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Considering palmar erythema requires memory (and lane)


DII small banner By Warren R. Heymann, MD
Oct. 26, 2017

  palmar-erythema.jpg
Bilateral palmar erythema with scattered purplish telangiectasias.
Credit: JAAD
I cannot imagine how many patients I have seen with palmar erythema (PE) during my career. It is usually asymptomatic, and when present, is often in the context of a known medical problem such as liver disease, connective tissue disease, or an endocrinopathy such as Graves disease. Honestly, I can’t recall patients presenting with PE as a chief complaint, although it must have happened on occasion. Either the reason was readily apparent, or I shrugged my shoulders, admitted my ignorance, and left it at that.

I had never heard of Lane Disease (LD, aka Erythema Palmare Hereditarium, EPH) until I read article by Gurioli et al. LD is a rare, benign condition presenting as persistent erythema involving the palms. EPH can appear at birth or later in life and usually in at least two members of the same family, although a sporadic case has been reported. The authors detailed five cases of EPH and reviewed the current literature. The first and second cases were twin boys presenting with erythema mainly on the thenar and hypothenar eminences and on the phalanges that appeared 8 months after birth. The third case was a girl with congenital palmar erythema and two other capillary malformations. The fourth case was a 58-year-old woman with palmar erythema that appeared after pregnancy. Her 32-year-old daughter had presented with the same palm redness since birth. Their review of the literature showed that women are affected almost three times more than men. Dermoscopic evaluation revealed red structureless areas with arborizing vessels, mainly running parallel along follicular openings. Biopsies and imaging studies were not performed. RASA1 mutations were negative in one patient tested who also had capillary malformations. The authors concluded that EPH should be considered in all patients presenting with PE, especially in familial long-lasting forms. It can be congenital or acquired, but the pathogenic mechanism is unknown. (1)

John Lane first reported this entity precisely at the same time of the stock market crash (October, 1929). He discussed two men, a lawyer aged 51 and a 69 year-old editor, both of whom had the disorder for years. The lawyer’s sister was reported to be affected as was the editors’ father and sister. The lesions were completely asymptomatic, although the cause of embarrassment. Dr. Lane offered no explanation for the disorder, although, in a discussion following the article, luminaries such as Drs. McKee, Pusey, Dennie, and Jadassohn, seemed to concur that the condition is not rare. (What a treat to read such a discussion immediately following the article – maybe that tradition should be reinstituted!). The following is Dr. Lane’s description of the condition:

Except for a slightly different distribution of the coloring, the appearance of the palms was the same in both patients. The skin of the palms was suffused with a bright red color, which was limited to the palms, and which terminated abruptly at the junction of the palmar skin with that of the wrist and sides of the hands. The color was so bright that a short distance it might easily have been taken for the effect of an acute inflammation. (2)

It is interesting that Dr. Lane specifically stated that he did not see telangiectasias clinically (I assume nothing like dermoscopy was used in 1929), however they are clearly noted on dermoscopy in the paper by Gurioli et al. Histology demonstrates dilated vessels in the entire dermis with no inflammatory infiltrate. Capillaroscopy reveals an increased number of capillary loops running parallel to the surface. Autosomal recessive transmission has been reported, although familial cases over three generations strongly suggest dominant or pseudodominant transmission. (3)
 
I know I have seen this before, but as the lyric goes “I can’t remember where or when”. Before diagnosing Lane disease, it may pay to stroll down memory lane to remember other entities that may present with PE.

Serrao et al provide an exhaustive review of PE, which can exist as a primary physiologic finding or as a secondary marker of systemic pathology. Primary or physiologic PE can be due to heredity, occurs in at least 30% of pregnant women as a result of associated alterations in the function of the skin and its microvasculature, or may be a diagnosis of exclusion (i.e. idiopathic PE). Secondary PE from systemic pathology encompasses a wide range of disease states. Twenty-three percent of patients with liver cirrhosis, from varying causes, can manifest PE as a result of abnormal serum estradiol levels. Patients with rare neonatal liver diseases such as Wilson disease or hereditary hemochromatosis may exhibit PE along with the other systemic manifestations of the genodermatoses. PE has been reported to occur in >60% of patients with rheumatoid arthritis and is associated with a favorable prognosis. Up to 18% of patients with thyrotoxicosis and 4.1% of patients with diabetes mellitus can have PE. PE can be seen in early gestational syphilis and among patients with human T-lymphotrophic virus-1-associated myelopathy. Drug-induced PE with hepatic damage has been documented with use of amiodarone, gemfibrozil, and cholestyramine, while topiramate and albuterol (salbutamol) have been reported to cause PE in the setting of normal liver function. Fifteen percent of patients with both metastatic and primary brain neoplasms may have PE. Increased levels of angiogenic factors and estrogens from solid tumors have been postulated as the cause of PE in such cases. Patients with atopic diathesis are more likely to have PE than matched control subjects. Smoking and chronic mercury poisoning are environmental causes of PE No treatment of primary PE is indicated. If medication is the cause of PE, the drug responsible should be discontinued if possible. Identification of PE related to underlying disorders should be followed by treatment of the underlying condition. (4)

Surprisingly, there are no reports of laser therapy for Lane disease to date. Undoubtedly, those reports will come, if dermatologists (including myself) remember the diagnosis!

1. Gurioli C, et al. Lane’s disease (erythema palmare hereditarium): A report of five cases and a review of the literature. Pediatr Dermatol 2017; 34: 590-4.
2. Lane JE. Erythema palmare hereditarium. Arch Dermatol Syph 1929; 20: 445-8.
3. Kluger N, Guillot B. Erythema palmare hereditarium (Lane’s red palms): A forgotten entity? J Am Acad Dermatol 2010; 63: e46.
4. Serrao R, et al. Palmar erythema. Am J Clin Dermatol 2007; 8: 347-56.

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