Chronic sole ulcer in a child with dyskeratosis congenita: An atypical wound successfully treated with punch grafting

Dyskeratosis congenita (DC) is an unusual inherited disease characterized by the triad of mucosal leukoplakia, nail dystrophy, and skin pigmentation. Hyperkeratosis of the palms and soles is another reported skin finding. This hyperkeratosis can lead to fissures, chronic erosion, and deep ulcerations. These atypical wounds are not only a diagnostic but a therapeutic challenge for clinicians, and there are no standardized treatments for these types of chronic wounds. Punch grafting is a traditional and minimally invasive technique to enhance wound healing, and it has been associated with significant and quick pain reduction in ulcers with various underlying causes. Herein, we describe a patient with DC with a chronic and refractory plantar ulcer successfully treated with punch grafting.     

Spiny Hyperkeratosis palmaris et plantaris

Spiny hyperkeratosis of the palms and soles is a rare dermatosis presenting as multiple tiny keratotic plugs on the palms and soles. There are two different forms of spiny keratoderma of the palms and soles – the hereditary form and the acquired form. The latter is usually associated with internal malignancies or systemic disease. Since spiny hyperkeratosis of the palms and soles has been under-reported and under-diagnosed, it is important for dermatologists not to forget this disease in daily practice. We report this rare disease for the first time in the German literature.     

Keratoderma climactericum (Haxthausen's disease): clinical signs, laboratory findings and etretinate treatment in 10 patients

10 cases of keratoderma climactericum are reported. This keratosis of the palms and soles appears late in women of menopausal age. The keratotic lesions first develop at the plantar pressure points, making walking troublesome. Involvement of the hands remains discrete. Examination for contact allergy, fungal tests, vitamin A serum levels, and sex hormones were negative or normal in all the 10 patients. Microscopy revealed a lichenified eczema with evidence of mechanical irritation. Etretinate (0.78 mg/kg/day) brought about partial or total remission of the hyperkeratosis. Pain on walking disappeared in all the patients.     

THE SURGICAL TREATMENT OF PALMO-PLANTAR KERATODERMA

SUMMARY. Identical twin boys with palmoplantar keratoderma were treated by total excision of the skin of one sole to the level of the plantar aponeurosis, followed by grafting. The hyperkeratosis did not recur on the treated sole, and the keratoderma of the untreated soles and of the palms disappeared. In both cases the keratoderma eventually recurred on the palms and on the untreated sole.     

板层状鱼鳞病二例

2例具有板层状鱼鳞病的典型皮损,全身皮肤灰褐色或灰棕色鳞屑,呈菱形、多角形或成大片,中央黏着,边缘游离高起,部分厚如铠甲.2例均有不同程度的眼睑、唇外翻及掌跖中度角化过度.例2皮损组织病理检查:角化过度,棘层及颗粒层增厚,表皮突延长,真皮浅层血管周围少量炎性细胞浸润.2例均口服阿维A胶囊,经尿素乳膏及鱼肝油软膏外用后,症状较前缓解.     

Hyperkeratosis of palms and soles : clinical study

Typical lesions of hyperkeratosis of palms and soles do not create any problem but when slight variation is there it becomes difficult to diagnose and that changes the line of treatment. For that we have studied 300 fresh cases of hyperkeratosis of palms and soles which included 110 cases of hyperkeratotic eczema. 42 cases of hyperkeratotic tinea, 64 cases of psoriasis of palms and soles and 84 cases of pustulosis palmaris et plantaris (PPP). To reach the final diagnosis alongwith the detailed history, general physical examination and routine investigations, help was taken of special investigations like pus for culture and sensitivity, KOH smear examination of skin scrapings and nail cutting and culture on Sabouraud's dextrose agar medium.     

