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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_141_библиотеки_им_акад_М_И_Перельмана

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Fig. 98.3 Facial lesions of acquired ichthyosis
98 Acquired Ichthyosis
observed in adults after puberty without a family history, an investigation for possible causes of acquired ichthyosis is needed. Histopathologically, it is known to show hyper­keratosis of the epidermis and decrease or loss of the granular layer, similar to ichthyosis vulgaris. If the under­lying disease is treated or the triggering drug is discontin­ued, the skin lesions tend to improve. Because the skin barrier function is decreased and there is a lot of water loss through the skin, symptomatic treatment is used to supply moisture to the skin and promote lubrication and keratolysis. For H, I prescribed a cream and moisturizer that are effective for itching, hyperkeratosis, and dryness and told his father that a comprehensive examination is needed to nd the cause of the student’s acquired ichthyo­sis (Figs. 98.3, 98.4, 98.5, 98.6, 98.7, 98.8, 98.9 and
98.10).
Figs. 98.4 and 98.5 Acquired ichthyosis of the arm
Figs. 98.6 and 98.7 Acquired ichthyosis of the trunk
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Figs. 98.8 and 98.9 Acquired ichthyosis observed on the leg
405
Fig. 98.10 Acquired ichthyosis
Black Heel, Talon Noir, Calcaneal
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Petechiae
99
I recently noticed black spots on my heel, could this be a serious disease? (Fig.99.1).
Mr. Y, who was almost a professional tennis player during his school days, recently rediscovered his love for tennis with his wife. One day, while taking a bath, he was startled to nd many black spots on his heel. That’s because he recently heard that dark spots on the skin could be skin cancer.
When playing sports like basketball, volleyball, and tennis, sudden stops, starts, and jumps can cause a large number of irregular black spots to appear on the heel. This is known as black heel (talon noir/traumatic calcaneal petechiae), and it is thought to be caused by the rupture of small blood vessels in the dermal papillae due to friction and twisting between the heel and shoes during vigorous exercise. It was rst
Fig. 99.1 Black heel (calcaneal petechiae)
described in 1961. It often occurs in adolescents who are physically active and is characterized by the sudden appear­ance of black or dark blue spots on the back or side of the heel. In many cases, the skin around the pigmented spots can be seen to be hyperkeratotic. It is relatively common, but because it is asymptomatic, naturally disappears, and occurs in a location that is difcult to check with the naked eye, it is usually discovered accidentally by the individual or someone else. It usually appears on one foot but can sometimes be observed on both feet, and there have been cases reported where it has appeared on the front of the foot, toes, and hands. When similar signs appear on the palm, it is called black palm. It can occur in mogul skiers who repeatedly hit the snow with their poles, tennis players who hit the ball hard, or people who practice golf intensively.
An important point about this condition is that if a large number of black spots merge, it can be confused with mel­anoma. When repeatedly cut with a scalpel, a characteris­tic appearance of reddish-brown dried hemorrhagic material is observed. Once this disease is experienced, the diagnosis can be easily made based on the location of the lesion, age, exercise, sudden occurrence, and characteris­tics after cutting with a scalpel. However, it can be difcult to diagnose if the lesion occurs in areas other than the heel. Histologically, you can observe amorphous material of a yellowish-brown color gathered in a round shape in the epidermis and stratum corneum. This is due to the agglom­eration of red blood cells from bleeding at the tip of the dermal papillae. It is not necessary to treat it as it will dis­appear on its own once the cause is gone, but local heat fomentation with a thermal pack can help calm the lesion, and continuous trimming alone can remove the lesion. After hearing the detailed explanation, Mr. Y left the clinic with a relieved expression (Figs.99.2, 99.3, 99.4, 99.5,
99.6 and 99.7).
