 neonates who demonstrate variable presentations of neonatal cutaneous HSV and the difficulties that can occur in diagnosis. Our aim is to increase awareness of the clinical variation in neonatal HSV to facilitate early diagnosis, prompt treatment and improved outcomes.
Loxoscelism is produced by the bite of Loxosceles spiders. There is in the literature a rare variant of cutaneous loxoscelism, mainly edematous, which is called edematous cutaneous loxoscelism (ECL). Here is reported the case of a 12-year-old girl, who presented ECLwith upper airway obstruction.
These characteristics of the Ct bring them closer to secondary chilblains, especially those that occur in connective tissue diseases (lupus erythematosus, antiphospholipid syndrome), in tumor diseases (leukemia), and even more so in genetic interferonopathies, such as familial chilblain lupus erythematosus due to TREX1 mutation, SAVI or childhood-onset vasculopathy associated with STING (interferon stimulating genes) and Aicardi-Goutiers syndrome. In all these conditions, there is an activation of interferon type 1, that is genetically determined or secondary to the need to eliminate non-self oligonucleotides. The activation of type-1 interferon induces microangiopathic alterations responsible for secondary chilblains as a side effect. It is conceivable that the presence of SARS-CoV-2 nucleotides stimulates the signaling pathway of interferon 1. This hypothesis is supported by a) the higher frequency of Ct in teens, in whom the interferon-mediated innate immunity is much more active (3); b) the histological similarities between chilblains of interferonopathies and Ct; c) the recent demonstration of the presence of Myxovirus resistance protein A, which is a recognized tissue marker of interferon 1 activity, in the epidermis, endothelial cells and in the inflammatory infiltrate of the Ct (5). The existence of a strong response of type 1 interferon could also explain the scarce if any simptomatology of SARS-CoV-2 infection in Ct, and the negativity of molecular swabs and serological tests, linked to the rapid elimination of the virus.
The implications of leprosy detected in a child are numerous. Apart from making it mandatory to search for the source in the family, it indicates continued transmission of the disease in the community and thus poses a great hurdle in achieving the targets laid by World Health Organization (WHO) and National Leprosy Eradication Programme (NLEP) in the direction of leprosy elimination. In spite of an effective treatment and the tireless work of various government and non government organizations, the number of childhood cases have not drastically reduced. Elimination of childhood leprosy needs to get the highest priority, not only because of the physical deformities and disabilities but also because of the psychological trauma it causes due to the social stigma attached to it. So we undertook a retrospective study of 5 years from 2015 to 2020 retrieved from the departmental data involving all diagnosed leprosy cases below 18 years of age and studied their clinical spectrum, histopathology and treatment outcomes. In our study, the mean age at the time of diagnosis was 12.95 years. Borderline tuberculoid was the most common type of leprosy in 61.9% of cases. 19.04% had lepra reactions and 14.6% had deformities in the form of claw hand and nerve abscess. The overall incidence of childhood cases was 17.07% that was much higher in comparison to the national figure of 6.87%, probably owing to the small sample size and as the study was conducted in an endemic area. This study highlights the fact that leprosy, especially in children, appears to be far from the point of elimination and a stringent strategy is required to achieve the goal of its eradication.
Among the stimuli capable of causing nevi and influencing the production of melanin there are, in addition to genetic factors, phototype, immune suppression, sun radiation, even hormonal factors (1). Clinical evidence of this is the exceptional occurrence of melanoma before puberty, the increase in moles and melanic production in conjunction with puberty and pregnancy, and the regression of many moles in the last decades of life (4). However, there are also experimental studies that demonstrate the role of hormones and in particular of estrogens in the appearance of benign and malignant melanocytic lesions. Prior to the discovery of estrogen receptors (ER) beta, studies with ER alpha had not demonstrated a role of estrogen on the pathophysiology of benign and malignant melanocytic lesions (8). After the discovery of ER beta, it was found that these receptors are present in all melanocytic lesions, both benign and malignant (6). As regards benign melanocytic lesions, ER beta are particularly present in congenital (2) and in Spitz-Reed nevi (7). The vulvar melanocytic nevus of the actual report is probably a poorly pigmented congenital junc-tional nevus, also due to the non-photo-exposed site, which has suddenly become visible due to the effect of peripuberal hormonal stimuli. There is another very similar example in clinical practice, namely the congenital melanocytic nevus not evident at birth (3). Melanocytic nevi that appear in the first year of life are usually thought to be congenital (5). However, many of these nevi are really present at birth, but not visible because they are junctional and non-pigmented (3). The accuracy of this hypothesis is demonstrated by the fact that at their first appearance these nevi already have many times a diameter greater than one centimeter and that after becoming evident due to the pigmentation generally induced by the first sun exposures they never show autonomous growth.
Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized by concomitant vascular malformation and melanocytic lesions. The disorder may manifest only on the skin or be accompanied by abnormalities of other organs. PPV is divided into five types; the most reported case is type II, while type V is uncommon and the subtype Va is rare.
Smooth muscle hamartoma (SMH) is difficult to diagnose due to the variability of size, shape and color. When it has a brownish color and hypertrichosis is not very visible, it can simulate mastocytoma also because at palpation it can become more prominent and evident (pseudo Darier).
The pandemic infection caused by the new coronavirus SARS-CoV-2 (COVID-19) has been associated, like numerous other viral infections, with heterogeneous skin manifestations. The latter are present both in severe forms, where they are less important compared to the involvement of internal organs, and in mild, poorly symptomatic cases where they may be important for tracking the disease. The current report describes two brothers with an atypical eruption associated with COVID-19 characterized by prevalent involvement of the face and very late onset, two months after modest systemic symptoms.