Diffuse and focal palmoplantar keratoderma can be caused by a keratin 6c mutation

The palmoplantar keratodermas (PPKs) are a large group of genodermatoses comprising nearly 60 genetically distinct diseases. They are characterized by hyperkeratosis on the palms and soles with or without extrapalmoplantar hyperkeratotic lesions. Focal PPK is one of the hallmarks of pachyonychia congenita, a rare autosomal dominant disorder resulting from mutations in the keratin genes KRT6A, KRT6B, KRT16 or KRT17. Recently, in-frame deletion mutations of KRT6C have been identified in three families with focal PPK with slight or no nail changes. We report here a novel KRT6C mutation identified in a Japanese family with PPK with phenotypic heterogeneity, presenting with not only focal but also diffuse hyperkeratosis. The proband had diffuse hyperkeratosis on the soles and small focal hyperkeratoses on the palms, while the two other affected individuals showed focal hyperkeratoses on the soles. All three patients were heterozygotes for c.1414G>A in KRT6C, predicted to result in p.Glu472Lys. These findings strongly suggest that screening of patients with nonepidermolytic diffuse PPK, in whom the pathogenic mutations are yet to be determined, might identify mutations in KRT6C.     

回旋形线状鱼鳞病一家系调查

报告1例回旋形线状鱼鳞病患者的家系调查,该家系3代14人中仅有母女2人患病。2例患者均表现为出生后不久全身出现泛发性环形红斑和鳞屑,掌跖角化过度,无毛发异常。组织病理学检查显示:表皮轻度疣状增生,颗粒层变薄,棘层肥厚,角化过度等非特异性改变。     

Dorsal sensory impairment in hands and feet of people affected by Hansen's disease in Israel

INTRODUCTION: Sensory testing in people affected by Hansen's disease is usually performed on palms and soles only. In Israel, both palmar/plantar and dorsal aspects of limbs are routinely tested. OBJECTIVES: The aim of this study was to describe the magnitude of dorsal sensory impairment (SI) in limbs and compare the frequency of SI on palms and soles with that on the dorsum of hands and feet. DESIGN: In a cross-sectional study, limbs of 140 patients registered at The Israel Hansen's Disease Centre during the years 1999-2003 were tested for their sensory status. Both palmar/plantar and dorsal aspects were tested using Semmes-Weinstein monofilaments. SI was defined as not feeling stimuli applied with the 2 g monofilament. RESULTS: SI was detected on the dorsum in 43% of sites on hands and only in 27% on palms. 64% of sites on dorsum of feet had SI compared to 53% on the soles. SI was detected in up to 18% in hands with no palmar SI, and in 6% of feet with no plantar SI. Furthermore, SI on palms and soles was found to be accompanied by dorsal SI in all hands and in 97% of feet. CONCLUSION: SI on dorsum of limbs occurs more frequently than SI on palms and soles. Therefore sensory testing should also consider inclusion of the dorsal aspect of hands and feet.     

Constitutional painful callosities. Analgesic efficacy of etretinate

We have recently evaluated eight patients with an unusual genetic dermatosis that was characterized by the development of painful callosities at pressure points of soles, with tender yellowish-brown hyperkeratosis. These lesions are very painful: walking produced pain that was severe. The patients performing manual labor, in addition, had multiple callosities on the palms. Hyperhidrosis of the soles and palms was frequently present. Skin lesions were first noted in adolescence. There was no similar family history. There was no personal nor family history of hair, nail or dental disease. A biopsy was performed in one case and showed a simple hyperkeratosis similar to these observed in callosities. Classification of our cases is difficult. They are not similar to the entity named "hereditary painful callosities" by Roth et al. Review of the literature did not show similar cases. The patients received Etretinate (1 mg/day/kg). In all 8 cases, a dramatic improvement was obtained within seven days; the pain disappeared entirely and the patients were able to walk again without any pain. Then, the per-day dose was diminished without reappearance of the pain.     