© The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2024 J. Y. Jeong, Dermatology Diaries, https://doi.org/10.1007/978-981-97-1578-7_99
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Fig. 99.2 Black heel
99 Black Heel, Talon Noir, Calcaneal Petechiae
Fig. 99.3 Black heel
Figs. 99.4 and 99.5 Black spots observed on the big toe area
Figs. 99.6 and 99.7 Black palm occurred after golf practice
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100
One day, I have a black line on my nail. Is it skin cancer? (Figs.100.1100.3).
Mr. J, a man in his 50s, was deeply troubled. He had read an internet article saying that the black line that had appeared on his nail could be a symptom of skin cancer, melanoma. Many people come to check if the black spots on their skin
or the black lines on their nails are signs of a serious disease, but Mr. J seems to be particularly nervous. However, there is no need to worry in advance.
One of the common concerns encountered in the clinic, but often lacking a clear answer, is the black line that appears on the nails. Melanonychia refer to the nails turning black or
Figs. 100.1–100.3 Before and after treatment of melanonychia with Q-switched Nd:YAG laser
© The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2024 J. Y. Jeong, Dermatology Diaries, https://doi.org/10.1007/978-981-97-1578-7_100
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100 Melanonychia
brown without pain or itching. In a narrow sense, it refers only to those caused by melanin pigmentation, and in a broad sense, it includes all cases that can cause black or brown pig­mentation on the nails, but since the components of the pig­ment cannot be conrmed with the naked eye, it is considered rationale to apply a broad denition in dening black nails clinically. Nail pigmentation can appear clinically in longitu­dinal, local, extensive, and transverse forms, but among them, the case where pigmentation occurs along the longitu­dinal axis where the nail plate grows is called longitudinal melanonychia. Longitudinal melanonychia is understood as a phenomenon where melanin pigment production increases in most cases from the nail matrix, and as a result, melanin pigment is deposited on the nail plate.
The difference between the melanocytes of the nail matrix and the melanocytes of the surrounding skin is that the num­bers are small, most are not activated, and they are distrib­uted in the lower 2–4 cell layers of the nail matrix. The causes of melanonychia can be divided into those of melano­cyte origin and those not of melanocyte origin. Melanocyte origin can be divided into increased melanocyte activity (melanocytic activation, melanocytic stimulation, functional melanonychia), benign melanocytic proliferation, atypical melanocytic proliferation, melanoma in situ, and malignant melanoma according to histological features. Increased melanocyte activity means an increase in melanin pigment in the basal cell layer without an increase in the number of melanocytes. Benign melanocytic proliferation is accompa­nied by an increase in the number of melanocytes, and if the melanocytes do not form a nest, it is called a lentigo, and if the melanocytes form one or more nests, it is called a mela-
nocytic nevus. If the number of melanocytes increases and some atypical nuclei and Paget’s disease-like spread appear, it is called atypical melanocytic proliferation, and differen­tiation from melanoma in situ is necessary. Unlike melanoma in situ, atypical melanocytic proliferation does not show highly atypical nuclei, high transepidermal migration of melanocytes, or disruption of epidermal contour. Clinically, there is still controversy over whether atypical melanocytic proliferation can cause malignant changes in the future, but surgical removal is necessary for preventive purposes (Figs. 100.4, 100.5100.7, 100.8, 100.9, 100.10, 100.11,
100.12 and 100.13).
