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.