对称性肢端角化病四例

报道4例对称性肢端角化病患者,其中男3例,女1例。表现为手腕、手背及足背部对称性红褐色角化性斑片,遇水皮损发白,冬季明显缓解。组织病理：角化过度,角化不全,表皮增生,棘层增厚,真皮浅层血管周围可见少许淋巴细胞浸润。符合对称性肢端角化病。     

Topical 0.25% methotrexate gel in a hydrogel base for palmoplantar psoriasis

Palmoplantar psoriasis (PPP) is a chronic, disfiguring condition, and its management is difficult. The systemic side effects of methotrexate can be avoided if it is effective topically. We studied the efficacy and safety of a recently marketed topical methotrexate (0.25%) preparation in a hydrogel base in patients with palmoplantar lesions. A total of 14 adult patients diagnosed clinically as plaque type of palmoplantar psoriasis (>30% of the palm and/or sole areas involved) were included in the study. Topical methotrexate 0.25% in a hydroxygel base was applied twice daily to the lesions for twelve weeks. The lesions were assessed for degree of erythema, scaling, induration and fissuring (ESIF) and were scored on a severity score of 0-3 (0--clear, 1--mild, 2--moderate, 3--severe) every two weeks. The most severe condition was given 12 points; 0 denoted no disease. The response at the end of the study was graded as minimal if there was up to 25% reduction in the EISF score, mild as 26-50% reduction, moderate improvement as 51-75% reduction in score, and marked improvement as >75% reduction in score. The average age of the 11 male and female patients was 41.5 years (18-57 years) with the duration of the disease varying from 2 months to 15 years. Ten patients had both palmar and plantar lesions; two each had only palmar lesions or plantar lesions. The ESI score at baseline was 5.8 +/- 0.9 for the palms and 6.8 +/- 0.5 for the soles. The scores at the end of the study were 3.5 +/- 0.7 for palms and 4.8 +/- 0.2 for the soles. The average time taken for improvement was at least six weeks. None of the patients had complete clearance of lesions. The drug was well tolerated by all patients. Methotrexate 0.25% in a hydrophilic gel is well tolerated but is not very effective in controlling the lesions of psoriasis on the palms and soles. A higher concentration in a different base with better penetration could possibly provide better results.     

Mycosis-fungoides-type cutaneous T cell lymphoma of the hands and soles: a variant causing delay in diagnosis and adequate treatment of patients with palmoplantar eczema

BACKGROUND: The etiopathology of chronic eczematous lesions of the palms and/or soles remains elusive in a considerable proportion of patients. Accumulating evidence suggests that a rare variant of mycosis fungoides (MF)-type cutaneous T cell lymphoma (CTCL) restricted to the palms and/or soles may mimic common palmoplantar dermatoses. OBJECTIVE: In the present study, we analyzed the clinical and histological characteristics of 3 adult patients with preexisting nonclassified chronic palmoplantar eczema poorly responding to standard therapies. Palmar and/or plantar MF was eventually diagnosed. METHODS: The course of the disease, response to previous therapies and dermatological features are described, results of histochemical and immunohistochemical analyses are reported, including T cell receptor gamma gene rearrangement where obtainable. RESULTS: Onset of cutaneous lesions with broad clinical variation was experienced 2-10 years prior to diagnosis; conventional therapies led to short-time or partial remission only; except for 1 patient, the epidermotropic infiltrate was predominantly composed of CD4-positive cells; topical photochemotherapy seems to result in more durable responses. CONCLUSION: As therapeutic strategies for this disease variant differ from symptomatic standard treatment regimens, awareness of MF-type CTCL as a relevant differential diagnosis of palmoplantar eczema should be expanded to prevent delay in diagnosis and adequate therapy.     

Solitary angiokeratoma on palms and soles: A clinicopathological analysis of 21 cases

Angiokeratoma includes a group of unrelated diseases characterized by hyperkeratosis and dilation of vessels in the superficial dermis. In this study, 21 cases of solitary lesions located on palms and soles and histopathologically diagnosed as angiokeratoma were analyzed retrospectively. All lesions presented as red to violet macules or plaques. Histopathologically, all of them showed hyperkeratosis and vascular dilation in the papillary to deep dermis. The elongated vessels were of different sizes and shape, lined by a single layer of endothelial cells. In 13 lesions, the endothelial cells protruded into the lumen, forming a hobnail appearance. Immunohistochemical study showed the endothelial cells were positive for CD31 and CD34, negative for D2‐40, and focally positive for Wilms tumor 1 (WT‐1) in most cases. Our cases represented a rare type of angiokeratoma located on the palms and soles, in which the focal positivity of WT‐1 was different from previously reported vascular proliferations.     