Malignant melanoma shows differences in incidence and
predilection sites depending on race and environmental fac-
Fig. 100.4 Longitudinal melanonychia
Figs. 100.5–100.7 Longitudinal melanonychia
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Figs. 100.8 and 100.9 Longitudinal melanonychia
411
Figs. 100.10 and 100.11 10× magnication of longitudinal melanonychia
Figs. 100.12 and 100.13 10× magnication of longitudinal melanonychia
tors, and the prevalence of longitudinal melanonychia also differs by race, with 1% in whites, 77% in blacks, and 10–20% in Asians. About 6% of adults with longitudinal melanonychia have been conrmed to have malignant mela­noma, but it is extremely rare for longitudinal melanonychia
in children to be diagnosed as subungual melanoma. In a study of 75 patients with longitudinal melanonychia in Korea, 11 patients (14.7%) were classied as malignant dis­eases, consisting of 6 malignant melanomas (8.0%), 3 mela­nomas in situ (4.0%), and 2 atypical melanocytic
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proliferations (2.7%). Sixty-four patients (85.3%) were clas­sied as benign diseases, consisting of 49 with melanocytic activation (65.3%), 5 with melanocytic nevi (6.7%), 5 with hemorrhage (6.7%), 4 with onychomycosis (5.3%), and 1 person with nger mucous cysts (1.3%). Of the 58 patients who had longitudinal melanonychia on only one nail plate, 11 (19.0%) were classied as malignant diseases, as were 11 of the 40 patients (27.5%) whose pigmented bands were not uniform in color, 10 of the 12 patients (83.3%) who showed Hutchinson’s sign, and 9 of the 20 patients (45.0%) who had deformities or ulcers of the nail plate. All 15 patients (100%) diagnosed at age 18 or younger, 21 of the 22 patients (95.5%) who were 18 or younger at onset, all 17 patients (100%) who had more than one nail plate affected, all 35 patients (100%) whose pigmented bands were uniform in color, 47 of the 48 patients (97.9%) whose pigmented bands were less than half the width of the entire nail plate, 62 of the 63 patients (98.4%) who did not show Hutchinson’s sign, and 53 of the 55 patients (96.4%) who did not have deformities or ulcers of the nail plate were classied as benign diseases. In other words, all 11 patients with longitudinal melanonychia classi­ed as malignant diseases had it on only one nail plate, all 11 had pigmented bands that were not uniform in color, 10 (90.9%) showed positive ndings for Hutchinson’s sign, and 9 (81.8%) had deformities or ulcers of the nail plate. Of the 64 patients with longitudinal melanonychia classied as benign diseases, 62 (96.9%) did not show Hutchinson’s sign, and 53 (82.8%) did not have deformities or ulcers of the nail plate. The age group in which subungual malignant mela­noma is most commonly diagnosed is known to be in the 40s and 50s, and the locations where subungual malignant mela­noma is most commonly diagnosed are reported to be the thumb, index nger, and big toe.
Hutchinson’s Nail Sign
This refers to the spread of brown or black pigment from the nail bed, matrix, and nail plate to the adjacent cuticle and the proximal or lateral nail fold. It is an important clinical clue for subungual melanoma. It was rst described by Hutchinson in 1886. Hutchinson’s sign is also observed in malignant tumors of the nail unit other than melanoma, such as squamous cell car­cinoma in situ (Bowen’s disease) and basal cell carci­noma, benign tumors such as onychomatricoma and supercial acral bromyxoma, and nail and mucosa pigmentation disorders such as Peutz–Jeghers syn­drome or Laugier–Hunziker syndrome. Also, it is observed in nevus of the nail unit, systemic conditions (AIDS, pregnancy, malnutrition), drug use (minocy-
100 Melanonychia
cline, zidovudine, amlodipine, hydroxycarbamide), fungal and bacterial infections, trauma-induced pig­mentation, post-inammatory hyperpigmentation, radiation therapy, and silver nitrate staining. The excessive pigmentation of the nail bed or matrix, which is similar to Hutchinson’s sign and can be seen through the transparent nail folds, is not uncommon and is referred to as “pseudo-Hutchinson’s sign.” However, in these cases, it is only observed directly above the pigmented band. There is also a “micro-Hutchinson’s sign,” which is pigmentation of the epidermis that is not visible to the naked eye but can be conrmed with a dermoscope (Figs.100.14100.16).
The three steps in diagnosing melanonychia are (1) distin-
guishing whether the pigmentation of the nail is melanin or not, (2) determining whether the occurrence of melanonychia is due to the activation or proliferation of matrix melano­cytes, and (3) if it is due to proliferation, evaluating whether it is benign or malignant. Melanonychia can be caused by a variety of diseases, including subungual hematoma, onycho­mycosis, drug side effects, melanocytic nevus, lentigo sim­plex, melanocytic hyperplasia, and subungual melanoma. Among these, the most attention is required for differential diagnosis of subungual melanoma. Usually, subungual hematoma and onychomycosis do not show longitudinal pig­mentation clinically and are relatively easy to diagnose due to the history of trauma and changes in nail shape. Transverse melanonychia is rare, but it has been reported in patients who have used drugs such as anticancer drugs (cyclophospha­mide, hydroxyurea, adriamycin), antimalarial drugs (amodi­aquine, quinacrine, chloroquine), antiviral drugs (zidovudine, lamivudine), iniximab, and imatinib. Other pigmented dis­eases, including melanocytic nevus, always need to be dif­ferentiated from malignant melanoma, so it is not easy to determine the diagnosis and treatment plan. In the case of longitudinal melanonychia, the most certain way to deter­mine whether the cause of the increase in melanin pigment in the nail bed is a benign or malignant disease is to perform a biopsy on the nail bed. However, because nail biopsy is inva­sive, it is problematic because it often causes cosmetic prob­lems, including permanent deformation of the nail. Especially in children, it is not easy to decide to perform a biopsy solely for diagnosis, as it can lead to deformation of the nails for the rest of their lives. Therefore, if we are aware of the possibil­ity that malignant disease is the cause and the clinical nd­ings suggesting malignancy and benignity, and if we can perform a biopsy selectively considering the patient’s daily
100 Melanonychia
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413
Figs. 100.14–100.16 Cases that received a biopsy due to being mistaken for Hutchinson’s sign
exogenous melanonychia have other substances attached to the nail plate and usually do not show a striped pattern. The most common non-melanin pigmentation is subungual hematoma, onychomycosis, and Pseudomonas infection. The number of affected ngers is also important, and if more than one is affected, it should rst be considered due to the activation of melanocytes, such as drug-induced melano­nychia, and a gray background of the band and thin gray par­allel lines are observed. If only one nger is affected, the proliferation process of melanocytes should be considered, and in such cases, it becomes a dilemma to distinguish between malignant and benign. The color of the band can be cloudy or clear, the boundaries can be clear or faint, and the width can range from very thin to covering the entire nail.
Fig. 100.17 Acral lentiginous melanoma
The nail plate can change or be completely normal. And black-brown pigmentation around the nails (Hutchinson’s
sign) can also be observed. The rst thing to consider in lon­life, it will be helpful in improving the patient’s quality of life (Fig.100.17).
Dermoscopy can be usefully used in the rst step of diag­nosing melanonychia. Generally, melanin pigmentation appears as black-brown stripes within the nail plate. However,
gitudinal melanonychia due to melanocytic proliferation is the patient’s age, as the nail matrix nevus appears mainly in childhood, congenitally or acquired, and ungual melanoma in children is extremely rare, so clinical and dermoscopic ndings applied to adults are useless in children.
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100 Melanonychia
Since 2007, dermoscopy has been attempted for the eval­uation of pigmentation of adult nails, but the results are not always reliable, so it should be evaluated together with the clinical “ABCDEF rule” as much as possible. Dermoscopic ndings indicate that the risk of nding ungual melanoma is three times higher if (1) the width of the band is more than 2/3 of the entire nail plate, (2) the presence of gray-black tones, and (3) there is onychodystrophy. Dermoscopy of the hyponychium and the tissue around the nails has been reported to potentially contribute to the diagnosis of mela­noma by early detection of “micro-Hutchinson’s sign.” Dermoscopy is also used to observe the pigment pattern of the nail matrix, where (1) a regular gray pattern indicates hypermelanosis, (2) a regular brown pattern indicates benign melanocytic proliferation, (3) a regular brown pattern with globules or spots indicates melanocytic nevus, and (4) an irregular pattern is classied as melanoma. Such dermoscopy performed during surgery has been reported to help select the most suitable site for biopsy. Differential diagnosis of nail diseases can sometimes be very difcult and uncertain, especially in the evaluation of nail pigmentation. Although the use of magnifying glasses such as dermoscopy is gradu­ally increasing, there are reports that magnifying glasses examination should not be used as a substitute for histopath­ological examination, and it is recommended to perform excisional biopsy in all cases of longitudinal melanonychia suspected of melanoma. When a patient with longitudinal melanonychia visits, it is impossible to conrm whether the disease is benign or malignant based on clinical ndings alone, so Mannava etal. suggested the following indications for suspecting malignant disease and performing a biopsy. However, I would like to add item No.12 here (Table100.1).