Lichen planus with lesions on the palms and/or soles: prevalence and clinicopathological study of 36 patients

According to the literature, palmoplantar lesions in lichen planus (LP) are uncommon, and do not usually have the classically described clinical morphology. The aim of this study was to evaluate the prevalence of palmoplantar lesions in LP and to describe the characteristic clinicopathological appearances of LP affecting palms and soles. Palmoplantar LP with accompanying skin involvement accounted for 26% of our cases. The presence of very pruriginous erythematous scaly and/or hyperkeratotic plaques, with well-defined edges, located on the internal plantar arch, without involvement of the fingertips, and which usually disappear in a few months, is characteristic of palmoplantar LP. Histopathological examination shows the characteristic features of LP.     

Histopathological study of hyperkeratosis of palms and soles

Clinically it is sometimes difficult to differentiate the various hyperkeratosis of palms and soles like psoriasis of palms and soles, pustulosis palmaris et plantaris, hyperkeratotic tinea and hyperkeratotic eczema. To differentiate amongst these conditions we have to take the help of histopathology. Here we are presenting the data of 292 cases of hyperkeratosis of palms & soles examined histologically by doing Haematoxylin and Eosin staining for the typical changes of the above mentioned conditions and PAS for the demonstration of the fungus in stratum corneum in cases of hyperkeratotic tinea.     

对称性肢端角化病1例

患者男,24岁。手足皮疹3年,加重1年。皮损特点为对称分布的红褐色角化性斑片,多发于手腕、手背及足背。遇水皮肤发白,冬季明显缓解。皮损组织病理示:角化过度、棘层肥厚、真皮浅层血管周围淋巴细胞浸润。诊断:对称性肢端角化病。     

Striate palmoplantar keratoderma (Brunauer-Fohs-Siemens syndrome)

A 64-year-old man presented with focal hyperkeratotic plaques on the fingers, palms, and soles. Histopathologic and electron microscopic results were consistent with striate palmoplantar keratoderma. Treatment with topical keratolytics was unsuccessful. Striate palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is an autosomal dominant condition that presents with linear hyperkeratosis on the palms and fingers and focal plaques on the plantar aspects of the feet. Histopathologic features include hyperkeratosis, hypergranulosis, and acanthosis with no epidermolysis. Electron microscopic examination shows diminished desmosomes, clumped keratin filaments, and enlarged keratohyalin granules. The syndrome has been linked to mutations in desmoglein 1, desmoplakin, and keratin 1. Treatment may include keratolytics, oral retinoids, and surgical debridement.     

Milia with Calcium Deposits on the Palms and Soles in a Patient with Multiple Syringoma

Abstract: An 8‐year‐old girl was referred for evaluation of multiple lesions on the neck, axillae, and genitalia. A great number of milia‐like lesions were also noticed on the palms and discrete keratotic plugs on the soles. Histopathology from lesions on the neck, axilla, and vulva were consistent with syringoma. On the palms and soles, eccrine ducts cystically dilated filled with proteinaceous material and calcifications were found. Although the nature of palmar and plantar lesions in our patient is uncertain, we think that they should be related to the other syringomas on her skin.     

Palmar filiform hyperkeratosis (FH) associated with underlying pathology?

We describe two patients with palmar filiform hyperkeratosis, characterized by multiple thin spiny keratotic projections on the palms. The condition has been associated with an underlying malignancy in some cases. One patient has myelofibrosis, an association not previously described. In addition we describe a further patient with filiform hyperkeratoses of both palms and soles with no associated underlying disorder. In view of the relatively high risk of underlying malignancy occurring in patients with filiform hyperkeratosis, these patients should always be investigated fully.