Especially, children, the frequency of malignant mela­noma of the nails is very low, so it is generally believed that tissue biopsy should be reserved until the age of 10, even if the pigmented area of the nail plate widens or darkens through regular observation. Also, when malignant mela­noma is suspected, a biopsy including the nail matrix and
Table 100.1 Indications for biopsy in longitudinal melanonychia
No. Indications for biopsy in longitudinal melanonychia 1 Any new LM in a fair-skinned patient 2 LM of one nail only 3 Any history of change in LM lesion 4 Width>6mm 5 Proximal width>distal width 6 Heterogeneous pigment (multi-colored) 7 Blurry or jagged borders 8 Associated nail fold pigment (Hutchinson’s sign) 9 Associated nail plate dystrophy, bleeding, or ulceration 10 High-risk digit involved (thumb, index nger, great toe) 11 Personal or family history of melanoma 12 No response to Q-switched Nd:YAG laser
Table 100.2
diseases in longitudinal melanonychia
Comparison Age of onset Under 18 Over 40s and 50s Number of
involved nails Color of
pigment band Border of
pigment band Width of
pigment band Hutchinson’s
sign Changes in the
nail plate
Nail location Other ngers and toes Thumb, index nger,
Response to Q-switched laser
Comparison of the likelihood of benign and malignant
Findings with a high likelihood of benign disease
More than two One
Homogeneous Heterogeneous
Clear Blurry
Less than 6mm Greater than 6mm
Doesn’t appear Appear
Unaccompanied Accompanied by
Yes No
Findings with a high likelihood of malignant disease
dystrophy, bleeding, or ulceration
or big toe
nail bed is effective for accurate diagnosis, but it can perma­nently cause nail deformity, so it is also considered to per­form a punch biopsy at 2–3 sites using a 2–3mm punch after removing part of the nail plate, but it is only attempted in very exceptional cases under the age of 14 (Table100.2).
In adults, dermoscopy or biopsy is necessary for accurate differential diagnosis of melanonychia, and surgical opera­tion is the rst choice for the treatment of subungual mela­noma. However, there is still no consensus on the treatment policy for longitudinal melanonychia in children. In a Korean study that analyzed 158 black nails from 92 patients with melanonychia striata under the age of 5 and followed up for an average of 27.8months in 80 patients who returned for a return visit, all of them, including eight cases in which biop­sies were performed, were clinically benign. Progress was shown, and 39% of cases maintained the initial condition dur­ing the follow-up period, and 44% of cases showed spontane­ous improvement by shrinking or softening. In some cases, changes such as darkening or widening of the pigment band were observed, and although a biopsy was performed in a few cases, no ndings suspicious of malignancy were conrmed. Therefore, it has been suggested that not only invasive biopsy but also regular follow-up observation can be a good treat­ment policy. There have been reports on the effect of 755nm Q-switched Alexandrite for the treatment of melanonychia accompanied by onychomycosis. In the case of longitudinal melanonychia caused by increased melanocyte activity rather than proliferation of melanocytes, treatment using Q-switched Nd:YAG laser has been effective. There are cases where the pigment band disappears without recurrence after 5–10 treat­ments at one-week intervals. Since 2010, the treatment of